Rare Cancer News & Clinical Trials

Sarcoma (292 unread)

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Small intestine leiomyosarcoma mimicking gastrointestinal stromal tumour

Fetched: September 16th, 2021, 5:02am GMT by Mohammed Fawaz

BMJ Case Rep. 2021 Sep 13;14(9):e244381. doi: 10.1136/bcr-2021-244381.

ABSTRACT

Small bowel malignant tumours make only 2% of all gastrointestinal (GI) malignancies. Small bowel leiomyosarcoma (LMS) is further rare, accounts for only 0.1%-3% fraction of these tumours. These cases can present as asymptomatic intra-abdominal mass, anaemia due to GI bleed or acute abdomen such as perforation peritonitis, intussusception and bowel ischaemia. Standard of care is surgical resection. Our case presented as large lobulated exophytic ileal LMS measuring 10.8×11×14.7 cm involving multiple small bowel loops and abutting right iliac vessels and uterus. Patient's clinical course was complicated with COVID-19 positivity, deep vein thrombosis and pulmonary thromboembolism. She was managed by preoperative anticoagulation followed by resection of the tumour with end ileostomy.

PMID:34518183 | DOI:10.1136/bcr-2021-244381

Permalink for 'Demographic characteristics, treatment, and outcome of patients with Kaposi sarcoma: Ten-year follow-up results of a single center'

Demographic characteristics, treatment, and outcome of patients with Kaposi sarcoma: Ten-year follow-up results of a single center

Fetched: September 16th, 2021, 5:02am GMT by Begüm Güneş

J Cosmet Dermatol. 2021 Sep 15. doi: 10.1111/jocd.14426. Online ahead of print.

ABSTRACT

BACKGROUND: Clinical features, types of Kaposi sarcoma, and treatment outcomes have not been well-defined in Turkey. In this study, we reviewed records of the patients who had been diagnosed with Kaposi sarcoma in the last decade in a single center and evaluated treatment results.

METHODS: Medical records of the Kaposi sarcoma patients seen in the last decade in a single tertiary center were evaluated in detail.

RESULTS: A total of 27 patients were identified; 18 patients had been checked for HHV8; and 16 of those patients were positive for HHV8. One patient was positive for HIV. Two patients having mycosis fungoides and myasthenia graves developed iatrogenic KS after immunosuppressive treatments. Interferon α was used in 21 patients; 8 patients achieved complete response; and 11 patients achieved a partial response. No serious side effects were observed. Systemic involvement did not develop in any patients except lymph node involvement in a patient with widespread skin lesions.

PMID:34525493 | DOI:10.1111/jocd.14426

Conjunctival Kaposi's Sarcoma

Fetched: September 16th, 2021, 5:02am GMT by Filipe Gouveia-Moraes

N Engl J Med. 2021 Sep 16;385(12):e36. doi: 10.1056/NEJMicm2105217.

NO ABSTRACT

PMID:34525288 | DOI:10.1056/NEJMicm2105217

CIC-NUTM1 Sarcomas Affecting the Spine: A Subset of CIC-Rearranged Sarcomas Commonly Present in the Axial Skeleton

Fetched: September 16th, 2021, 5:02am GMT by Shaomin Yang

Arch Pathol Lab Med. 2021 Sep 15. doi: 10.5858/arpa.2021-0153-OA. Online ahead of print.

ABSTRACT

CONTEXT.—: Tumors harboring CIC-NUTM1 fusion are a newly recognized rare sarcoma, but the documented cases are still limited. It is unclear whether it is the same as classic CIC-DUX4 sarcoma in terms of its clinical, pathologic, and behavioral aspects.

OBJECTIVE.—: To further explore the clinicopathologic characteristics of CIC-NUTM1 sarcoma.

DESIGN.—: The cases were diagnosed based on immunophenotype, next-generation sequencing, and fluorescence in situ hybridization tests and compared with the reported CIC-NUTM1 sarcomas in the literature.

RESULTS.—: Three cases of CIC-NUTM1 sarcomas involving the spine in adults were described. They were 2 men and 1 woman, aged 38 to 61 years. Two tumors were located in thoracic vertebrae and 1 in a cervical vertebra. All were locally advanced lesions destroying the bone and soft tissues without spinal cord involvement or metastasis. The tumors were composed of monomorphic small to medium-sized cells with round to epithelioid appearance. The architecture was lobulated and solid with diffuse or multifocal myxoid stroma. Next-generation sequencing revealed an in-frame fusion between CIC (exon 16 or 17) and NUTM1 (exon 5 or 6) in 3 cases. Fluorescence in situ hybridization confirmed CIC and NUTM1 breaks, and immunohistochemistry showed NUT staining in the nucleus. The patients died of disease 8 to 15 months (mean, 10.7 months) after presentation. Of the CIC-NUTM1 sarcomas reported in the literature along with our cases (n = 11), 8 cases developed in axial bone (5 spine, 3 skull base).

CONCLUSIONS.—: CIC-NUTM1 sarcomas showed distinct anatomic tropism for the axial skeleton and unfavorable behavior compared with classic CIC sarcoma.

PMID:34525172 | DOI:10.5858/arpa.2021-0153-OA

Permalink for 'Implementing a Machine Learning Strategy to Predict Pathologic Response in Patients With Soft Tissue Sarcomas Treated With Neoadjuvant Chemotherapy'

Implementing a Machine Learning Strategy to Predict Pathologic Response in Patients With Soft Tissue Sarcomas Treated With Neoadjuvant Chemotherapy

Fetched: September 16th, 2021, 5:02am GMT by Amandine Crombé

JCO Clin Cancer Inform. 2021 Sep;5:958-972. doi: 10.1200/CCI.21.00062.

ABSTRACT

PURPOSE: Neoadjuvant chemotherapy (NAC) has been increasingly used in patients with locally advanced high-risk soft tissue sarcomas in the past decade, but definition and prognostic impact of a good histologic response (GHR) are lacking. Our aim was to investigate which histologic feature from the post-NAC surgical specimen independently correlated with metastatic relapse-free survival (MFS) in combination with clinical, radiologic, and pathologic features using a machine learning approach.

METHODS: This retrospective study included 175 consecutive patients (median age: 59 years, 75 women) with resectable disease, treated with anthracycline-based NAC between 1989 and 2015 in our sarcoma reference center, and with quantitative histopathologic analysis of the surgical specimen. The outcome of interest was the MFS. A multimodel, multivariate survival analysis was used to define GHR. The added prognostic value of GHR was investigated through the comparisons with the standard model (including histologic grade, size, and depth) and SARCULATOR nomogram using concordance indices (c-index) and Monte-Carlo cross-validation.

RESULTS: Seventy-two patients (72 of 175, 41.1%) had a metastatic relapse. Stepwise Cox regression, random survival forests, and least absolute shrinkage and selection operator-penalized Cox regression all converged toward the same definition for GHR, ie, < 5% stainable tumor cells. The five-year MFS probability was 1 (95% CI, 1 to 1) in patients with GHR versus 0.73 (95% CI, 0.65 to 0.81) in patients without GHR (log-rank P = .0122). The final prognostic model incorporating the GHR was significantly better than the standard model and SARCULATOR (average c-index in testing sets = 0.72 [95% CI, 0.61 to 0.82] v 0.57 [95% CI, 0.44 to 0.70] and 0.54 [95% CI, 0.45 to 0.64], respectively; P = .0414 and .0091).

CONCLUSION: Histologic response to NAC improves the prediction of MFS in patients with soft tissue sarcoma and represents a possible end point in future studies exploring innovative regimens in the neoadjuvant setting.

PMID:34524884 | DOI:10.1200/CCI.21.00062

Leiomyosarcoma: Does Location of Primary Help to Determine the Best Systemic Therapy Options?

Fetched: September 16th, 2021, 5:02am GMT by Jan Philipp Novotny

Curr Treat Options Oncol. 2021 Sep 15;22(11):99. doi: 10.1007/s11864-021-00897-2.

ABSTRACT

Management of leiomyosarcoma is based on the specifics of each individual case. Specifically, the location of the disease and whether the disease is metastatic or localized and if localized disease, whether the tumor is resectable or unresectable. In patients with recurrent or metastatic disease, factors such as disease-free interval and pattern of spread should be considered within the context of treatment planning. In general, patients with metastatic disease are typically treated with systemic chemotherapy with either an anthracycline-based regimen or gemcitabine-based regimen as first-line therapy. Additional systemic options include trabectedin, pazopanib, eribulin, and DTIC. Uterine LMS has been the most studied site-specific LMS with respect to systemic therapy. The increasing use of tumor genomics may ultimately define subsets which may benefit from tailored systemic therapies.

PMID:34524549 | DOI:10.1007/s11864-021-00897-2

Retroperitoneal soft tissue sarcoma: role of radiotherapy

Fetched: September 16th, 2021, 5:02am GMT by Alexander Nieto

Chirurg. 2021 Sep 15. doi: 10.1007/s00104-021-01498-3. Online ahead of print.

ABSTRACT

BACKGROUND: Retroperitoneal soft tissue sarcomas (RPS) include tumors of mesenchymal origin with overall well-defined histological subtypes and heterogenic prognosis. For the first time with the publication of the STRASS study, which investigated the value of neoadjuvant radiotherapy in primary RPS, there is phase III evidence for the use of radiotherapy.

OBJECTIVE: The primary objective of the present article is to present the role of neoadjuvant radiotherapy in RPS since the publication of the STRASS study.

MATERIAL AND METHODS: We performed a non-systematic literature search. The results of retrospective and observational studies were compared to those of the STRASS study.

RESULTS: In the two of the largest analyses, the surveillance, epidemiology, and end results program (SEER) and the American National Cancer Database (NCDB), an improvement in overall survival due to radiotherapy in RPS could be shown. In contrast to these results, there was no significant improvement in 3‑year abdominal recurrence-free survival in the STRASS study. There was solely a trend to improved abdominal recurrence-free survival in initially unplanned subgroup analyses for patients with liposarcoma as well as low-grade sarcoma but not for leiomyosarcoma or high-grade sarcoma.

CONCLUSION: Thanks to international collaboration an academic randomized trial was even feasible in such a rare disease as RPS. The results of the STRASS study have relativized the potential benefit of radiotherapy in RPS. A longer follow-up especially regarding the role of radiotherapy in liposarcomas is desirable.

PMID:34524488 | DOI:10.1007/s00104-021-01498-3

Permalink for 'Is compartmental surgery always mandatory in retroperitoneal liposarcoma? A case report of multifocal synchronous well-differentiated liposarcoma'

Is compartmental surgery always mandatory in retroperitoneal liposarcoma? A case report of multifocal synchronous well-differentiated liposarcoma

Fetched: September 16th, 2021, 5:02am GMT by Giuseppe Brisinda

Ann Ital Chir. 2021;92:419-423.

ABSTRACT

AIM: Sarcomas are rare tumors representing 0.7% of all cancer cases in adults, and approximately 15-20% of those occur in the retroperitoneum. Diagnosis is usually late. Liposarcoma and leiomyosarcoma are the most frequent forms. Liposarcomas have high local recurrence rates (35-60%) and a high metastasis rate only if dedifferentiated (30%), whereas leiomyosarcoma has a high distant metastasis rate (60%) and a low local recurrence rate (20%).

CASE REPORT: A case report of multifocal synchronous well-differentiated liposarcoma is presented. The patient underwent a surgical excision of all the masses. The postoperative period was uneventful, with a postoperative hospital stay of 9 days. The patient underwent systemic chemotherapy and clinical and instrumental follow-up. A relapse of the disease was observed 24 months after surgery: a 25 mm mass was diagnosed close to the pancreatic stump, as well as a 24 mm mass in the left upper abdominal quadrant. The patient underwent a second laparotomy: recurrent lesions were identified and excised en-bloc with the body of the pancreas.

CONCLUSION: Surgery is the gold standard of therapy. The best chance for curative resection is at the time of the first diagnosis of the disease. Compartmental surgery is a macroscopically complete resection through en bloc excision of adjacent structures, even if not clearly infiltrated. Many controversies still exist in the treatment of retroperitoneal liposarcoma, such as the extent of primary and secondary resections, the benefit of chemotherapy and radiation therapy, and when these treatments should be delivered.

KEY WORDS: Compartmental surgery, Liposarcoma, Surgery, Retroperitoneal sarcoma.

PMID:34524112

The ORF45 protein of Kaposi Sarcoma-associated Herpesvirus (KSHV) is an inhibitor of p53 signaling during viral reactivation

Fetched: September 16th, 2021, 5:02am GMT by Dina Alzhanova

J Virol. 2021 Sep 15:JVI0145921. doi: 10.1128/JVI.01459-21. Online ahead of print.

ABSTRACT

Kaposi Sarcoma-associated herpesvirus (KSHV) is a carcinogenic double-stranded DNA virus and the etiological agent of Kaposi's Sarcoma (KS), primary effusion lymphoma (PEL), and multicentric Castleman's Disease (MCD). To prevent premature apoptosis and support its replication cycle, KSHV expresses a series of open reading frames (ORFs) that regulate signaling by the p53 tumor suppressor protein. Here we describe a novel viral inhibitor of p53 encoded by KSHV ORF45 and identify its mechanism of action. ORF45 binds to p53 and prevents its interactions with USP7, a p53 deubiquitinase. This results in decreased accumulation, localization of p53 to the cytoplasm, and diminished transcriptional activity. IMPORTANCE Unlike in other cancers, the tumor suppressor protein p53 is rarely mutated in Kaposi Sarcoma (KS). Rather, Kaposi Sarcoma-associated herpesvirus (KSHV) inactivates p53 through multiple viral proteins. One possible therapeutic approach to KS is the activation of p53, which would result in apoptosis and tumor regression. In this regard, it is important to understand all the mechanisms used by KSHV to modulate p53 signaling. This work describes a novel inhibitor of p53 signaling and a potential drug target, ORF45, and identifies the mechanisms of its action.

PMID:34523970 | DOI:10.1128/JVI.01459-21

A new form of Kaposi sarcoma

Fetched: September 16th, 2021, 5:02am GMT by Leonie A J Derickx

Ned Tijdschr Geneeskd. 2021 Aug 19;165:D5966.

ABSTRACT

BACKGROUND: Kaposi sarcoma is an vascular neoplasm caused by infection with human herpesvirus-8. Known risk groups are Mediterranean, eastern European Jewish and African ancestry men or men with AIDS. Nowadays we distinguish more subgroups.

CASE DESCRIPTION: We present a healthy 39 year old man with a lesion on the right foot, having homosexual contacts, without HIV infection. Previous histology revealed signs of hemangioma. Recent clinical signs and histology confirmed multifocal Kaposi sarcoma. He was treated with radiotherapy. We also present a 65 year old MSM with and a lesion on the sole of the foot. Histology revealed for a solitary nodular Kaposi sarcoma. A short term relapse after surgical excision occurred.

CONCLUSION: Kaposi sarcoma is subdivided into different categories. Kaposi Sarcoma in HIV-negative MSM is seen more frequently today, yet usually shows an indolent course. An adjusted less aggressive treatment and follow-up is therefore justified.

PMID:34523837

Exercise intervention decreases acute and late doxorubicin-induced cardiotoxicity

Fetched: September 16th, 2021, 5:02am GMT by Fei Wang

Cancer Med. 2021 Sep 15. doi: 10.1002/cam4.4283. Online ahead of print.

ABSTRACT

BACKGROUND: Doxorubicin (Dox) is one of the most effective chemotherapy agents used to treat adolescent and young adult sarcoma patients. Unfortunately, Dox causes cardiotoxicities that compromise long-term survival. We investigated whether exercise prevented cardiotoxicity and increased survival following myocardial infarction.

METHODS: Juvenile mice received Dox, Dox + exercise (Exer), Dox then exercise or were exercised during and after Dox. Mice were evaluated by echocardiography and histology immediately after therapy and 12 weeks later. Mice subjected to permanent ligation of the left anterior descending artery 90 days after therapy were assessed for survival at 45 and 100 days.

RESULTS: Mice treated with Dox, but not Dox + Exer, had decreased ejection fraction (EF) and fractional shortening (FS) immediately after Dox therapy, which continued to deteriorate over 12 weeks with the development of diastolic failure and fibrosis. Acute Dox-induced cardiotoxicity was documented by induction of autophagy and abnormal mitochondria and vascular architecture with decreased pericytes. These abnormalities persisted 12 weeks after therapy. These acute and late changes were not seen in the Dox + Exer group. Initiating exercise after Dox therapy promoted recovery of EF and FS with no functional or histologic evidence of Dox-induced damage 12 weeks after therapy. Survival rates at 100 days after MI were 67% for control mice, 22% for mice that received Dox alone, and 56% for mice that received Dox + Exer.

CONCLUSIONS: Exercise inhibited both early and late Dox-induced cardiotoxicity and increased recovery from an ischemic event. Exercise interventions have the potential to decrease Dox-induced cardiac morbidity.

PMID:34523825 | DOI:10.1002/cam4.4283

Update and interpretation of 2021 National Comprehensive Cancer Network (NCCN) "Clinical Practice Guidelines for Bone Tumors"

Fetched: September 16th, 2021, 5:02am GMT by Ming Ni

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2021 Sep 15;35(9):1186-1191. doi: 10.7507/1002-1892.202103073.

ABSTRACT

The incidence of primary malignant bone tumors is low, and clinical cognition is insufficient. The establishment of diagnostic criteria is of great significance for prognosis of tumors. National Comprehensive Cancer Network (NCCN) regularly publishes "Clinical Practice Guidelines for Bone Tumors" to summarize the latest treatment progress of bone tumors. In the latest version of the guidelines released in November 2020, surgery is the main treatment for chondrosarcoma, chordoma, and giant cell tumor of bone, which can be combined with radiotherapy or targeted therapy. Ewing's sarcoma and osteosarcoma are treated by surgery combined with chemotherapy. Immunotherapy can be used to treat high-grade undifferentiated pleomorphic sarcoma. For recurrent tumors, surgery combined with radiotherapy, chemotherapy, and/or targeted therapy can be used for control. The guidelines provide a reference for the standard treatment of bone tumors.

PMID:34523287 | DOI:10.7507/1002-1892.202103073

Cesarean section in patient with metastatic Ewing sarcoma requiring VA-ECMO support

Fetched: September 16th, 2021, 5:02am GMT by Joao R Breda

J Card Surg. 2021 Sep 15. doi: 10.1111/jocs.15984. Online ahead of print.

ABSTRACT

A 26-year-old pregnant woman, with multiple metastatic Ewing sarcoma, presented with a sternal mass that began enlarging during pregnancy. Due to high-risk pregnancy, the patient was discussed in a multidisciplinary meeting and intubation was considered too risky without cardiopulmonary support. Computed tomography showed extrinsic tumor compression of the right ventricle outflow tract. Veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) was initiated before general anesthesia, followed by Cesarean section (C-section). VA ECMO was initiated with the patient in the awake position, ECMO support was discontinued when the patient had stable ventilation and hemodynamics. This case represents a unique indication of VA ECMO, during C-section, with maternal and fetal survival.

PMID:34523160 | DOI:10.1111/jocs.15984

Uterine mesenchymal tumors harboring ALK fusions and response to ALK-targeted therapy

Fetched: September 16th, 2021, 5:02am GMT by Chrisann Kyi

Gynecol Oncol Rep. 2021 Sep 1;37:100852. doi: 10.1016/j.gore.2021.100852. eCollection 2021 Aug.

ABSTRACT

Inflammatory myofibroblastic tumor (IMT) of the uterus is a rare but aggressive malignancy that is often misdiagnosed. Approximately 50% of uterine IMTs (UMT) harbor rearrangements involving the ALK gene on chromosome 2p23 with subsequent overexpression of the ALK protein. Molecular characterization and wider availability of immunohistochemistry (IHC) and next generation sequencing (NGS) have improved clinical recognition and accurate diagnosis of UMT. The discovery of ALK fusions as a genomic driver led to the FDA approval of ALK inhibitors in ALK-altered lung cancers, but there are limited data to date on the spectrum of ALK fusions or patterns of response and resistance to ALK inhibitors in ALK-altered UMT. In this report we describe the genomic and histopathological characteristics and the response to ALK-targeted therapy in four patients with UMT. In all four patients, clinical activity of ALK inhibition was observed, with durable responses lasting 12 months or more. Moreover, three patients derived benefit from a second-generation ALK inhibitor after progression of disease or intolerance to the first-generation inhibitor crizotinib. Our report advocates for consideration of expanding the current National Comprehensive Cancer Network (NCCN) guidelines to include later-generation ALK inhibitors for the treatment of ALK-rearranged UMTs.

PMID:34522753 | PMC:PMC8427213 | DOI:10.1016/j.gore.2021.100852

A Rare Entity of Undifferentiated Pleomorphic Sarcoma of the Mandible - A Case Report

Fetched: September 16th, 2021, 5:02am GMT by T S Archana

Ann Maxillofac Surg. 2021 Jan-Jun;11(1):156-159. doi: 10.4103/ams.ams_334_20. Epub 2021 Jul 24.

ABSTRACT

THE RATIONALE: Undifferentiated pleomorphic sarcoma originally known as malignant fibrous histiocytoma was declassified by the World Health Organization in 2002 as a formal diagnostic entity and renamed as an undifferentiated pleomorphic sarcoma. It accounts for <1% of malignant tumours of the long bone.

PATIENT CONCERNS: A 33-year-old male patient reported with swelling and pain in the lower left posterior jaw region for 3 months with a history of fall on the floor 3 months back.

DIAGNOSIS: On examination, diffuse solitary swelling was present on the left lower third of the face and was diagnosed radiographically and histopathologically as undifferentiated pleomorphic sarcoma of the mandible.

TREATMENT AND OUTCOMES: Selective neck dissection, followed by reconstruction with fibula osteomyocutaneous flap and then referred for adjuvant radiotherapy.

TAKE-AWAY LESSONS: Vimentin staining plays a substantial role in the diagnosis of undifferentiated pleomorphic sarcoma. A long-term follow-up after treatment is required to increase the chances of disease-free survival for the patients.

PMID:34522674 | PMC:PMC8407644 | DOI:10.4103/ams.ams_334_20

Permalink for 'Genetically predicted high circulating insulin-like growth factor-1 and insulin-like growth factor binding protein-3 increase the risks of soft tissue sarcoma'

Genetically predicted high circulating insulin-like growth factor-1 and insulin-like growth factor binding protein-3 increase the risks of soft tissue sarcoma

Fetched: September 16th, 2021, 5:02am GMT by Yifan Xu

Am J Cancer Res. 2021 Aug 15;11(8):3980-3989. eCollection 2021.

ABSTRACT

Insulin growth factor-1 (IGF-1) plays important roles in carcinogenesis. Previous studies have linked circulating IGF-1 and its main binding protein, insulin-like growth factor-binding protein-3 (IGFBP-3), to cancer risks. However, no study has been conducted in soft tissue sarcoma (STS). In this study, we investigated the relationship of genetically predicted circulating IGF-1 and IGFBP-3 with STS risks. Recent large genome-wide association studies (GWAS) have identified 413 single nucleotide polymorphisms (SNPs) associated with IGF-1 and 4 SNPs associated with IGFBP-3. We genotyped these SNPs in 821 patients and 851 healthy controls. We constructed weighted genetic risk scores (GRS) to predict circulating IGF-1 and IGFBP-3. We determined the associations of individual SNPs and GRS with the risks of STS using multivariate logistic regression analysis. We found high genetically predicted circulating IGF-1 and IGFBP-3 were both associated with increased STS risks. Dichotomized at the median values of IGF-1 and IGFBP-3 in controls, individuals with high level of IGF-1 exhibited a 27% increased risk of STS (odds ratio [OR]=1.27, 95% confidence interval [CI]=1.04-1.54, P=0.017), whereas the OR for high IGFBP-3 was 1.45 (95% CI=1.20-1.77, P<0.001). Interestingly, the significant association between IGFBP-3 and STS risk was only evident in women (OR=1.88, 95% CI=1.42-2.49, P<0.001), but not in men (OR=1.00, 95% CI=0.75-1.33, P=0.992). In stratified analyses by major STS subtypes, the strongest associations were observed in angiosarcoma for IGF-1, leiomyosarcoma for IGFBP-3, and gastrointestinal stromal tumors for IGFBP-3 in women. In conclusion, high circulating IGF-1 and IGFBP-3 levels were both associated with increased STS risks.

PMID:34522462 | PMC:PMC8414386

Comprehensive identification of FGFR1-4 alterations in 5 557 Chinese patients with solid tumors by next-generation sequencing

Fetched: September 16th, 2021, 5:02am GMT by Weiquan Gu

Am J Cancer Res. 2021 Aug 15;11(8):3893-3906. eCollection 2021.

ABSTRACT

Deregulation of fibroblast growth factor receptor (FGFR) network is common in cancer due to activating mutations, gene amplifications and chromosomal translocations. Currently, various FGFR inhibitors are being developed. In order to optimize their clinical applications, understanding the frequencies and types of FGFR alterations in multiple cancer types appears to be extremely important. This study characterized FGFR1-4 alterations in solid tumors by next-generation sequencing (NGS). Between Jun. 2019 and Aug. 2020, the sequencing data of 5 557 solid tumors of diverse types in the database of Simcere Diagnostics, Inc. (Nanjing, China) were retrospectively analyzed. A panel-based NGS assay was used to detect FGFR1-4 alterations in tumor samples. 9.2% of cancer cases had FGFR1-4 alterations, in which gene amplifications (51.5%) and mutations (40.7%) were frequent, whereas gene rearrangements were less common (10.0%). FGFR1 was involved in 4.6% of 5 557 cases, FGFR2 in 2.1%, FGFR3 in 1.6%, and FGFR4 in 1.4%. Of patients with FGFR1-4 alterations, TP53, MUC16, NSD3, MYC and LRP1B genes were the top 5 mutant genes. FGFR1-4 aberrations occurred in almost every type of solid tumors, with the most common tumor being endometrial carcinoma (22.2%), followed by sarcoma (17.3%), breast cancer (13.2%), gastric cancer (12.2%), and more. 0.6% of cancer cases harbored FGFR1-4 fusions, with the most common fusion partner being TACC3. Two cases of GBM harboring FGFR3-TACC3 fusions were responsive to anlotinib treatment. In conclusion, FGFR1-4 alterations are prevalent in solid tumors of diverse types, with the majority being gene amplifications and mutations. FGFR1-4 fusions only occur in a minority of cancer cases, and those with glioblastoma harboring FGFR3-TACC3 fusions may benefit from anlotinib.

PMID:34522456 | PMC:PMC8414391

Undifferentiated embryonal sarcoma masquerading as liver abscess: A case report with typical imaging features

Fetched: September 16th, 2021, 5:02am GMT by Ranjan K Patel

SA J Radiol. 2021 Aug 31;25(1):2179. doi: 10.4102/sajr.v25i1.2179. eCollection 2021.

ABSTRACT

Undifferentiated embryonal sarcoma (UES) is an uncommon paediatric hepatic tumour that clinically simulates a liver abscess when present with fever. This report describes a case of UES in a 12-year-old boy, who presented with abdominal pain, swelling and fever, all simulating a liver abscess. The possibility of UES was considered at imaging, based on the solid appearance on ultrasound and cystic appearance with serpiginous peripheral vessels on computed tomography/magnetic resonance imaging. The diagnosis was confirmed at histopathology.

PMID:34522435 | PMC:PMC8424748 | DOI:10.4102/sajr.v25i1.2179

Angiosarcoma of the pulmonary artery mimicking pulmonary thromboembolism

Fetched: September 16th, 2021, 5:02am GMT by Sitang Nirattisaikul

SA J Radiol. 2021 Aug 10;25(1):2150. doi: 10.4102/sajr.v25i1.2150. eCollection 2021.

ABSTRACT

Primary pulmonary angiosarcomas (PPAs) are rare, and often, their diagnosis is delayed because of insidious clinical symptoms and imaging findings mimicking pulmonary thromboembolism (PE). A 33-year-old female patient presented with chest pain and progressive dyspnoea. Her initial diagnosis, based on clinical symptoms and CT pulmonary angiography (PA) findings, was PE. However, after treatment with anticoagulants, the patient failed to improve. A follow-up CTPA and further CT-guided biopsy results were compatible with angiosarcoma.

PMID:34522432 | PMC:PMC8424761 | DOI:10.4102/sajr.v25i1.2150

Targeting mutated GTPase KRAS in tumor therapies

Fetched: September 15th, 2021, 5:02am GMT by Guangjin Fan

Eur J Med Chem. 2021 Sep 4;226:113816. doi: 10.1016/j.ejmech.2021.113816. Online ahead of print.

ABSTRACT

Kirsten rat sarcoma virus oncogene (KRAS) mutation accounts for approximately 85% of RAS-driven cancers, and participates in multiple signaling pathways and mediates cell proliferation, differentiation and metabolism. KRAS has been considered as an "undruggable" target due to the lack of effective direct inhibitors, although high frequency of KRAS mutations have been identified in multiple carcinomas in the past decades. Encouragingly, the KRAS^G12C inhibitor AMG510 (sotorasib), which has been approved for treating NSCLC and CRC recently, makes directly targeting KRAS the most promising strategy for cancer therapy. To better understand the current state of KRAS inhibitors, this review summarizes the biological functions of KRAS, the structure-activity relationship studies of the small-molecule inhibitors that directly target KRAS, and highlights the therapeutic agents with improved selectivity, bioavailability and physicochemical properties. Furthermore, the combined medication that can enhance efficacy and overcome drug resistance of KRAS covalent inhibitors is also reviewed.

PMID:34520956 | DOI:10.1016/j.ejmech.2021.113816

Pediatric liposarcoma: A case series and literature review

Fetched: September 15th, 2021, 5:02am GMT by Yiressy Izaguirre Baday

Pediatr Blood Cancer. 2021 Sep 14:e29327. doi: 10.1002/pbc.29327. Online ahead of print.

ABSTRACT

Liposarcoma is arare soft tissue sarcoma in children. While prognosis, clinical behavior, and response to therapy among the various histologic subtypes are well described in adults, data in children are limited. Here, we describe our experience treating 14 children with liposarcoma at a large, academic pediatric center and review the available pediatric literature. This comprehensive report adds treatment, survival, and genomic data to pediatric liposarcoma literature.

PMID:34520106 | DOI:10.1002/pbc.29327

3D Biofabricated In Vitro Models of Vascularized and Mineralized Bone Tissues

Fetched: September 15th, 2021, 5:02am GMT by Maria Vittoria Colombo

Methods Mol Biol. 2022;2373:283-296. doi: 10.1007/978-1-0716-1693-2_17.

ABSTRACT

This protocol describes a comprehensive practical guide for the biofabrication of 3D in vitro models of vascularized and mineralized bone Minitissues. These models give the possibility to study the contribution of physical and biochemical parameters on bone vascularization, as well as the osteoblast/osteoclast mediated matrix remodeling. Based on the specific pathophysiological processes to be investigated, the 3D bone Minitissues allow to select the most suitable cell composition, by coculturing up to four cell types, and to customize the material properties of the hydrogel matrix. Considering their versatility, these 3D bone Minitissues could be relevant for the recapitulation of bone pathologies such as bone tumors and metastases and could be and used as screening platforms to test antimetastatic drugs.

PMID:34520019 | DOI:10.1007/978-1-0716-1693-2_17

Prognostic implications of histological organ involvement in retroperitoneal sarcoma

Fetched: September 15th, 2021, 5:02am GMT by R Tyler

BJS Open. 2021 Sep 6;5(5):zrab080. doi: 10.1093/bjsopen/zrab080.

ABSTRACT

BACKGROUND: The prognostic significance of histological organ involvement by retroperitoneal sarcoma subtype is unknown. The present study aimed to describe organ involvement across the subtypes, and the implications for survival.

METHODS: Patients undergoing surgery for primary retroperitoneal sarcoma at the Queen Elizabeth Hospital, Birmingham from April 2005 to September 2018 were identified retrospectively. Histological organ involvement was classed as pushing, infiltrative or neither. Univariable and multivariable Cox regression models were produced to analyse the association between histological organ involvement and both overall (OS) and recurrence-free (RFS) survival for the cohort as a whole, and by histological subtype.

RESULTS: Data were available for a total of 197 patients, of whom 171 (86.8 per cent) had at least one organ resected. Infiltrative organ behaviour was seen in 37 patients (18.8 per cent), and pushing behaviour in 67 (34.0 per cent). For the cohort as a whole, infiltration (hazard ratio (HR) 4.32, 95 per cent c.i. 2.35 to 7.93; P < 0.001), but not pushing (HR 1.62, 0.90 to 2.92; P = 0.106), was associated with significantly shorter OS, in comparisons with a group with neither of these behaviours. However, this effect was found to differ significantly by histological subtype (P = 0.009). For patients with dedifferentiated liposarcoma, there was no significant association between tumour behaviour and either OS (P = 0.508) or RFS (P = 0.313). However, in leiomyosarcoma, infiltrative behaviour was associated with shorter OS (P = 0.002), and both infiltrative (P < 0.001) and pushing (P = 0.010) behaviours were associated with shorter RFS, compared with tumours with neither behaviour. Multivariable analyses of both OS and RFS returned similar results.

CONCLUSION: The prognostic implications of organ involvement in retroperitoneal sarcoma vary by histological subtype.

PMID:34518873 | DOI:10.1093/bjsopen/zrab080

Unusual lung tumors-from morphology to genetics

Fetched: September 15th, 2021, 5:02am GMT by Jennifer M Boland

Mod Pathol. 2021 Sep 13. doi: 10.1038/s41379-021-00914-7. Online ahead of print.

ABSTRACT

Most pathologists are well versed in the diagnosis of lung cancer, given the common nature of the disease. Occasionally more unusual neoplasms are encountered in lung biopsies and resections, which may be difficult to distinguish from "run of the mill" lung cancer cases based on overlapping morphologic and immunophenotypic features. The accurate diagnosis of these rare entities is quite challenging and requires careful morphological examination paired with judicious use of ancillary immunohistochemical and genetic studies. Herein, the clinicopathological and genetic features of five unusual lung tumors will be reviewed, including thoracic SMARCA4-deficient undifferentiated tumor, NUT carcinoma, sclerosing pneumocytoma, primary pulmonary myxoid sarcoma/angiomatoid fibrous histiocytoma, and bronchiolar adenoma/ ciliated muconodular papillary tumor. Since recognition of these entities by pathologists is of increasing importance to guide prognosis and therapy, emphasis will be placed on practical tips to reach these rare diagnoses with confidence.

PMID:34518633 | DOI:10.1038/s41379-021-00914-7

Permalink for 'A Phase 1 Study of an MPS1 Inhibitor (BAY 1217389) in Combination with Paclitaxel using a novel Randomized Continual Reassessment Method for Dose Escalation'

A Phase 1 Study of an MPS1 Inhibitor (BAY 1217389) in Combination with Paclitaxel using a novel Randomized Continual Reassessment Method for Dose Escalation

Fetched: September 15th, 2021, 5:02am GMT by Florence Atrafi

Clin Cancer Res. 2021 Sep 13:clincanres.4185.2020. doi: 10.1158/1078-0432.CCR-20-4185. Online ahead of print.

ABSTRACT

PURPOSE: MPS1 kinase inhibitor, BAY 1217389 (BAY) synergizes with paclitaxel. This phase 1 study assessed the combination of BAY with paclitaxel using a novel randomized continuous reassessment method (rCRM) to improve dose determination.

EXPERIMENTAL DESIGN: Patients with solid tumors were randomized to receive oral BAY (BID 2‑days‑on/5‑days‑off) with weekly paclitaxel (90 mg/m²) or paclitaxel monotherapy in cycle 1. Dose escalation was guided by CRM modeling. Primary objectives were to assess safety, establish the maximum tolerated dose (MTD) of BAY and to evaluate the pharmacokinetic profiles for both compounds. Simulations were performed to determine the contribution of the rCRM for dose determination.

RESULTS: In total, 75 patients were enrolled. The main dose limiting toxicities were hematologic toxicities (55.6%). The MTD of BAY was established at 64 mg BID with paclitaxel. Inclusion of a control arm enabled the definitive attribution of grade {greater than or equal to}3 neutropenia to higher BAY exposure (AUC0-12 (P< .001)). After determining the MTD, we included nineteen breast cancer patients at this dose for dose expansion. Other common toxicities were nausea (45.3%), fatigue (41.3%) and diarrhea (40.0%). Overall confirmed responses were seen in 31.6% of evaluable patients. Simulations showed that rCRM outperforms traditional designs in determining the true MTD.

CONCLUSIONS: The combination of BAY with paclitaxel was associated with considerable toxicity without a therapeutic window. However, the use of the rCRM design enabled us to determine the exposure-toxicity relation for BAY. Therefore we propose that the rCRM could improve dose determination in phase I trials that combine agents with overlapping toxicities.

PMID:34518310 | DOI:10.1158/1078-0432.CCR-20-4185

Carcinosarcoma of the Cervix: A Case Report

Fetched: September 15th, 2021, 5:02am GMT by Birendra Bhagat

JNMA J Nepal Med Assoc. 2021 Aug 12;59(240):814-817. doi: 10.31729/jnma.6566.

ABSTRACT

Cervical carcinoma is the most common cause of mortality due to cancer in Nepal. Carcinosarcoma is a very rare subtype of cervical cancer which is characterized by the presence of both epithelial and mesenchymal malignant component. It constitutes less than 1% of cervical carcinoma. Due to the low occurrence of the disease, most of the data on treatment and prognosis are based on case reports and series. Here, we report a case of 69 years, female with cervical cancer (FIGO IIA2). Histopathological and immunohistochemical analysis of cervical biopsy initially showed primary adenosarcoma of the cervix. The tumor was non-responsive to primary treatment with concurrent chemoradiation. Later she was treated with abdominal hysterectomy and bilateral salpingo-oophorectomy. The final histopathology of the resected specimen showed a sarcomatous component along with carcinomatous changes in the endocervical glands favouring the diagnosis of carcinosarcoma of the cervix.

PMID:34508482 | DOI:10.31729/jnma.6566

Pulmonary metastasectomy in bone and soft tissue sarcoma with metastasis to the lung

Fetched: September 14th, 2021, 5:02am GMT by Charles A Gusho

Interact Cardiovasc Thorac Surg. 2021 Sep 13:ivab178. doi: 10.1093/icvts/ivab178. Online ahead of print.

ABSTRACT

OBJECTIVES: This study investigated the outcomes of sarcoma patients with lung metastases who underwent pulmonary metastasectomy (PM), compared to patients who underwent medical management alone. The secondary objective was to compare survival after PM between variables of interest.

METHODS: This was a retrospective review of 565 sarcoma patients with confirmed, isolated pulmonary metastasis identified from the Surveillance, Epidemiology and End Results database between 2010 and 2015. 1:4 propensity score matching was used to select PM and non-PM groups. The multivariable Cox proportional hazards model was used to analyse prognostic factors of disease-free survival (DFS).

RESULTS: Of the eligible 565 patients, 59 PM patients were matched to 202 non-PM patients in a final ratio of 3.4. After propensity matching, there were no significant differences in baseline characteristics between PM and non-PM patients. The median DFS after PM was 32 months (interquartile range 18-59), compared to 20 months (interquartile range 7-40) in patients without PM (P = 0.032). Using a multivariable Cox proportional hazards model, metastasectomy (hazard ratio 0.536, 95% confidence interval 0.33-0.85; P = 0.008) was associated with improved DFS. In a subset analysis of patients who underwent PM only, the median DFS was longer in males compared to females (P = 0.021), as well as in bone sarcoma compared to soft tissue sarcoma (P = 0.014).

CONCLUSIONS: For sarcoma patients with metastatic lung disease, PM appears to improve the prognosis compared to medical management. Furthermore, there may be a survival association with gender and tumour origin in patients who underwent PM. These data may be used to inform the surgical indications and eligibility criteria for metastasectomy in this setting.

PMID:34516633 | DOI:10.1093/icvts/ivab178

Intra-muscular follicular dendritic cell sarcoma in the thigh: A case report

Fetched: September 14th, 2021, 5:02am GMT by Yong Jin Cho

Medicine (Baltimore). 2021 Sep 10;100(36):e27209. doi: 10.1097/MD.0000000000027209.

ABSTRACT

RATIONALE: Follicular dendritic cell sarcoma (FDCS) is an intermediate-grade malignancy originating from follicular dendritic cells. Nodal FDCS is the most common type, meaning that the extranodal type may not be recognized and could be easily misdiagnosed. Reported extranodal sites include the head and neck, retroperitoneum, spleen, liver, and gastrointestinal tract. FDCS in the soft tissue is extremely rare.

PATIENT CONCERNS: A 75-year-old male presented with complaints of a localized swelling and intra-muscular soft tissue mass in the left upper thigh.

DIAGNOSIS: The present tumor consisted of fascicular or vague storiform-arranged spindle cells with less pleomorphism and many lymphoid aggregates. Tumor cells were positive for CD21, CD35, CD68, vimentin, and EGFR. Intra-muscular FDCS was confirmed by immunohistochemical studies.

INTERVENTIONS: The patient received a wide marginal excision, followed by adjuvant radiotherapy.

OUTCOMES: Symptomatic improvements were achieved and no subsequent relapses were observed.

LESSONS: If the tumor arises in the extranodal sites, especially in the soft tissue, it is difficult to include FDCS in the differential diagnosis. When the immunoprofile is not consistent with that of common spindle cell tumors, immunostaining for follicular dendritic cell markers such as CD21, CD23, and CD35, as well as further immunohistochemistry for D2-40, CD68, EGFR, Epstein-Barr virus, and BRAF can be helpful for the diagnosis and subtyping of FDCS. To the best of our knowledge, the present case is the first case of intramuscular FDCS.

PMID:34516525 | PMC:PMC8428715 | DOI:10.1097/MD.0000000000027209

Viral manipulation of a mechanoresponsive signaling axis disassembles processing bodies

Fetched: September 14th, 2021, 5:02am GMT by Elizabeth L Castle

Mol Cell Biol. 2021 Sep 13:MCB0039921. doi: 10.1128/MCB.00399-21. Online ahead of print.

ABSTRACT

Processing bodies (PBs) are ribonucleoprotein granules important for cytokine mRNA decay that are targeted for disassembly by many viruses. Kaposi's sarcoma-associated herpesvirus is the etiological agent of the inflammatory endothelial cancer, Kaposi's sarcoma, and a PB-regulating virus. The virus encodes Kaposin B (KapB), which induces actin stress fibres (SFs) and cell spindling as well as PB disassembly. We now show that KapB-mediated PB disassembly requires actin rearrangements, RhoA effectors and the mechanoresponsive transcription activator, YAP. Moreover, ectopic expression of active YAP or exposure of ECs to mechanical forces caused PB disassembly in the absence of KapB. We propose that the viral protein KapB activates a mechanoresponsive signaling axis and links changes in cell shape and cytoskeletal structures to enhanced inflammatory molecule expression using PB disassembly. Our work implies that cytoskeletal changes in other pathologies may similarly impact the inflammatory environment.

PMID:34516278 | DOI:10.1128/MCB.00399-21

Sequence Determines the Switch in the Fibril Forming Regions in the Low-Complexity FUS Protein and Its Variants

Fetched: September 14th, 2021, 5:02am GMT by Abhinaw Kumar

J Phys Chem Lett. 2021 Sep 13:9026-9032. doi: 10.1021/acs.jpclett.1c02310. Online ahead of print.

ABSTRACT

Residues spanning distinct regions of the low-complexity domain of the RNA-binding protein, Fused in Sarcoma (FUS-LC), form fibril structures with different core morphologies. Solid-state NMR experiments show that the 214-residue FUS-LC forms a fibril with an S-bend (core-1, residues 39-95), while the rest of the protein is disordered. In contrast, the fibrils of the C-terminal variant (FUS-LC-C; residues 111-214) have a U-bend topology (core-2, residues 112-150). Absence of the U-bend in FUS-LC implies that the two fibril cores do not coexist. Computer simulations show that these perplexing findings could be understood in terms of the population of sparsely populated fibril-like excited states in the monomer. The propensity to form core-1 is higher compared to core-2. We predict that core-2 forms only in truncated variants that do not contain the core-1 sequence. At the monomer level, sequence-dependent enthalpic effects determine the relative stabilities of the core-1 and core-2 topologies.

PMID:34516126 | DOI:10.1021/acs.jpclett.1c02310

Permalink for 'Identifying the Risk Factors and Estimating the Prognosis in Patients with Pelvis and Spine Ewing Sarcoma: A Population-Based Study'

Identifying the Risk Factors and Estimating the Prognosis in Patients with Pelvis and Spine Ewing Sarcoma: A Population-Based Study

Fetched: September 14th, 2021, 5:02am GMT by Runyi Jiang

Spine (Phila Pa 1976). 2021 Oct 1;46(19):1315-1325. doi: 10.1097/BRS.0000000000004022.

ABSTRACT

STUDY DESIGN: Retrospective analysis.

OBJECTIVE: The study was designed to: (1) figure out risk factors of metastasis; (2) explore prognostic factors and develop a nomogram for pelvis and spine Ewing sarcoma (PSES).

SUMMARY OF BACKGROUND DATA: Tools to predict survival of PSES are still insufficient. Nomogram has been widely developed in clinical oncology. Moreover, risk factors of PSES metastasis are still unclear.

METHODS: The data were collected and analyzed from the Surveillance, Epidemiology, and End Results (SEER) database. The optimal cutoff values of continuous variables were identified by X-tile software. The prognostic factors of survival were performed by Kaplan-Meier method and multivariate Cox proportional hazards modeling. Nomograms were further constructed for estimating 3- and 5-year cancer-specific survival (CSS) and overall survival (OS) by using R with rms package. Meanwhile, Pearson χ2 test or Fisher exact test, and logistic regression analysis were used to analyze the risk factors for the metastasis of PSES.

RESULTS: A total of 371 patients were included in this study. The 3- and 5-year CSS and OS rate were 65.8 ± 2.6%, 55.2 ± 2.9% and 64.3 ± 2.6%, 54.1 ± 2.8%, respectively. The year of diagnosis, tumor size, and lymph node invasion were associated with metastasis of patients with PSES. A nomogram was developed based on identified factors including: age, tumor extent, tumor size, and primary site surgery. The concordance index (C-index) of CSS and OS were 0.680 and 0.679, respectively. The calibration plot showed the similar trend of 3-year, 5-year CSS, and OS of PSES patients between nomogram-based prediction and actual observation, respectively.

CONCLUSION: PSES patients with earlier diagnostic year (before 2010), larger tumor size (>59 mm), and lymph node invasion, are more likely to have metastasis. We developed a nomogram based on age, tumor extent, tumor size, and surgical treatments for determining the prognosis for patients with PSES, while more external patient cohorts are warranted for validation.Level of Evidence: 3.

PMID:34517400 | DOI:10.1097/BRS.0000000000004022

Anaplastic Sarcoma of the Kidney With Heterologous Ganglioneuroblastic Differentiation: Another DICER1-Associated Tumor

Fetched: September 14th, 2021, 5:02am GMT by Lauren Kroll-Wheeler

Pediatr Dev Pathol. 2021 Sep 13:10935266211043861. doi: 10.1177/10935266211043861. Online ahead of print.

ABSTRACT

Anaplastic sarcoma of the kidney (ASK) is a rare renal tumor for which less than thirty cases have been described in the literature to date. Diagnosis of ASK is primarily based on histology, which features solid spindle cell neoplastic islands arranged in a fascicular pattern, prominent anaplastic nuclear morphology, brisk mitoses, and multiple multiloculated cysts lined by hobnail epithelium reminiscent of cystic nephroma. Chondroid or rhabdomyocytic differentiation is often present within the sarcoma. It has been recently suggested that this tumor entity belongs to the DICER1 syndrome tumors based on identification of DICER1 mutations. We report on a case of this rare tumor found in a twenty-month-old female. In addition to the typical histologic findings of ASK, this case also displayed heterologous neuroblastic-gangliocytic differentiation, which has not been previously described in the literature. TP53 and BRAF v600E had aberrant immunostaining. Chromosomal microarray and genomic sequencing revealed loss of chromosome 10 p15.3-p11.2 and both somatic and germline DICER1 mutations, consistent with recent research and further supporting the classification of this tumor within the DICER1 syndrome associated tumors.

PMID:34515577 | DOI:10.1177/10935266211043861

Permalink for 'Identified Enrollment Challenges of Adolescent and Young Adult Patients on the Nonchemotherapy Arm of Children's Oncology Group Study ARST1321'

Identified Enrollment Challenges of Adolescent and Young Adult Patients on the Nonchemotherapy Arm of Children's Oncology Group Study ARST1321

Fetched: September 14th, 2021, 5:02am GMT by Viswatej Avutu

J Adolesc Young Adult Oncol. 2021 Sep 9. doi: 10.1089/jayao.2021.0103. Online ahead of print.

ABSTRACT

ARST1321, a trial of patients with advanced soft tissue sarcoma, was the first National Clinical Trials Network study codeveloped by pediatric and adult consortia with two treatment cohorts. We report on the findings of a survey to identify barriers to enrolling adolescent and young adult patients (15-39 years) onto the nonchemotherapy arm. The survey response rate was 31% with a 70% completion rate. Common identified reasons for low accrual in order of decreasing frequency included insufficient funding, lack of study awareness or interest, competing trials, toxicity concerns, philosophical differences in the therapy backbone, and regulatory and infrastructure barriers. Clinical Trials.gov ID: NCT02180867.

PMID:34515544 | DOI:10.1089/jayao.2021.0103

Primary Renal BCOR-CCNB3 Fusion Sarcoma: A Case Report and Review of the Literature

Fetched: September 14th, 2021, 5:02am GMT by Ming Zhao

Urol Int. 2021 Sep 8:1-4. doi: 10.1159/000518563. Online ahead of print.

ABSTRACT

BCOR-CCNB3 fusion sarcoma is a recently described undifferentiated sarcoma with a novel recurrent inversion of 2 nearby genes BCOR and CCNB3. It typically affects bone and soft tissues of the pelvis, extremity, and paraspinal region and pursues variable clinical course. Primary renal BCOR-CCNB3 fusion sarcoma is very rare, and only a small number of cases have been documented. Accurate diagnosis is often challenging, and there is not any agreement for the treatment of this entity due to its rarity. We report findings of primary renal BCOR-CCNB3 fusion sarcoma in a 16-year-old boy with a brief review of the literature.

PMID:34515251 | DOI:10.1159/000518563

A Relapsing Meningeal Acute Myeloid Leukaemia FLT3-ITD+ Responding to Gilteritinib

Fetched: September 14th, 2021, 5:02am GMT by Salvatore Perrone

Chemotherapy. 2021 Aug 24:1-5. doi: 10.1159/000518356. Online ahead of print.

ABSTRACT

A patient with a therapy-related acute myeloid leukaemia (AML), NPM1mut, and FLT3-ITD+ was treated with induction and consolidation with CPX-351, obtaining a complete response (CR) but minimal residual disease persisted positive. Later, she complained progressive burning leg pain, weakening of the right hand and leg muscles, associated with absence of osteotendinous leg reflexes. Examination of cerebrospinal fluid (CSF) showed a meningeal relapse of AML. Moreover, a magnetic resonance imaging (MRI) showed 2 right meningeal implants of myeloid sarcoma and bone marrow revealed haematologic relapse of disease. She was treated with medicated lumbar punctures (LPs) followed by an FLA-Ida scheme, and she achieved a 2nd CR. Unfortunately, the patient developed hyperleucocytosis and reappearance of meningeal myeloid sarcoma at MRI. For this reason, a monotherapy with gilteritinib (an FLT3 inhibitor) was started: after 3 months of therapy, central nervous system (CNS)-disease shrunken and then faded, while AML in the bone marrow achieved only a partial response. This is the 1st report of a positive biological effect of gilteritinib on CNS (meningeal) myeloid sarcoma. There are no studies of gilteritinib concentration into CSF and penetration of gilteritinib into the blood-brain barrier should be further studied, given the paucity of drugs active on CNS relapse of AML. In patients receiving CPX-351 only, diagnostic LP should be considered after induction.

PMID:34515081 | DOI:10.1159/000518356

Cytology of Undifferentiated Round-Cell Sarcomas of Bone and Soft Tissue: Ewing Sarcoma or Not Ewing Sarcoma , That Is the Question

Fetched: September 14th, 2021, 5:02am GMT by Pawel Gajdzis

Acta Cytol. 2021 Aug 25:1-12. doi: 10.1159/000518146. Online ahead of print.

ABSTRACT

BACKGROUND: Undifferentiated round-cell sarcomas (URCSs) of soft tissue and bone are a group of clinically heterogeneous tumors. Diagnosis of these malignancies is based mainly on recurrent genetic alterations. The most common and the best known representative of this group is Ewing sarcoma (ES) which is characterized by gene fusions including EWSR1 or FUS and ETS transcription factors family. Other newly described entities are CIC-rearranged sarcoma, sarcoma with BCOR genetic alterations, and round-cell sarcoma with EWSR1-non-ETS fusions. All these novel tumors are known as Ewing-like sarcomas.

SUMMARY: It is believed that morphologic features of ES and Ewing-like sarcomas vary only slightly or even that cytomorphology is not relevant. But differences are usually obvious, and some cytologic findings, such as spindle cells, connective tissue fragments, or myxoid stroma, are typical for Ewing-like sarcomas but not for ES. Each of these entities is also characterized by different immunoprofiles. The aim of this review was to summarize cytomorphologic and immunohistochemical features of URCS and compare them with other small round-cell tumors. Key Messages: Cytology can be successfully used in URCS diagnosis as a complementary tool for core-needle biopsy or even alone in selected cases, especially in recurrent and metastatic tumors. Knowing the morphologic and immunohistochemical differences between URCS is essential to provide appropriate ancillary studies and make a definitive diagnosis.

PMID:34515032 | DOI:10.1159/000518146

Permalink for 'What Would Next Generation Sequencing Bring to the Diagnosis and Treatment of Sarcomas? A Series of 20 Cases, a Single Institution's Experience'

What Would Next Generation Sequencing Bring to the Diagnosis and Treatment of Sarcomas? A Series of 20 Cases, a Single Institution's Experience

Fetched: September 14th, 2021, 5:02am GMT by Ibrahim Kulac

Turk Patoloji Derg. 2021 Jun 26. doi: 10.5146/tjpath.2021.01544. Online ahead of print.

ABSTRACT

OBJECTIVE: Soft tissue tumors comprise a small proportion of a pathologist's routine practice. Although morphology and immunohistochemistry are quite helpful for diagnosing these tumors, many require molecular tests. Fluorescence in-situ hybridization has been the most commonly used method for the detection of specific genomic alteration, but next generation sequencing (NGS) could be more informative in many ways. Here we present our targeted NGS experience on soft tissue tumors with a series of 20 cases.

MATERIAL AND METHOD: The Laboratory Information System (LIS) was screened for soft tissue tumors that had been sequenced by NGS (between January 2018 - February 2021). 20 consecutive cases were included in the study. All cases were sequenced using a commercial targeted sequencing panel designed for soft tissue tumors.

RESULTS: We were able to run a reliable sequencing study for 16 (80%) of the cases but 4 (20%) of them failed in quality tests. We have found pathogenic alterations in 12 (60%) of the cases. The most common alterations were EWSR1 fusions, FLI1 being the most common partner. NGS results drastically changed the initial diagnosis, and thus the treatment modalities, in 3 cases (15%): the case with ETV6-NTRK3 fusion, the case with FUS-TFCP2 fusion, and the case of rhabdomyosarcoma (RMS) that was favored to be of the alveolar subtype and turned out to lack FOXO1 fusions.

CONCLUSION: A targeted NGS panel is robust and very informative. It not only allows pathologists to further specify and/or confirm their diagnosis but it could also play an important role in predicting the outcome.

PMID:34514574 | DOI:10.5146/tjpath.2021.01544

Multifocal Pseudomyogenic Hemangioendothelioma Involving the Scalp and Nose, Misdiagnosed as A Sarcoma: A Rare Case Report

Fetched: September 14th, 2021, 5:02am GMT by Neha Mittal

Turk Patoloji Derg. 2021 May 17. doi: 10.5146/tjpath.2021.01539. Online ahead of print.

ABSTRACT

This case report aims to present clinicopathological features of an extremely rare case of multifocal pseudomyogenic hemangioendothelioma (PMHE) in the scalp. A 21-year-old male developed multiple, focally ulcerated, nodules over the root of his nose and scalp. One of the skin lesions was sampled at another dermatology clinic, where this was diagnosed as a sarcoma. A review of biopsy sections showed well-circumscribed dermal lesions, comprising plump spindle and epithelioid cells, mimicking rhabdomyoblasts. Immunohistochemically, tumor cells were positive for AE1/AE3, CD31, FLI-1 and ERG. INI-1 was retained. A diagnosis of PMHE was offered. Subsequently, the patient underwent wide excision and has been asymptomatic for 8 months, post-surgery. PMHE is rarely reported in the head and neck region, where it can constitute a diagnostic pitfall. Awareness of this tumor and appropriate immunohistochemical stains are necessary for its timely diagnosis, in order to avoid radical treatments. A review of similar, previously documented cases is presented.

PMID:34514569 | DOI:10.5146/tjpath.2021.01539

Permalink for 'Clinicopathologic and Molecular Analysis of Inflammatory Pseudotumor-Like Follicular/Fibroblastic Dendritic Cell Sarcoma: A Case Report and Review of Literature'

Clinicopathologic and Molecular Analysis of Inflammatory Pseudotumor-Like Follicular/Fibroblastic Dendritic Cell Sarcoma: A Case Report and Review of Literature

Fetched: September 14th, 2021, 5:02am GMT by Bryan Morales-Vargas

Turk Patoloji Derg. 2021 Feb 8. doi: 10.5146/tjpath.2021.01523. Online ahead of print.

ABSTRACT

Inflammatory pseudotumor-like follicular/fibroblastic dendritic cell (FDC/FRC) sarcoma is an extremely rare neoplasm of the spleen associated with EBV and characterized by spindle cell morphology, dense mixed chronic inflammatory background, and a broad immunophenotypic profile often causing a diagnostic challenge for pathologists. The molecular features of FDC/FRC sarcoma are largely unknown due to a lack of comprehensive data. Here we present the results of next-generation sequencing and Single Nucleotide Polymorphism Copy Number array analysis in a case of FDC/FRC and review the literature.

PMID:34514557 | DOI:10.5146/tjpath.2021.01523

Permalink for 'SIX1 Activation Is Involved in Cell Proliferation, Migration, and Anti-inflammation of Acute Ischemia/Reperfusion Injury in Mice'

SIX1 Activation Is Involved in Cell Proliferation, Migration, and Anti-inflammation of Acute Ischemia/Reperfusion Injury in Mice

Fetched: September 14th, 2021, 5:02am GMT by Yong Jin

Front Mol Biosci. 2021 Aug 26;8:725319. doi: 10.3389/fmolb.2021.725319. eCollection 2021.

ABSTRACT

Nephrogenic proteins are re-expressed after ischemia/reperfusion (I/R) injury; however, the role of these proteins is still unknown. We found that sine oculis homeobox 1 (SIX1), a developmentally regulated homeoprotein, is reactivated in tubular epithelial cells after I/R injury associated with cell proliferation/migration and anti-inflammation. We demonstrated that SIX1 promoted cell proliferation by upregulating cyclin and glycolytic genes, and might increase cell migration by upregulating the expression of matrix metalloproteinase 9 (MMP9) directly or indirectly in the cell model. Notably, SIX1 targeted the promoters of the amino-terminal enhancer of split (AES) and fused in sarcoma (FUS), which are cofactors of nuclear factor-κB (NF-κB) subunit RELA, and then inhibited the transactivation function of RELA. The expression of monocyte chemotactic protein-1 (MCP-1) was decreased by the SIX1-mediated NF-κB pathway. Our results showed that the expression of cyclin, glycolytic genes, and MMP9 were significantly increased, and the infiltration of monocytes/macrophages (Mophs) was suppressed in SIX1 overexpression kidney at 1, 2, and 3 days after reperfusion. The overexpression of SIX1 resulted in reducing kidney damage from I/R injury in mice by promoting cell proliferation and migration and by inhibiting inflammation. Our study provides evidence that SIX1 involved in cell proliferation, migration, and anti-inflammation in the I/R model, which might be a potential therapeutic target that could be used to ameliorate kidney damage.

PMID:34513929 | PMC:PMC8427868 | DOI:10.3389/fmolb.2021.725319

Immunotherapy for the Treatment of Cutaneous Squamous Cell Carcinoma

Fetched: September 14th, 2021, 5:02am GMT by Andrea Boutros

Front Oncol. 2021 Aug 26;11:733917. doi: 10.3389/fonc.2021.733917. eCollection 2021.

ABSTRACT

Cutaneous squamous cell carcinoma (CSCC) accounts for approximately 20% of all keratinocytic tumors. In most cases, the diagnosis and treatments are made on small, low-risk lesions. However, in about 5% of cases, CSCC may present as either locally advanced or metastatic (i.e. with locoregional lymph nodes metastases or distant localizations). Prior to the introduction of immunotherapy in clinical practice, the standard treatment of advanced CSCC was not clearly defined, and up to 60% of patients received no systemic therapy. Thanks to a strong pre-clinical rationale, clinical trials led to the FDA (Food and Drug Administration) and EMA (European Medicines Agency) registration of cemiplimab, a PD-1 inhibitor that achieved encouraging results in terms of objective response, overall survival, and quality of life. Subsequently, the anti-PD-1 pembrolizumab received the approval for the treatment of advanced CSCC by the FDA only. In this review, we will focus on the definition of advanced CSCC and on the current and future therapeutic options, with a particular regard for immunotherapy.

PMID:34513710 | PMC:PMC8427439 | DOI:10.3389/fonc.2021.733917

Permalink for 'Prognostic Impact of Sarcopenia on Clinical Outcomes in Malignancies Treated With Immune Checkpoint Inhibitors: A Systematic Review and Meta-Analysis'

Prognostic Impact of Sarcopenia on Clinical Outcomes in Malignancies Treated With Immune Checkpoint Inhibitors: A Systematic Review and Meta-Analysis

Fetched: September 14th, 2021, 5:02am GMT by Shuluan Li

Front Oncol. 2021 Aug 26;11:726257. doi: 10.3389/fonc.2021.726257. eCollection 2021.

ABSTRACT

BACKGROUND: The effect of sarcopenia on the clinical outcomes of patients with malignant neoplasms receiving immune checkpoint inhibitors (ICIs) is unclear. The aim of this study was to evaluate the effect and survival of patients with malignancies and sarcopenia receiving ICIs.

METHODS: We systematically searched related studies in PubMed, Embase, and Cochrane Library up to March 2021 according to the inclusion and exclusion criteria. Information pertaining to the hazard ratio (HR) corresponding to 95% confidence interval (CI) of overall survival (OS) and progression-free survival (PFS) as determined by univariate and multivariate analyses; the odds ratio (OR) corresponding to the 95% CI of the disease control rate (DCR) and objective response rate (ORR); and immune-related adverse events (irAEs) was collected and analyzed using the RevMan 5.4 software. Study heterogeneity and sensitivity were also assessed.

RESULTS: A total of 19 studies were finalized that included 1763patients with lung, gastrointestinal, and head and neck cancers as well as those with melanoma, renal cell carcinoma, urothelial carcinoma, pancreatic cancer, and soft tissue sarcoma. According to univariate and multivariate analyses, patients with sarcopenia at pre-immunotherapy had poorer PFS and OS than those without. HRs and the corresponding 95% CI of PFS were 1.91(1.55-2.34, p <0.00001) and 1.46 (1.20-1.78, p =0.0001), respectively, and HRs and the corresponding 95% CI of OS were 1.78 (1.47-2.14, p <0.00001) and 1.73 (1.36-2.19, p <0.0001), respectively. Patients with sarcopenia showed poor PFS and OS during treatment. In addition, patients with sarcopenia had worse ORR (OR 0.46, 95% CI 0.28-0.74, p = 0.001) and DCR (OR 0.44, 95% CI 0.31-0.64, p<0.0001); however, the incidence of irAEs of any grade and high-grade in patients with sarcopenia did not increase, OR and the corresponding 95% CI were 0.58(0.30-1.12, p = 0.10) and 0.46(0.19-1.09, p = 0.08). Further, we performed subgroup analysis, skeletal muscle mass index (SMI) and psoas muscle mass index (PMI) stratification. In the SMI group, patients with sarcopenia had poor ORR, DCR, PFS, and OS than those without. In the PMI group, sarcopenia had poor ORR,DCR, and was a poor prognostic factor for PFS and OS according to univariate analysis but had no effect on PFS and OS according to multivariate analysis.

CONCLUSIONS: Patients with malignancies and sarcopenia at pre-immunotherapy or follow-up visits had poorer clinical outcomes than those without, and sarcopenia was a poor predictive factor of ICI immunotherapy outcomes.

PMID:34513704 | PMC:PMC8427761 | DOI:10.3389/fonc.2021.726257

Permalink for 'Synchronous Malignant Gastrointestinal Neuroectodermal Tumor and SMARCA4-Deficient Undifferentiated Carcinoma With Independent Origins in the Small Intestine: A Case Report'

Synchronous Malignant Gastrointestinal Neuroectodermal Tumor and SMARCA4-Deficient Undifferentiated Carcinoma With Independent Origins in the Small Intestine: A Case Report

Fetched: September 14th, 2021, 5:02am GMT by Cuimin Chen

Front Oncol. 2021 Aug 27;11:665056. doi: 10.3389/fonc.2021.665056. eCollection 2021.

ABSTRACT

BACKGROUND: Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare malignant mesenchymal neoplasm that commonly arises in the small bowel, stomach or colon. Meanwhile, SMARCA4-deficient undifferentiated carcinoma is a rarely reported entity with highly aggressive behavior that may involve the ovary, lung, gastrointestinal (GI) tract, endometrium and other organs. To our knowledge, we describe for the first time, an extremely rare case of synchronous GNET and SMARCA4-deficient undifferentiated carcinoma with independent origins in the small intestine.

CASE PRESENTATION: A 46-year-old woman presented with multiple small intestine masses and underwent surgical resection. Two distinct entities, GNET and SMARCA4-deficient undifferentiated carcinoma, were identified. GNET was composed of epithelioid and spindle cells with clear or eosinophilic cytoplasm arranged in sheets, nest, papillary, fascicular, palisade, rosette like or pseudoalveolar pattern. The neoplastic cells were positive for S-100 and SOX-10. Ewing sarcoma breakpoint region 1 gene (EWSR1) rearrangement was confirmed by fluorescence in situ hybridization (FISH), and EWSR1-CREB1 fusion was revealed by next-generation sequencing (NGS). SMARCA4-deficient undifferentiated carcinoma was composed mainly of poorly adhesive rhabdoid cells with eosinophilic cytoplasm arranged in a diffuse pattern. Multifocal necrosis, brisk mitotic figures as well as multinucleated tumor cells were observed. The neoplastic cells diffusely expressed pancytokeratin and vimentin, and was negative for SMARCA4(BRG1). Frame shift mutation of SMARCA4 was detected by NGS.

CONCLUSIONS: This is the first report that GNET and SMARCA4-deficient undifferentiated carcinoma occurred simultaneously in the small intestine, with the latter showing multiple involvement of the jejunum and ileum. The potential mechanism underlying co-existence of these two rare malignancies is unknown and need further investigations and concern.

PMID:34513665 | PMC:PMC8429901 | DOI:10.3389/fonc.2021.665056

Liver transplantation in malignant disease

Fetched: September 14th, 2021, 5:02am GMT by Sven Arke Lang

World J Clin Oncol. 2021 Aug 24;12(8):623-645. doi: 10.5306/wjco.v12.i8.623.

ABSTRACT

Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology. Following the implementation of the Milan criteria, hepatocellular carcinoma (HCC) was the first generally accepted indication for transplantation in patients with cancer. Subsequently, more liberal criteria for HCC have been developed, and research on this topic is still ongoing. The evident success of liver transplantation for HCC has led to the attempt to extend its indication to other malignancies. Regarding perihilar cholangiocarcinoma, more and more evidence supports the use of liver transplantation, especially after neoadjuvant therapy. In addition, some data also show a benefit for selected patients with very early stage intrahepatic cholangiocarcinoma. Hepatic epithelioid hemangioendothelioma is a very rare but nonetheless established indication for liver transplantation in primary liver cancer. In contrast, patients with hepatic angiosarcoma are currently not considered to be optimal candidates. In secondary liver tumors, neuroendocrine cancer liver metastases are an accepted but comparability rare indication for liver transplantation. Recently, some evidence has been published supporting the use of liver transplantation even for colorectal liver metastases. This review summarizes the current evidence for liver transplantation for primary and secondary liver cancer.

PMID:34513597 | PMC:PMC8394155 | DOI:10.5306/wjco.v12.i8.623

Real-World Outcome and Prognostic Factors of Pazopanib in Advanced Soft Tissue Sarcoma

Fetched: September 14th, 2021, 5:02am GMT by Bader Alshamsan

Cancer Manag Res. 2021 Aug 29;13:6755-6766. doi: 10.2147/CMAR.S323499. eCollection 2021.

ABSTRACT

PURPOSE: Pazopanib has been approved for treating soft tissue sarcomas (STS) after chemotherapy. We aimed to evaluate the prognostic factors, clinical outcomes, and tolerability of pazopanib in patients with STS.

PATIENTS AND METHODS: Forty-five patients treated between June 2015 and August 2019 were reviewed. Clinical outcome was measured by assessing the disease control rate (DCR) using Response Evaluation Criteria in Solid Tumors (version 1.1). Progression-free survival (PFS) and overall survival (OS) were estimated using the Kaplan-Meier method. Adverse effects were assessed using the Common Terminology Criteria for Adverse Events (version 5.0).

RESULTS: The median age of patients at diagnosis was 28 (interquartile range (IQR), 23-45) years. Pazopanib was used as the second-line treatment in 46.7% and the subsequent line in 53.3% of patients. The overall DCR was 55.6%, and at 8 and 12 weeks, it was 52.3% and 35.5%, respectively; the median duration of response was 7 (IQR: 2-18) months. Pazopanib-induced hypothyroidism was associated with DCR, with an odds ratio of 7 (95% confidence interval [95% CI: 1.7-27.5], p<0.01). The median PFS and OS were 4.1 (95% CI: 0.85-7.42) and 12.4 months (95% CI: 6.5-18.36), respectively. Hypothyroidism and response to pazopanib, better ECOG PS, histological subtypes desmoid tumor/aggressive fibromatosis (DT/AF), and alveolar soft part sarcoma (ASPS) were favorable prognostic factors for PFS. Hypothyroidism and response to pazopanib were significant favorable factors for OS. There was no statistical difference in the OS between patients using pazopanib as the second-line therapy and those using it as the subsequent-line therapy.

CONCLUSION: Pazopanib is an effective treatment for STS. However, it showed variability in the clinical outcome in favor of ASPS and an outstanding response in the DT/AF subtype. Pazopanib-induced hypothyroidism is a good prognostic factor for disease control and is associated with prolonged PFS and OS.

PMID:34512015 | PMC:PMC8414075 | DOI:10.2147/CMAR.S323499

Permalink for 'Expression, Clinical Significance, and Prospective Pathway Signaling of miR-501-3p in Ovarian Cancer Based on Database and Informatics Analysis'

Expression, Clinical Significance, and Prospective Pathway Signaling of miR-501-3p in Ovarian Cancer Based on Database and Informatics Analysis

Fetched: September 14th, 2021, 5:02am GMT by Buze Chen

Int J Gen Med. 2021 Sep 3;14:5193-5201. doi: 10.2147/IJGM.S327673. eCollection 2021.

ABSTRACT

BACKGROUND: The present study aims to explore the expression, clinical significance, and prospective pathway signaling of miR-501-3p in ovarian cancer (OC) based on database and informatics analysis.

METHODS: Kruskal-Wallis test, Wilcoxon sign-rank test, and logistic regression were used to evaluate the relationship between clinical features and miR-501-3p expression. Kaplan-Meier survival curve analysis was used to explore the relationship between miR-501-3p expression and the prognosis of OC patients. The miRNA targets were obtained from databases TargetScan, miRanda, TarBase, miRTarBase, miR2Disease, miRecords, and miRWalk. GO and KEGG analyses were used to analyze the significant involvement of miR-501-3p target genes in function.

RESULTS: The low miR-501-3p expression in OC was significantly associated with histologic grade (P=0.015). Low miR-501-3p expression predicted a poorer overall survival (HR: 0.77; 95% CI: 0.61-0.96; P=0.02) and disease-specific survival (HR: 0.77; 95% CI: 0.61-0.99; P=0.038). GO and KEGG analyses demonstrated that miR-501-3p might participate in the development of OC by pathways including one carbon pool by folate, protein digestion and absorption, cell cycle, kaposi sarcoma-associated herpesvirus infection, and viral carcinogenesis.

CONCLUSIONS: Low miR-501-3p expression is significantly associated with poor survival in OC patients. It may be a promising prognostic biomarker for OC patients.

PMID:34512002 | PMC:PMC8423717 | DOI:10.2147/IJGM.S327673

Permalink for 'Contributions to Multidisciplinary Team Meetings in Cancer Care: Predictors of Complete Case Information and Comprehensive Case Discussions'

Contributions to Multidisciplinary Team Meetings in Cancer Care: Predictors of Complete Case Information and Comprehensive Case Discussions

Fetched: September 14th, 2021, 5:02am GMT by Jessica Wihl

J Multidiscip Healthc. 2021 Sep 4;14:2445-2452. doi: 10.2147/JMDH.S309162. eCollection 2021.

ABSTRACT

PURPOSE: Multidisciplinary team (MDT) meetings integrate complex information and base recommendations for clinical management on interdisciplinary and multiprofessional decision-making. To support high-quality decision-making and define key performance indicators, we aimed to determine completeness of case information and contributions to MDT case discussions in cancer care.

METHODS: In a prospective observational study design, based on three MDTs, we applied the Metric for Observation of Decision-Making (MODe) tool to assess the quality of case presentation and team members' contributions to case discussions. The MDTs handled patients with brain tumors, soft tissue sarcomas and hepatobiliary cancers. The results were correlated to patient and team characteristics and to MDT leadership skills.

RESULTS: Data were collected from 349 case discussions during 32 MDT meetings. Information on radiology received the highest scores, followed by case history and information on histopathology. Patient-related information was less frequently mentioned and generally received low scores. Contributions to the case discussions were predominantly by the chair, surgeons, and oncologists with limited contributions from nurses. Leadership skills showed a positive correlation with case presentations scores and failure to reach a treatment recommendation correlated with lower case discussion scores.

CONCLUSION: Considerable resources are spent on MDT meetings in cancer care, which motivate initiatives to ensure high-quality and efficient decision-making processes. We identify unbalanced contributions from team members during MDT meetings, demonstrate limited provision of patient-related information and show that leadership skills may positively influence the quality of the case presentations. We suggest that MDTs should consider and develop these aspects to ensure high-quality MDT-based case management and decision-making.

PMID:34511928 | PMC:PMC8426643 | DOI:10.2147/JMDH.S309162

Palliative Surgery for Advanced Cancer: Clinical Profile, Spectrum of Surgery and Outcomes from a Tertiary Care Cancer Centre in Low-Middle-Income Country

Fetched: September 14th, 2021, 5:02am GMT by S V S Deo

Indian J Palliat Care. 2021 Apr-Jun;27(2):281-285. doi: 10.25259/IJPC_399_20. Epub 2021 Aug 12.

ABSTRACT

OBJECTIVES: Palliative surgery for cancer plays an important role in the overall management, especially in low-middle countries with a significant burden of advanced cancers. There is a paucity of literature related to the field of palliative surgery. In this study, we present the clinical spectrum, profile of surgical interventions and outcomes of palliative surgical procedures performed at a tertiary cancer centre involving multiple organ systems.

MATERIALS AND METHODS: A retrospective analysis of prospectively maintained surgical oncology database of a tertiary care cancer centre was performed. Patients fulfilling the criteria of palliative surgery were analysed for clinical spectrum, indications for surgery, palliative surgical procedures and post-operative outcomes.

RESULTS: A total of 678 out of 8300 patients fulfilled the criteria for palliative surgery. Palliative surgical procedures were performed most commonly for gastro-oesophageal malignancies (36.4%) followed by colorectal cancers (24%) and breast cancer (12%). Palliative mastectomy was the most common procedure performed for advanced breast cancer and 7% of sarcoma patients had amputations. Symptom relief could be achieved in 80-90% of patients and post-operative morbidity was relatively high among hepatobiliary, gastrointestinal and gynaecological cancer patients.

CONCLUSION: Globally, a significant number of cancer patients need palliative surgical intervention, especially in LMIC with a high burden of advanced cancers. Results of the current study indicate that gastrointestinal cancer patients constitute a major proportion of patients undergoing palliative surgery. Overall results of the current study indicate that excellent palliation can be achieved in majority of patients with acceptable morbidity and hospital stay.

PMID:34511797 | PMC:PMC8428898 | DOI:10.25259/IJPC_399_20

Tumor Lysis Syndrome due to Eribulin Administration for Metastatic Undifferentiated Pleomorphic Sarcoma of the Buttock

Fetched: September 14th, 2021, 5:02am GMT by Hiroyuki Tsuchie

Acta Med Okayama. 2021 Aug;75(4):533-538. doi: 10.18926/AMO/62408.

ABSTRACT

Tumor lysis syndrome (TLS) is a complication of cancer treatment that requires urgent intervention. It is extremely rare in the treatment of soft tissue sarcoma (STS) of the limbs or trunk, and there are currently no reports of TLS occurrence from eribulin therapy. We report the case of a 78-year-old woman with an undiffer-entiated pleomorphic sarcoma on the right buttock. We initiated chemotherapy with intravenous eribulin mesylate. Deterioration of renal function, mild hyperkalemia, hyperuricemia, hypocalcemia, and hyperphos-phatemia were confirmed on examination, suggesting the presence of TLS. We present an extremely rare case of TLS from eribulin for STS.

PMID:34511623 | DOI:10.18926/AMO/62408

Permalink for 'Well-differentiated systemic mastocytosis with associated myeloid sarcoma and myelodysplastic syndrome: Diagnostic challenges of an underrecognized entity'

Well-differentiated systemic mastocytosis with associated myeloid sarcoma and myelodysplastic syndrome: Diagnostic challenges of an underrecognized entity

Fetched: September 14th, 2021, 5:02am GMT by Siba El Hussein

Leuk Lymphoma. 2021 Sep 11:1-4. doi: 10.1080/10428194.2021.1978089. Online ahead of print.

NO ABSTRACT

PMID:34510998 | DOI:10.1080/10428194.2021.1978089

Permalink for 'The prevalence of homologous recombination deficiency (HRD) in various solid tumors and the role of HRD as a single biomarker to immune checkpoint inhibitors'

The prevalence of homologous recombination deficiency (HRD) in various solid tumors and the role of HRD as a single biomarker to immune checkpoint inhibitors

Fetched: September 14th, 2021, 5:02am GMT by Hana Kim

J Cancer Res Clin Oncol. 2021 Sep 12. doi: 10.1007/s00432-021-03781-6. Online ahead of print.

ABSTRACT

PURPOSE: Homologous recombination deficiency (HRD) is related to tumorigenesis. Currently, the possibility of HRD as a prognostic biomarker to immune checkpoint inhibitors is unknown. We aimed to investigate whether HRD has potential as a biomarker for immunotherapy.

METHODS: The status of homologous recombination deficiency (HRD) was assessed with the next-generation sequencing (NGS) TruSight^™ Oncology 500 assay in 501 patients with advanced solid tumor including gastrointestinal (GI), genitourinary (GU), or rare cancer.

RESULTS: among the 501 patients, HRD was observed as follows: 74.7% (347/501) patients; GU cancer (92.0%, 23 of 25), colorectal cancer (CRC) (86.1%, 130 of 151), hepatocellular carcinoma (HCC) (83.3%, 10 of 12), pancreatic cancer (PC) (76.2%, 32 of 42), biliary tract cancer (BTC) (75.0%, 36 of 48), sarcoma (65.0%, 39 of 60), melanoma (52.4%, 11 of 21), other GI cancers (50.0%, 11 of 22), and rare cancer (50.0%, 2 of 4). Sixty-five of the 501 patients had received immune checkpoint inhibitors (ICIs) during the course of the disease. Tumor types of 65 patients treated with ICIs are as follows: melanoma (95.2%, 20 of 21), HCC (33.3%, 4 of 12), rare cancer (25.0%, 1 of 4), GC (12.2%, 14 of 116), BTC (10.4%, 5 of 48), and sarcoma (5.0%, 3 of 60). The most frequently reported mutations were BRCA2 (n = 90), ARID1A (n = 77), ATM (n = 71), BARD1 (n = 67). Patients without HRD exhibited an objective response rate (ORR) of 33.3% (4 of 12), and patients with HRD exhibited an ORR of 34.0% (18 of 53). There was no significant difference in ORR between patients with and without HRD (P = 0.967). Progression-free survival (PFS) was 6.5 months (95% CI 0.000-16.175) in patients without HRD and 4.1 months (95% CI 2.062-6.138) in patients with HRD, revealing no statistical significance (P = 0.441).

CONCLUSION: Herein, we reported the status of HRD using a cancer-panel for various solid tumor patients in routine clinical practice and demonstrated that HRD as a single biomarker was not sufficient to predict efficacy of ICIs in solid tumor patients.

PMID:34510272 | DOI:10.1007/s00432-021-03781-6

Histone H3K27 demethylase KDM6A is an epigenetic gatekeeper of mTORC1 signalling in cancer

Fetched: September 14th, 2021, 5:02am GMT by Steffie Revia

Gut. 2021 Sep 11:gutjnl-2021-325405. doi: 10.1136/gutjnl-2021-325405. Online ahead of print.

ABSTRACT

OBJECTIVE: Large-scale genome sequencing efforts of human tumours identified epigenetic modifiers as one of the most frequently mutated gene class in human cancer. However, how these mutations drive tumour development and tumour progression are largely unknown. Here, we investigated the function of the histone demethylase KDM6A in gastrointestinal cancers, such as liver cancer and pancreatic cancer.

DESIGN: Genetic alterations as well as expression analyses of KDM6A were performed in patients with liver cancer. Genetic mouse models of liver and pancreatic cancer coupled with Kdm6a-deficiency were investigated, transcriptomic and epigenetic profiling was performed, and in vivo and in vitro drug treatments were conducted.

RESULTS: KDM6A expression was lost in 30% of patients with liver cancer. Kdm6a deletion significantly accelerated tumour development in murine liver and pancreatic cancer models. Kdm6a-deficient tumours showed hyperactivation of mTORC1 signalling, whereas endogenous Kdm6a re-expression by inducible RNA-interference in established Kdm6a-deficient tumours diminished mTORC1 activity resulting in attenuated tumour progression. Genome-wide transcriptional and epigenetic profiling revealed direct binding of Kdm6a to crucial negative regulators of mTORC1, such as Deptor, and subsequent transcriptional activation by epigenetic remodelling. Moreover, in vitro and in vivo genetic epistasis experiments illustrated a crucial function of Deptor and mTORC1 in Kdm6a-dependent tumour suppression. Importantly, KDM6A expression in human tumours correlates with mTORC1 activity and KDM6A-deficient tumours exhibit increased sensitivity to mTORC1 inhibition.

CONCLUSION: KDM6A is an important tumour suppressor in gastrointestinal cancers and acts as an epigenetic toggle for mTORC1 signalling. Patients with KDM6A-deficient tumours could benefit of targeted therapy focusing on mTORC1 inhibition.

PMID:34509979 | DOI:10.1136/gutjnl-2021-325405

Permalink for 'Ultrasound-guided high volume bi-level opposite direction erector spinae plane block for giant undifferentiated sarcoma excision'

Ultrasound-guided high volume bi-level opposite direction erector spinae plane block for giant undifferentiated sarcoma excision

Fetched: September 14th, 2021, 5:02am GMT by Neslihan Kucun

J Clin Anesth. 2021 Sep 9;75:110507. doi: 10.1016/j.jclinane.2021.110507. Online ahead of print.

NO ABSTRACT

PMID:34509961 | DOI:10.1016/j.jclinane.2021.110507

Malignant gastrointestinal neuroectodermal tumor: Cytologic, histologic, immunohistochemical, and molecular pitfalls

Fetched: September 14th, 2021, 5:02am GMT by Priyadharshini Sivasubramaniam

Ann Diagn Pathol. 2021 Sep 6;55:151813. doi: 10.1016/j.anndiagpath.2021.151813. Online ahead of print.

ABSTRACT

Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare malignant primary gastrointestinal mesenchymal tumor which can be diagnosed via fine-needle aspiration (FNA) cytology. In the context of FNA, the diagnosis requires a cell block and the use of significant resources including immunohistochemical stains and molecular testing. The differential diagnosis of GNET includes clear cell sarcoma (CCS), gastrointestinal stromal tumor (GIST), gastric schwannoma, metastatic melanoma, malignant perivascular epithelioid cell tumor (PEComa) and granular cell tumor, among others. Here we describe a case which was initially diagnosed as malignant granular cell tumor by FNA which was later revised to GNET following the finding of an EWSR1-ATF1 fusion gene rearrangement.

PMID:34509898 | DOI:10.1016/j.anndiagpath.2021.151813

Permalink for 'Priorities and preferences of advanced soft tissue sarcoma patients starting palliative chemotherapy: baseline results from the HOLISTIC study'

Priorities and preferences of advanced soft tissue sarcoma patients starting palliative chemotherapy: baseline results from the HOLISTIC study

Fetched: September 14th, 2021, 5:02am GMT by E Younger

ESMO Open. 2021 Sep 9;6(5):100258. doi: 10.1016/j.esmoop.2021.100258. Online ahead of print.

ABSTRACT

INTRODUCTION: Palliative chemotherapy is the principal treatment of patients with advanced soft tissue sarcomas (STS); however prognosis is limited (median overall survival 12-19 months). In this setting, patient values and priorities are central to personalised treatment decisions.

PATIENTS AND METHODS: The prospective HOLISTIC study was conducted in the UK and the Netherlands assessing health-related quality of life in STS patients receiving palliative chemotherapy. Participants completed a questionnaire before starting chemotherapy, including attitudes towards quality of life (QoL) versus length of life (LoL), decisional control preferences, and decisional conflict. Chi-square and Fisher's exact tests were used to evaluate associations between patient characteristics and preferences.

RESULTS: One hundred and thirty-seven patients with advanced STS participated (UK: n = 72, the Netherlands: n = 65). Median age was 62 (27-79) years. Preference for extended LoL (n = 66, 48%) was slightly more common than preference for QoL (n = 56, 41%); 12 patients (9%) valued LoL and QoL equally (missing: n = 3). Younger patients (age <40 years) prioritised LoL, whereas two-thirds of older patients (aged ≥65 years) felt that QoL was equally or more important than LoL (P = 0.020). Decisional conflict was most common in patients who prioritised QoL (P = 0.024). Most patients preferred an active (n = 45, 33%) or collaborative (n = 59, 44%) role in treatment decisions. Gender, performance status, and country were significantly associated with preferred role. Concordance between preferred and actual role in chemotherapy decision was high (n = 104, 76%).

CONCLUSIONS: Heterogeneous priorities and preferences among advanced STS patients support personalised decisions about palliative treatment. Considering individual differences during treatment discussions may enhance communication and optimise patient-centred care.

PMID:34509803 | DOI:10.1016/j.esmoop.2021.100258

Cancer risk in adolescents and young adults living with HIV in South Africa: a nationwide cohort study

Fetched: September 14th, 2021, 5:02am GMT by Yann Ruffieux

Lancet HIV. 2021 Sep 9:S2352-3018(21)00158-2. doi: 10.1016/S2352-3018(21)00158-2. Online ahead of print.

ABSTRACT

BACKGROUND: Literature on cancer in adolescents and young adults (AYA; aged 15-24 years) living with HIV is scarce. We studied cancer incidence in AYA living with HIV in South Africa between 2004 and 2014.

METHODS: In this nationwide cohort study, we included individuals between 15 and 24 years old who had at least two HIV-related laboratory measurements on separate days between Jan 1, 2004, and Dec 31, 2014, recorded in the National Health Laboratory Service database. We used privacy-preserving probabilistic record linkage methods to identify HIV-related laboratory records that most likely belonged to the same individual and to then link these individuals to cancer diagnoses from the National Cancer Registry. We computed incidence rates for the most common cancers in AYA living with HIV, and we assessed associations between these cancers and sex, age, calendar year, and CD4 cell count using Cox proportional hazards models and adjusted hazard ratios (aHRs).

FINDINGS: We included 782 454 AYA living with HIV (698 066 [89·2%] women) with 1 428 114 person-years of follow-up. Of those, 867 developed incident cancer (incidence rate 60·7 per 100 000 person-years), including 429 who developed Kaposi sarcoma (30·0 per 100 000 person-years), 107 non-Hodgkin lymphoma (7·5 per 100 000 person-years), 48 Hodgkin lymphoma (3·4 per 100 000 person-years), 45 cervical cancer (3·4 per 100 000 woman-years), and 32 leukaemia (2·2 per 100 000 person-years). Kaposi sarcoma was more common in the 20-24 year age group than the 15-19 year age group (aHR 1·39, 95% CI 1·03-1·86). Male sex was associated with higher rates of Kaposi sarcoma (2·06, 1·61-2·63), non-Hodgkin lymphoma (3·17, 2·06-4·89), Hodgkin lymphoma (4·83, 2·61-8·93), and leukaemia (unadjusted HR 5·90, 95% CI 2·87-12·12). Cancer rates decreased over the study period, driven by declining Kaposi sarcoma rates. Lower baseline CD4 cell counts were associated with higher rates of Kaposi sarcoma, cervical cancer, non-Hodgkin lymphoma, and Hodgkin lymphoma, but not leukaemia.

INTERPRETATION: Infection-related cancers were the most common cancer types in AYA living with HIV in South Africa, and their incidence rates increased with lower CD4 cell counts. Therefore, innovative strategies to maintaining high CD4 cell counts are needed to reduce the cancer burden in this vulnerable population.

FUNDING: US National Institutes of Health and Swiss National Science Foundation.

PMID:34509198 | DOI:10.1016/S2352-3018(21)00158-2

Long non-coding RNA ZMIZ1-AS1 promotes osteosarcoma progression by stabilization of ZMIZ1

Fetched: September 12th, 2021, 5:01am GMT by Yichi Zhou

Cell Biol Toxicol. 2021 Sep 10. doi: 10.1007/s10565-021-09641-w. Online ahead of print.

ABSTRACT

BACKGROUND: Osteosarcomas (OS) are frequent primary sarcomas of the bone in children and adolescents. The long non-coding RNAs (lncRNAs) can affect the progression of many cancers by their sense transcripts. The present study was designed to probe the role of ZMIZ1-AS1 and the downstream pathway in OS progression.

METHODS: Cell proliferation, invasion, and migration were detected by colony formation, transwell, and wound healing assays. The binding of SOX2 or MYC protein with ZMIZ1-AS1 promoter was explored by ChIP assay and dual-luciferase reporter assay. Interaction between PTBP1 protein and ZMIZ1-AS1 (or ZMIZ1 mRNA) was detected by RIP assay.

RESULTS: SOX2 and MYC are the downstream effectors of the Hippo pathway and transcriptionally activated ZMIZ1-AS1. Compared to the controls, OS tissues and cells contained higher ZMIZ1-AS1 expression. Silencing of ZMIZ1-AS1 repressed OS cell viability, proliferation, migration, and invasion. Our findings further showed that ZMIZ1-AS1 recruits RNA-binding protein PTBP1 to stabilize ZMIZ1 mRNA. PTBP1 or ZMIZ1 overexpression rescues the suppressive effects of silenced ZMIZ1-AS1 on OS cellular processes. Importantly, ZMIZ1-AS1 promotes OS growth in vivo by stabilization of ZMIZ1.

CONCLUSIONS: Long non-coding RNA ZMIZ1-AS1 promotes OS progression by stabilization of ZMIZ1. The Hippo pathway is inactivated in osteosarcoma. Transcriptional factors SOX2 and MYC downstream the Hippo pathway induce the upregulation of ZMIZ1-AS1 in osteosarcoma. ZMIZ1-AS1 recruits RNA binding protein PTBP1 that stabilizes ZMIZ1, the sense transcript of ZMIZ1-AS1. ZMIZ1-AS1 promotes osteosarcoma cell viability, proliferation, migration, and invasion by ZMIZ1 in a PTBP1 dependent manner.

PMID:34508303 | DOI:10.1007/s10565-021-09641-w

Permalink for 'MRI-based Delta-Radiomics predicts pathologic complete response in high-grade soft-tissue sarcoma patients treated with neoadjuvant therapy'

MRI-based Delta-Radiomics predicts pathologic complete response in high-grade soft-tissue sarcoma patients treated with neoadjuvant therapy

Fetched: September 12th, 2021, 5:01am GMT by Jan C Peeken

Radiother Oncol. 2021 Sep 7:S0167-8140(21)06717-7. doi: 10.1016/j.radonc.2021.08.023. Online ahead of print.

ABSTRACT

PURPOSE: In high-grade soft-tissue sarcomas (STS) the standard of care encompasses multimodal therapy regimens. While there is a growing body of evidence for prognostic pretreatment radiomic models, we hypothesized that temporal changes in radiomic features following neoadjuvant treatment ("delta-radiomics") may be able to predict the pathological complete response (pCR).

METHODS: MRI scans (T1-weighted with fat-saturation and contrast-enhancement (T1FSGd) and T2-weighted with fat-saturation (T2FS)) of patients with STS of the extremities and trunk treated with neoadjuvant therapy were gathered from two independent institutions (training: 103, external testing: 53 patients). pCR was defined as < 5% viable cells. After segmentation and preprocessing, 105 radiomic features were extracted. Delta-radiomic features were calculated by subtraction of features derived from MRI scans obtained before and after neoadjuvant therapy. After feature reduction, machine learning modeling was performed in 100 iterations of 3-fold nested cross-validation. Delta-radiomic models were compared with single timepoint models in the testing cohort.

RESULTS: The combined delta-radiomic models achieved the best area under the receiver operating characteristic curve (AUC) of 0.75. Pre-therapeutic tumor volume was the best conventional predictor (AUC 0.70). The T2FS-based delta-radiomic model had the most balanced classification performance with a balanced accuracy of 0.69. Delta-radiomic models achieved better reproducibility than single timepoint radiomic models, RECIST or the peri-therapeutic volume change. Delta-radiomic models were significantly associated with survival in multivariate Cox regression.

CONCLUSION: This exploratory analysis demonstrated that MRI-based delta-radiomics improves prediction of pCR over tumor volume and RECIST. Delta-radiomics may one day function as a biomarker for personalized treatment adaptations.

PMID:34506832 | DOI:10.1016/j.radonc.2021.08.023

Adenosarcoma of Uterus- Rare Biphasic Malignant Tumor: A Case Report

Fetched: September 12th, 2021, 5:01am GMT by Dilasma Ghartimagar

JNMA J Nepal Med Assoc. 2021 Feb 28;59(234):200-203. doi: 10.31729/jnma.5373.

ABSTRACT

Uterine adenosarcoma is a rare variant of mixed Mullerian tumors comprised of neoplastic glands with the benign appearance and sarcomatous stroma. The epithelium most often consists of endometrium- like cells, while the sarcomatous component usually shows low-grade homologous uterine sarcoma. These tumors present as a pelvic mass or an enlarged uterus with abnormal vaginal bleeding. Here, we present a case of 61 years old postmenopausal female patient with chief complaints of excessive vaginal bleeding and urine retention.

PMID:34506473 | DOI:10.31729/jnma.5373

Kreftbehandling med isolert ekstremitetsperfusjon

Fetched: September 12th, 2021, 5:01am GMT by Milan Spasojevic

Tidsskr Nor Laegeforen. 2021 Aug 19;141(2021-12). doi: 10.4045/tidsskr.21.0194. Print 2021 Sep 7.

ABSTRACT

For locally advanced soft tissue sarcomas and metastases from melanoma located in the extremities, mutilating surgery or amputation may be necessary to achieve local control. Isolated limb perfusion with high-dose chemotherapy may represent an alternative to amputation for this patient group.

PMID:34505491 | DOI:10.4045/tidsskr.21.0194

Permalink for 'Pedicled anterolateral thigh flap transfer for the reconstruction of a large gluteal defect assisted by preoperative computed tomographic angiography and intraoperative indocyanine green angiography: A case report'

Pedicled anterolateral thigh flap transfer for the reconstruction of a large gluteal defect assisted by preoperative computed tomographic angiography and intraoperative indocyanine green angiography: A case report

Fetched: September 12th, 2021, 5:01am GMT by Yuma Fuse

Microsurgery. 2021 Sep 10. doi: 10.1002/micr.30809. Online ahead of print.

ABSTRACT

The efficacy of the use of the pedicled ALT flap for buttock defects has not been established in the literature. Here, we present a case of a successful reconstruction of a large gluteal defect after sarcoma resection using a pedicled ALT flap assisted by preoperative computed tomographic angiography (CTA) and intraoperative indocyanine green (ICG) angiography. A 94-year-old man underwent resection of a large soft tissue tumor in the gluteal region resulting in a 19 × 15 cm defect. Based on three-dimensional reconstructed images of the preoperative CTA, the length of the pedicle of an ALT flap was estimated. Perfusion of the whole flap was confirmed using intraoperative ICG angiography. The flap was inserted through a subcutaneous tunnel and covered the defect completely. The postoperative course was uneventful. The patient resumed ambulation 1 week after the operation. The follow-up at 2 months showed complete flap survival, and the patient was able to walk with a cane. The pedicled ALT flap transfer may be a reliable alternative for gluteal reconstruction when guided by pre- and intraoperative imaging modalities.

PMID:34505303 | DOI:10.1002/micr.30809

Complement activation promoted by the lectin pathway mediates C3aR-dependent sarcoma progression and immunosuppression

Fetched: September 12th, 2021, 5:01am GMT by Elena Magrini

Nat Cancer. 2021 Feb;2(2):218-232. doi: 10.1038/s43018-021-00173-0. Epub 2021 Feb 18.

ABSTRACT

Complement has emerged as a component of tumor promoting inflammation. We conducted a systematic assessment of the role of complement activation and effector pathways in sarcomas. C3 ^-/-, MBL1/2 ^-/- and C4 ^-/- mice showed reduced susceptibility to 3-methylcholanthrene sarcomagenesis and transplanted sarcomas, whereas C1q and factor B deficiency had marginal effects. Complement 3a receptor (C3aR), but not C5aR1 and C5aR2, deficiency mirrored the phenotype of C3 ^-/- mice. C3 and C3aR deficiency were associated with reduced accumulation and functional skewing of tumor-associated macrophages, increased T cell activation and response to anti-PD-1 therapy. Transcriptional profiling of sarcoma infiltrating macrophages and monocytes revealed the enrichment of MHC II-dependent antigen presentation pathway in C3-deficient cells. In patients, C3aR expression correlated with a macrophage population signature and C3 deficiency-associated signatures predicted better clinical outcome. These results suggest that the lectin pathway and C3a/C3aR axis are key components of complement and macrophage-mediated sarcoma promotion and immunosuppression.

PMID:34505065 | PMC:PMC8425276 | DOI:10.1038/s43018-021-00173-0

Rhabdomyosarcoma in Adults: A Retrospective Analysis of Case Records Diagnosed between 1979 and 2018 in Western Denmark

Fetched: September 12th, 2021, 5:01am GMT by Vivi-Nelli Mäkinen

Sarcoma. 2021 Aug 30;2021:9948885. doi: 10.1155/2021/9948885. eCollection 2021.

ABSTRACT

INTRODUCTION: Adult rhabdomyosarcoma is a rare tumour that has an inferior survival compared to the paediatric patient population. The reason for this consistently worse outcome remains mostly unknown. It has been suggested that this disparity may be related to biological and/or treatment-related factors, which in the literature has been shown to be distributed differently among paediatric and adult patients. The aim of this study was to clarify treatment outcome and clinicopathological factors for adult patients with rhabdomyosarcoma that were treated in Aarhus, Denmark, since 1979.

METHODS: By searching the Aarhus Sarcoma Registers, data for all rhabdomyosarcoma patients, aged 18 years or more, between 1979 and 2018, were retrieved and analysed.

RESULTS: Data from 50 patients were collected. No patients were lost to follow-up. For the entire cohort, 5- and 10-year overall survival rates were 30% and 18%, respectively. The median age was 46.5 years, and the median overall survival was 2.3 years. Tumour histology was embryonal 18%, alveolar 22%, pleomorphic 44%, and not otherwise specified 16%. The tumour site was unfavourable in more than 80% of the patients. Significant factors associated with inferior overall survival were histology and disease stage, although histological subtype was not significant in the multivariate model. Five-year overall survival was 40% for localised disease versus 15% for metastatic disease.

CONCLUSION: Rhabdomyosarcoma in adults has a poorer prognosis than paediatric rhabdomyosarcoma and other high-grade sarcomas in adults. Adult rhabdomyosarcoma should continue to be treated aggressively, but new and tailored treatment strategies are needed to improve the long-term outcome. Previous predictors of poor survival in paediatric patients were valid in adults except for age, site (favourable versus unfavourable), and tumour size.

PMID:34504392 | PMC:PMC8423536 | DOI:10.1155/2021/9948885

Identification of novel SSX1 fusions in synovial sarcoma

Fetched: September 12th, 2021, 5:01am GMT by Akihiko Yoshida

Mod Pathol. 2021 Sep 9. doi: 10.1038/s41379-021-00910-x. Online ahead of print.

ABSTRACT

Synovial sarcoma is characterized by variable epithelial differentiation and specific SS18-SSX gene fusions. The diagnosis is primarily based on phenotype, but fusion gene detection is increasingly being considered indispensable, with SS18 break-apart fluorescence in situ hybridization (FISH) being favored in many laboratories. However, SS18 FISH assay produces negative or atypical results in a minority of cases, leaving uncertainties in diagnosis and management. Here, we analyzed this challenging subset of SS18 FISH-negative/atypical synovial sarcoma using RNA sequencing and monoclonal antibodies that recognize SS18-SSX and the SSX C-terminus. Among 99 synovial sarcoma cases that were previously subjected to SS18 break-apart FISH, eight cases were reported as negative and three cases were indeterminate, owing to atypical signal patterns. Three of these 11 tumors (two monophasic and one biphasic) harbored novel EWSR1-SSX1 fusions, were negative for SS18-SSX staining, and were positive for SSX C-terminus staining. One monophasic tumor harbored a novel MN1-SSX1 fusion, and showed negative SS18-SSX expression and positive SSX C-terminus staining. Another monophasic tumor carried an SS18L1-SSX1 fusion, and was weakly positive for SS18-SSX, while SMARCB1 expression was reduced. The presence of these novel and/or rare fusions was confirmed using RT-PCR and Sanger sequencing. EWSR1-SSX1 was further validated by EWSR1 FISH assay. The remaining six tumors (five monophasic and one biphasic) showed strong SS18-SSX expression, and RNA sequencing successfully performed in three cases identified canonical SS18-SSX2 fusions. Based on a DNA methylation-based unsupervised clustering, the tumors with EWSR1-SSX1 and SS18L1-SSX1 clustered with synovial sarcoma, while the MN1-SSX1-positive tumor was not co-clustered despite classic histology and immunoprofile. In summary, we discovered novel and rare SSX1 fusions to non-SS18 genes in synovial sarcoma. The expanded genetic landscape carries significant diagnostic implications and advances our understanding of the oncogenic mechanism.

PMID:34504309 | DOI:10.1038/s41379-021-00910-x

Predictors of attrition among adults in a rural HIV clinic in southern Mozambique: 18-year retrospective study

Fetched: September 12th, 2021, 5:01am GMT by Edy Nacarapa

Sci Rep. 2021 Sep 9;11(1):17897. doi: 10.1038/s41598-021-97466-2.

ABSTRACT

HIV remains a major cause of morbidity and mortality for people living in many low-income countries. With an HIV prevalence of 12.4% among people aged over 15 years, Mozambique was ranked in 2019 as one of eight countries with the highest HIV rates in the world. We analyzed routinely collected data from electronical medical records in HIV-infected patients aged 15 years or older and enrolled at Carmelo Hospital of Chokwe in Chokwe from 2002 to 2019. Attrition was defined as individuals who were either reported dead or lost to follow-up (LTFU) (≥ 90 days since the last clinic visit with missed medical pick-up after 3 days of failed calls). Kaplan-Meier survival curves and Cox regression analyses were used to model the incidence and predictors of time to attrition. From January 2002 to December 2019, 16,321 patients were enrolled on antiretroviral therapy (ART): 59.2% were women, and 37.9% were aged 25-34 years old. At the time of the analysis, 7279 (44.6%) were active and on ART. Overall, the 16,321 adults on ART contributed a total of 72,987 person-years of observation. The overall attrition rate was 9.46 per 100 person-years. Cox regression showed a higher risk of attrition in those following an inpatient regimen (hazard ratio [HR] 3.18, 95% confidence interval [CI] 2.89-3.50; p < 0.001), having CD4 counts under 50 cells/µL (HR 1.91, 95% CI 1.63-2.24, p < 0.001), receiving anti-TB treatment within 90 days of ART initiation (HR 6.53, 95% CI 5.72-7.45; p < 0.001), classified as WHO clinical stage III (HR 3.75, 95% CI 3.21-4.37; p < 0.001), and having Kaposi's sarcoma (HR 1.99, 95% CI 1.65-2.39, p < 0.001). Kaplan-Meier analysis showed that patients with CD4 counts of less than 50 cells/µL on ART initiation had a 40% lower chance of survival at 18 years. Low CD4 cell counts, ART initiation as an inpatient, WHO clinical stage III, and anti-tuberculosis treatment within 90 days of ART initiation were strongly associated with attrition. Strengthening HIV testing and ART treatment, improving the diagnosis of tuberculosis before ART initiation, and guaranteed psychosocial support systems are the best tools to reduce patient attrition after starting ART.

PMID:34504234 | PMC:PMC8429703 | DOI:10.1038/s41598-021-97466-2

Genomic landscape of gliosarcoma: distinguishing features and targetable alterations

Fetched: September 12th, 2021, 5:01am GMT by Mark M Zaki

Sci Rep. 2021 Sep 9;11(1):18009. doi: 10.1038/s41598-021-97454-6.

ABSTRACT

Gliosarcoma is an aggressive brain tumor with histologic features of glioblastoma (GBM) and soft tissue sarcoma. Despite its poor prognosis, its rarity has precluded analysis of its underlying biology. We used a multi-center database to characterize the genomic landscape of gliosarcoma. Sequencing data was obtained from 35 gliosarcoma patients from Genomics Evidence Neoplasia Information Exchange (GENIE) 5.0, a database curated by the American Association of Cancer Research (AACR). We analyzed genomic alterations in gliosarcomas and compared them to GBM (n = 1,449) and soft tissue sarcoma (n = 1,042). 30 samples were included (37% female, median age 59 [IQR: 49-64]). Nineteen common genes were identified in gliosarcoma, defined as those altered in > 5% of samples, including TERT Promoter (92%), PTEN (66%), and TP53 (60%). Of the 19 common genes in gliosarcoma, 6 were also common in both GBM and soft tissue sarcoma, 4 in GBM alone, 0 in soft tissue sarcoma alone, and 9 were more distinct to gliosarcoma. Of these, BRAF harbored an OncoKB level 1 designation, indicating its status as a predictive biomarker of response to an FDA-approved drug in certain cancers. EGFR, CDKN2A, NF1, and PTEN harbored level 4 designations in solid tumors, indicating biological evidence of these biomarkers predicting a drug-response. Gliosarcoma contains molecular features that overlap GBM and soft tissue sarcoma, as well as its own distinct genomic signatures. This may play a role in disease classification and inclusion criteria for clinical trials. Gliosarcoma mutations with potential therapeutic indications include BRAF, EGFR, CDKN2A, NF1, and PTEN.

PMID:34504233 | PMC:PMC8429571 | DOI:10.1038/s41598-021-97454-6

Permalink for 'Clinical activity of ripretinib in patients with advanced gastrointestinal stromal tumor harboring heterogenous KIT/PDGFRA mutations in the phase 3 INVICTUS study'

Clinical activity of ripretinib in patients with advanced gastrointestinal stromal tumor harboring heterogenous KIT/PDGFRA mutations in the phase 3 INVICTUS study

Fetched: September 12th, 2021, 5:01am GMT by Sebastian Bauer

Clin Cancer Res. 2021 Sep 9:clincanres.1864.2021. doi: 10.1158/1078-0432.CCR-21-1864. Online ahead of print.

ABSTRACT

PURPOSE: Most patients with gastrointestinal stromal tumor (GIST) have activating mutations in KIT/PDGFRA and are initially responsive to tyrosine kinase inhibitors (TKIs). The acquisition of secondary mutations leads to refractory/relapsed disease. This study reports the results of an analysis from the phase 3 INVICTUS study (NCT03353753) characterizing the genomic heterogeneity of tumors from patients with advanced GIST and evaluating ripretinib efficacy across KIT/PDGFRA mutation subgroups.

PATIENTS AND METHODS: Tumor tissue and liquid biopsy samples that captured circulating tumor DNA were collected prior to study enrollment and sequenced using next-generation sequencing. Subgroups were determined by KIT/PDGFRA mutations and correlation of clinical outcomes and KIT/PDGFRA mutational status was assessed.

RESULTS: Overall, 129 patients enrolled (ripretinib 150 mg once daily, n=85; placebo, n=44). The most common primary mutation subgroup detected by combined tissue and liquid biopsies were in KIT exon 11 (ripretinib, 61.2%; placebo, 77.3%) and KIT exon 9 (ripretinib, 18.8%; placebo, 15.9%). Patients receiving ripretinib demonstrated progression-free survival (PFS) benefit vs placebo regardless of mutation status (hazard ratio 0.16) and in all assessed subgroups in Kaplan-Meier PFS analysis (exon 11, P <0.0001; exon 9, P=0.0023; exon 13, P <0.0001; exon 17, P <0.0001). Among patients with wild-type KIT/PDGFRA by tumor tissue, PFS ranged from 2-23 months for ripretinib vs 0.9-10.1 months for placebo Conclusions: Ripretinib provided clinically meaningful activity across mutation subgroups in patients with advanced GIST, demonstrating ripretinib inhibits a broad range of KIT/PDGFRA mutations in patients with advanced GIST who were previously treated with 3 or more TKIs.

PMID:34503977 | DOI:10.1158/1078-0432.CCR-21-1864

Pathological and Immunohistochemical Findings of Disseminated Histiocytic Sarcoma in a Sugar Glider (Petaurus breviceps)

Fetched: September 12th, 2021, 5:01am GMT by Nguyen V Son

J Comp Pathol. 2021 Aug;187:83-88. doi: 10.1016/j.jcpa.2021.07.005. Epub 2021 Aug 13.

ABSTRACT

A 6-year-old intact female sugar glider (Petaurus breviceps) had a swelling at the pouch region and died 8 days after presentation. At necropsy, the urinary bladder and abdominal lymph nodes were enlarged and a mass was located in the pericloacal region. Tumour infiltration was also observed in the medial iliac lymph nodes, liver, spleen, small intestine, uterus and left ovary. Histopathologically, the tumours were composed of pleomorphic histiocytes that had round or bizarre nuclei and abundant pale eosinophilic cytoplasm. Immunohistochemically, tumour cells were positive for ionized calcium-binding adaptor molecule 1 and human leukocyte antigen and negative for CD3, B lymphocyte antigen 36 and cytokeratin. Histopathological and immunohistochemical examinations confirmed a diagnosis of disseminated histiocytic sarcoma. This neoplasm has not been previously reported in a sugar glider.

PMID:34503659 | DOI:10.1016/j.jcpa.2021.07.005

TP53 in Biology and Treatment of Osteosarcoma

Fetched: September 12th, 2021, 5:01am GMT by Kamil Jozef Synoradzki

Cancers (Basel). 2021 Aug 25;13(17):4284. doi: 10.3390/cancers13174284.

ABSTRACT

The TP53 gene is mutated in 50% of human tumors. Oncogenic functions of mutant TP53 maintain tumor cell proliferation and tumor growth also in osteosarcomas. We collected data on TP53 mutations in patients to indicate which are more common and describe their role in in vitro and animal models. We also describe animal models with TP53 dysfunction, which provide a good platform for testing the potential therapeutic approaches. Finally, we have indicated a whole range of pharmacological compounds that modulate the action of p53, stabilize its mutated versions or lead to its degradation, cause silencing or, on the contrary, induce the expression of its functional version in genetic therapy. Although many of the described therapies are at the preclinical testing stage, they offer hope for a change in the approach to osteosarcoma treatment based on TP53 targeting in the future.

PMID:34503094 | PMC:PMC8428337 | DOI:10.3390/cancers13174284

The Role of Bacteria in KSHV Infection and KSHV-Induced Cancers

Fetched: September 12th, 2021, 5:01am GMT by Ashley Markazi

Cancers (Basel). 2021 Aug 25;13(17):4269. doi: 10.3390/cancers13174269.

ABSTRACT

The objective of this article is to review the current status of the bacteria-virus interplay in Kaposi's sarcoma-associated herpesvirus (KSHV) infection and KSHV-driven cancers. KSHV is the etiological agent of several cancers, including Kaposi's sarcoma (KS) and primary effusion lymphoma. Due to immunosuppression, patients with KSHV are at an increased risk for bacterial infections. Moreover, among patients coinfected by HIV and KSHV, patients with KS have distinct oral microbiota compared to non-KS patients. Bacterial biomarkers associated with KSHV-driven cancers can provide insights in discerning the mechanisms of KSHV-induced oncogenesis. For example, pathogen-associated molecular patterns and bacterial products of certain bacterial species can regulate the expression of KSHV lytic and latent genes, thereby affecting viral replication and dissemination. In addition, infection with distinct opportunistic bacterial species have been associated with increased cell proliferation and tumorigenesis in KSHV-induced cancers through activation of pro-survival and -mitogenic cell signaling pathways. By elucidating the various mechanisms in which bacteria affect KSHV-associated pathogenesis, we will be able to pinpoint therapeutic targets for KSHV infection and KSHV-related cancers.

PMID:34503079 | PMC:PMC8428360 | DOI:10.3390/cancers13174269

Machine Learning-Based Radiomics Signatures for EGFR and KRAS Mutations Prediction in Non-Small-Cell Lung Cancer

Fetched: September 12th, 2021, 5:01am GMT by Nguyen Quoc Khanh Le

Int J Mol Sci. 2021 Aug 26;22(17):9254. doi: 10.3390/ijms22179254.

ABSTRACT

Early identification of epidermal growth factor receptor (EGFR) and Kirsten rat sarcoma viral oncogene homolog (KRAS) mutations is crucial for selecting a therapeutic strategy for patients with non-small-cell lung cancer (NSCLC). We proposed a machine learning-based model for feature selection and prediction of EGFR and KRAS mutations in patients with NSCLC by including the least number of the most semantic radiomics features. We included a cohort of 161 patients from 211 patients with NSCLC from The Cancer Imaging Archive (TCIA) and analyzed 161 low-dose computed tomography (LDCT) images for detecting EGFR and KRAS mutations. A total of 851 radiomics features, which were classified into 9 categories, were obtained through manual segmentation and radiomics feature extraction from LDCT. We evaluated our models using a validation set consisting of 18 patients derived from the same TCIA dataset. The results showed that the genetic algorithm plus XGBoost classifier exhibited the most favorable performance, with an accuracy of 0.836 and 0.86 for detecting EGFR and KRAS mutations, respectively. We demonstrated that a noninvasive machine learning-based model including the least number of the most semantic radiomics signatures could robustly predict EGFR and KRAS mutations in patients with NSCLC.

PMID:34502160 | PMC:PMC8431041 | DOI:10.3390/ijms22179254

Dedifferentiated liposarcoma originating in the uterus: report of a case

Fetched: September 12th, 2021, 5:01am GMT by Q Lei

Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):1059-1061. doi: 10.3760/cma.j.cn112151-20210124-00074.

NO ABSTRACT

PMID:34496502 | DOI:10.3760/cma.j.cn112151-20210124-00074

Photodiagnosis and photodynamic effects of bacteriochlorin-naphthalimide conjugates on tumor cells and mouse model

Fetched: September 10th, 2021, 5:02am GMT by Natalia B Morozova

J Photochem Photobiol B. 2021 Aug 20;223:112294. doi: 10.1016/j.jphotobiol.2021.112294. Online ahead of print.

ABSTRACT

Photo-induced cytotoxicity and antitumor activity of a series of dual function agents for photodynamic therapy (PDT) and fluorescent imaging based on bacteriochlorin photosensitizer conjugated with various naphthalimide fluorophores was studied in vitro using murine tumor cells of S37 sarcoma and in vivo on mice bearing murine S37 sarcoma. Upon irradiation at the absorption maximum of the photosensitizer, the activity of conjugates was as high as in the case of individual bacteriochlorin, while an additional excitation of the naphthalimide fragment led to an increase in the PDT efficacy due to resonance energy transfer from the fluorophore to photosensitizer. The fluorescence contrast and specific cytotoxic activity measurements indicate that the conjugate of bacteriochlorin with 3,4-dimethoxestyrene-substituted naphthalimide is the most promising agent for the application as theranostic in PDT.

PMID:34500215 | DOI:10.1016/j.jphotobiol.2021.112294

Permalink for 'Precision guidelines for soft tissue and visceral sarcomas: the evidence, expert experience and ensuring optimal care for rare cancers, a 2021 update from ESMO-EURACAN-GENTURIS'

Precision guidelines for soft tissue and visceral sarcomas: the evidence, expert experience and ensuring optimal care for rare cancers, a 2021 update from ESMO-EURACAN-GENTURIS

Fetched: September 10th, 2021, 5:02am GMT by R A Gladdy

Ann Oncol. 2021 Sep 6:S0923-7534(21)04459-8. doi: 10.1016/j.annonc.2021.08.2155. Online ahead of print.

NO ABSTRACT

PMID:34500045 | DOI:10.1016/j.annonc.2021.08.2155

Bone sarcomas: ESMO-EURACAN-GENTURIS-ERNPaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up

Fetched: September 10th, 2021, 5:02am GMT by S J Strauss

Ann Oncol. 2021 Sep 1:S0923-7534(21)04280-0. doi: 10.1016/j.annonc.2021.08.1995. Online ahead of print.

NO ABSTRACT

PMID:34500044 | DOI:10.1016/j.annonc.2021.08.1995

Targeting KRAS in pancreatic cancer: new drugs on the horizon

Fetched: September 10th, 2021, 5:02am GMT by Sahar F Bannoura

Cancer Metastasis Rev. 2021 Sep 9. doi: 10.1007/s10555-021-09990-2. Online ahead of print.

ABSTRACT

Kirsten Rat Sarcoma (KRAS) is a master oncogene involved in cellular proliferation and survival and is the most commonly mutated oncogene in all cancers. Activating KRAS mutations are present in over 90% of pancreatic ductal adenocarcinoma (PDAC) cases and are implicated in tumor initiation and progression. Although KRAS is a critical oncogene, and therefore an important therapeutic target, its therapeutic inhibition has been very challenging, and only recently specific mutant KRAS inhibitors have been discovered. In this review, we discuss the activation of KRAS signaling and the role of mutant KRAS in PDAC development. KRAS has long been considered undruggable, and many drug discovery efforts which focused on indirect targeting have been unsuccessful. We discuss the various efforts for therapeutic targeting of KRAS. Further, we explore the reasons behind these obstacles, novel successful approaches to target mutant KRAS including G12C mutation as well as the mechanisms of resistance.

PMID:34499267 | DOI:10.1007/s10555-021-09990-2

Primary Aortic Angiosarcoma: A Review of Two Cases Highlighting Unique Imaging and Clinical Characteristics

Fetched: September 10th, 2021, 5:02am GMT by Case H Keltner

Radiol Cardiothorac Imaging. 2021 Jul 29;3(4):e210040. doi: 10.1148/ryct.2021210040. eCollection 2021 Aug.

ABSTRACT

Angiosarcomas are rare vascular connective tissue tumors originating from the tunica intima, media, or adventitia and may arise in large vessels. Here two cases of primary angiosarcoma of the aorta are described that highlight initial manifestations and unique complications from diffuse arterial tumor emboli, which led to delayed cancer diagnosis. In patients with aortic lesions, imaging characteristics must be thoughtfully reconciled with clinical scenarios, particularly in patients with no additional evidence of atherosclerosis or vasculitis. Keywords: Oncology, Neoplasms-Primary, MR-Angiography, Aorta © RSNA, 2021.

PMID:34498006 | PMC:PMC8415140 | DOI:10.1148/ryct.2021210040

TRIM8 in Ewing sarcoma

Fetched: September 10th, 2021, 5:02am GMT by Zhe Wang

Nat Cell Biol. 2021 Sep 8. doi: 10.1038/s41556-021-00755-1. Online ahead of print.

NO ABSTRACT

PMID:34497371 | DOI:10.1038/s41556-021-00755-1

Permalink for 'Diagnostic accuracy of ultrasound-guided core needle biopsy versus incisional biopsy in soft tissue sarcoma: an institutional experience'

Diagnostic accuracy of ultrasound-guided core needle biopsy versus incisional biopsy in soft tissue sarcoma: an institutional experience

Fetched: September 10th, 2021, 5:02am GMT by Miroslava Cernakova

Sci Rep. 2021 Sep 8;11(1):17832. doi: 10.1038/s41598-021-96953-w.

ABSTRACT

Core needle biopsy (CNB) is gaining in importance due to its advantages in the matter of patient morbidity, time and cost. Nevertheless, controversies still exist regarding the biopsy technique of choice for the accurate diagnosis of soft tissue sarcoma (STS). This retrospective cohort study compared the diagnostic performance between ultrasound-guided CNB and incisional biopsy (IB), both performed by orthopedic surgeons. The aims of the study were to answer the following questions: (1) Is ultrasound-guided CNB a highly reliable modality for diagnosing STSs? (2) Is CNB equally useful to IB for identifying histologic subtype? (3) Had patients who underwent CNB a reduced risk of complications? One-hundred and fifty-three patients who underwent resection of soft tissue sarcoma were classified into two groups according to biopsy technique prior to surgery; CNB group (n = 95) and IB group (n = 58). The final surgical specimens were in 40 patients liposarcoma (myxoid, pleomorphic and dedifferentiated), 39 undifferentiated pleomorphic sarcoma (UPS), 33 myxofibrosarcoma, 10 synovial sarcoma, 10 leiomyosarcoma and in the remaining 21 patients different soft tissue sarcoma entities. Sarcoma location of 71 patients was in the thigh, 19 in the lower leg, 22 in the upper arm and shoulder area; 10 in the knee and gluteal region, 9 in the thoracic region, the residual 12 in other body areas. Malignancy was correctly diagnosed in 87% (83 of 95) for the CNB group and 93% (54/58) for the IB group. Correct identification rate of histologic subtype was 80% (76 of 95) in the CNB group and 83% (48 of 58) in the IB group. There were no significant differences in the correct diagnosis rates of malignancy and subtype between the two techniques. No complications were seen in the CNB group, whereas 2 patients in whom IB was performed developed pulmonary embolism and 1 patient surgical site infection. Ultrasound-guided CNB is highly accurate and not inferior to IB in diagnosing the dignity of lesions and histologic subtype in patients with suspected STSs.

PMID:34497298 | PMC:PMC8426501 | DOI:10.1038/s41598-021-96953-w

Permalink for 'The Expression of Insulin-Like Growth Factor 2 Messenger RNA-Binding Protein 3 in Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma'

The Expression of Insulin-Like Growth Factor 2 Messenger RNA-Binding Protein 3 in Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma

Fetched: September 10th, 2021, 5:02am GMT by Konomi Yashige

Tohoku J Exp Med. 2021 Sep;255(1):27-31. doi: 10.1620/tjem.255.27.

ABSTRACT

Langerhans cell neoplasms, which include Langerhans cell histiocytosis and Langerhans cell sarcoma, are tumors that originate from dendritic cells. Langerhans cell sarcoma is defined as a high-grade neoplasm with overtly malignant cytological features and the Langerhans cell-like phenotype, and generally has a poorer prognosis and more aggressive phenotype than Langerhans cell histiocytosis. Insulin-like growth factor 2 messenger RNA-binding protein 3 (IGF2BP3 or IMP3) is an oncofetal protein that is expressed in various cancer types; its expression is often associated with a poor prognosis and aggressive phenotype. Here, we used immunohistochemistry to evaluate IGF2BP3 expression in Langerhans cell neoplasms. IGF2BP3 expression was scored as negative (< 1%) or positive (≥ 1%) by immunohistochemistry. All 4 patients with Langerhans cell sarcoma (100%) and 6 of 22 pediatric (age < 18 years) patients with Langerhans cell histiocytosis (27.3%) had positive results for IGF2BP3; however, 16 of 22 pediatric patients with Langerhans cell histiocytosis (72.7%) and all 15 adult (age ≥ 18 years) patients with Langerhans cell histiocytosis (100%) had a negative result. Among patients with Langerhans cell histiocytosis, IGF2BP3 expression was independent of sex, location, prognosis, and BRAF V600E staining results. Taken together, these results indicate that IGF2BP3 expression may be a helpful marker for distinguishing Langerhans cell sarcoma from Langerhans cell histiocytosis in adult patients.

PMID:34497165 | DOI:10.1620/tjem.255.27

How to avoid peritoneal tumor spillage during total abdominal hysterectomy in uterine cancers with cervical invasion

Fetched: September 10th, 2021, 5:02am GMT by Hélène Leray

Int J Gynecol Cancer. 2021 Sep 8:ijgc-2021-002649. doi: 10.1136/ijgc-2021-002649. Online ahead of print.

NO ABSTRACT

PMID:34497134 | DOI:10.1136/ijgc-2021-002649

Novel potent anti-STEAP1 bispecific antibody to redirect T cells for cancer immunotherapy

Fetched: September 10th, 2021, 5:02am GMT by Tsung-Yi Lin

J Immunother Cancer. 2021 Sep;9(9):e003114. doi: 10.1136/jitc-2021-003114.

ABSTRACT

BACKGROUND: The prognosis for metastatic Ewing sarcoma family of tumors (EFT) is still poor despite high-dose chemotherapy and radiation treatment. Immunotherapies hold promise, but cancer antigen-targeting immunotherapies have largely failed to induce effective T cell receptor-mediated antitumor response. However, T cell-engaging bispecific antibodies (T-BsAbs) have yet to be adequately explored.

METHODS: Rehumanized STEAP1-IgG was used to build T-BsAb (named BC261) using the 2+2 IgG-[L]-scFv platform carrying the anti-CD3 huOKT3 scFv as the second specificity. Its binding epitope mapping, species cross-reactivity, tumor cell line staining, and in vitro cytotoxicity were investigated thoroughly. Its potency in driving tumor-infiltrating lymphocytes (TILs) was quantified using bioluminescence, correlated with in vivo antitumor response against cell line-derived or patient-derived xenografts (CDXs or PDXs) and compared with anti-STEAP1 T-BsAbs built on representative antibody platforms.

RESULTS: BC261 binding epitope was mapped to its second extracellular domain of STEAP1 shared among canine and primate orthologs. BC261 induced potent cytotoxicity against panels of EFT, prostate cancer, and canine osteosarcoma cell lines despite their low antigen density. BC261 drove significantly more TILs into tumors (30-fold) and exerted superior antitumor effects compared with the other standard BsAb platforms. The antitumor efficacy of BC261 was consistent against EFT and prostate cancer CDXs and PDXs.

CONCLUSIONS: BC261 was highly efficient in driving T cell infiltration and tumor ablation. Either as stand-alone therapeutics or for ex vivo armed T cells, this novel anti-STEAP1 T-BsAb BC261 has therapeutic potential.

PMID:34497115 | DOI:10.1136/jitc-2021-003114

Clinicopathological features of malignant mixed mesodermal tumor: analysis of 50 cases

Fetched: September 10th, 2021, 5:02am GMT by X H Ding

Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):1008-1013. doi: 10.3760/cma.j.cn112151-20210126-00083.

ABSTRACT

Objective: To investigate the clinicpathological, immunohistochemical and molecular genetic features of malignant mixed mesodermal tumor (MMMT) in the female reproductive system. Methods: To analyze its histopathological characteristics, we performed a retrospective review of the MMMT cases diagnosed at PLA General Hospital, Beijing, China during 2005-2019 using its surgical and pathological databases. EnVision immunohistochemical staining was used to detect the expression of ER, PR, p16, p53 and MMR proteins. Results: Fifty cases were conformed to the diagnosis, including 29 cases originated in the uterus, 16 cases in ovary, 4 cases of synchronous occurrence in uterus and ovary, 1 case in cervix. The tumor was histologically composed of two components, namely carcinoma and sarcoma ones, with clear borderline or blend mutually. The proportion of cancer component in the whole tumor ranged from 5%-90%. The proportion of carcinoma was more than 50% in 76% of the cases, and less than 50% in 24% of cases, including 2 cases with<10% of carcinoma. In the cases of primary uterine MMMT, the main carcinoma type was high grade endometrioid carcinoma (55%, 16/29). In ovarian MMMT, the main carcinoma type was serous carcinoma (12/16), while that of cervical MMMT was squamous cell carcinoma. The others were clear cell carcinoma or the undifferentiated carcinoma. There was one carcinoma type in most cases, only 7 cases had two carcinoma types. Homologous sarcomas, including stromal sarcoma, leiomyosarcoma and high-grade spindle cell sarcomas, were more commonly found in uterine MMMT (72.4%, 21/29). While heterogenic sarcomas, including chondrosarcoma, osteosarcoma and rhabdomyosarcoma, were more commonly noted in ovarian MMMT (12/16) than MMMT of other sites. There were 10 cases that consisted of two types of sarcomas. The synchronous MMMT of uterus and ovary had similar morphology and the types of carcinoma and sarcoma. The tumor cells that spread or metastasized to lymph node, omentum, intestinal wall or skin were all carcinoma cells, and were morphologically consistent with the original tumors. Immunohistochemically, ER and PR were both negative (23/25 in uterine, 8/10 in ovarian tumors). p16 was strongly positive (11/11 in uterine tumors, and 6/6 in ovarian tumors), with similar expression patterns in the carcinoma and sarcoma components. p53 showed mutant-type staining (64%, 21/33) and expressed synchronously in carcinoma and sarcoma components. p53 mutation was found in 35% cases of endometrial carcinoma and 46.7% cases of non-endometrial carcinoma. p53 mutation was also found in only 31.8% cases of heterogenic sarcomas, but in 50% of non-heterogenic sarcomas. Twenty-eight cases (28/33, 85%) presented intact mismatch repair proteins, while 5 cases (5/33, 15%) presented deficient mismatch repair proteins. Conclusions: MMMT in female reproductive system is a rare high-grade biphasic tumor with complex and diverse morphology. The immunohistochemical features are characterized by negative ER/PR and strongly positive p16, mostly mutant p53 and proficient mismatch repair proteins. The patients with a high FIGO stage have worse prognosis.

PMID:34496490 | DOI:10.3760/cma.j.cn112151-20210126-00083

Standardization for pathological diagnosis of primary uterine sarcoma

Fetched: September 10th, 2021, 5:02am GMT by Collaborative Group of Female Reproductive System Disease, Chinese Society of Pathology

Zhonghua Bing Li Xue Za Zhi. 2021 Sep 8;50(9):987-993. doi: 10.3760/cma.j.cn112151-20210607-00415.

NO ABSTRACT

PMID:34496487 | DOI:10.3760/cma.j.cn112151-20210607-00415

BCR/ABL1-positive histiocytic sarcoma of the small intestine in a patient with chronic myeloid leukaemia

Fetched: September 9th, 2021, 5:01am GMT by Hiroshi Minato

Histopathology. 2021 Sep 8. doi: 10.1111/his.14563. Online ahead of print.

ABSTRACT

Histiocytic sarcoma (HS) is a rare myeloid-derived neoplasm with histiocytic differentiation and an aggressive clinical course. Recent studies suggest a common clonal origin between various haematolymphoid disorders. However, HS coexisting with chronic myeloid leukaemia (CML) without tyrosine kinase inhibitors (TKIs) has not been reported.

PMID:34496460 | DOI:10.1111/his.14563

Permalink for 'Outcome of patients with relapsed or progressive Ewing sarcoma enrolled on cooperative group phase 2 clinical trials: A report from the Children's Oncology Group'

Outcome of patients with relapsed or progressive Ewing sarcoma enrolled on cooperative group phase 2 clinical trials: A report from the Children's Oncology Group

Fetched: September 9th, 2021, 5:01am GMT by Anderson B Collier

Pediatr Blood Cancer. 2021 Sep 8:e29333. doi: 10.1002/pbc.29333. Online ahead of print.

ABSTRACT

SevenChildren's Oncology Group phase 2 trials for patients with relapsed/progressive solid tumors were analyzed to estimate the event-free survival (EFS) for relapsed/progressive Ewing sarcoma. One hundred twenty-eight Ewing sarcoma patients were enrolled and 124 events occurred. The 6-month EFS was 12.7%, demonstrating the poor outcome of these patients. Only docetaxel achieved its protocol-specified radiographic response rate for activity; however, the EFS for docetaxel was similar to other agents, indicating that a higher radiographic response rate may not translate into superior disease control. This EFS benchmark could be utilized as an additional endpoint in trials for recurrent Ewing sarcoma.

PMID:34496122 | DOI:10.1002/pbc.29333

Surgical and oncologic outcomes following arterial resection and reconstruction for advanced solid tumors

Fetched: September 9th, 2021, 5:01am GMT by Sylvia M Cruz

J Surg Oncol. 2021 Sep 8. doi: 10.1002/jso.26665. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Although arterial involvement for advanced tumors is rare, vascular resection may be indicated to achieve complete tumor resection. Given the potential morbidity of this approach, we sought to evaluate perioperative outcomes, vascular graft patency, and survival among patients undergoing tumor excision with en bloc arterial resection and reconstruction.

METHODS: From 2010 to 2020, we identified nine patients with tumors encasing or extensively abutting major arterial structures for whom en bloc arterial resection and reconstruction was performed.

RESULTS: Mean age was 53 ± 20 years, and 89% were females. Diagnoses were primary sarcomas (5), recurrent gynecologic carcinomas (3), and benign retroperitoneal fibrosis (1). Tumors involved the infrarenal aorta (2), iliac arteries (6), and superficial femoral artery (1). Three patients (33%) had severe perioperative morbidity (Grade III + ) with no mortality. At a median follow-up of 23 months, eight patients (89%) had primary graft patency, and five patients (56%) had no evidence of disease.

CONCLUSIONS: Arterial resection and reconstruction as part of the multimodality treatment of regionally advanced tumors is associated with acceptable short- and long-term outcomes, including excellent graft patency. In appropriately selected patients, involvement of major arterial structures should not be viewed as a contraindication to attempted curative surgery.

PMID:34495553 | DOI:10.1002/jso.26665

Permalink for 'Significant improvement in survival of advanced stage childhood and young adolescent cancer in the Netherlands since the 1990s'

Significant improvement in survival of advanced stage childhood and young adolescent cancer in the Netherlands since the 1990s

Fetched: September 8th, 2021, 5:02am GMT by Maya Schulpen

Eur J Cancer. 2021 Sep 4;157:81-93. doi: 10.1016/j.ejca.2021.08.001. Online ahead of print.

ABSTRACT

BACKGROUND: This is the first national study on trends in cancer survival and mortality for children and young adolescents in the Netherlands including unique information on stage at diagnosis.

METHODS: All neoplasms in patients <18 years, diagnosed between 1990 and 2015 (N = 14,060), were derived from the Netherlands Cancer Registry. Cohort and period survival analyses were used to estimate observed survival (OS). Time trends in OS and mortality rates were evaluated by parametric survival models and average annual percentage change, respectively.

RESULTS: Between 1990 and 2015, 5-year OS and 10-year OS of childhood and young adolescent cancer have improved significantly by 9 percent points, reaching 81% and 78%, respectively. Favourable trends in survival were observed for all age groups and most diagnostic (sub)groups, being particularly pronounced for advanced disease. Non-Hodgkin lymphomas Ann Arbor stage III, metastatic neuroblastomas (age ≥18 months) and Ewing bone sarcomas showed significant improvements in 5-year OS. Compared with 1990-99, the risk of dying within five years of diagnosis was decreased significantly during 2000-09 (hazard ratio [HR] = 0.8) and 2010-15 (HR = 0.6), after adjustment for age, gender and follow-up time. Nonetheless, the prognosis of young patients suffering from central nervous system tumours, neuroblastoma and osteosarcomas remained modest, with 5-year OS <70% and 10-year OS <65%. Childhood and young adolescent cancer mortality decreased by an average of 2.0% annually between 1990 and 2018.

CONCLUSIONS: Significant progress has been realised in the prognosis of childhood and young adolescent cancer in the Netherlands since the 1990s. Survival improvements were especially evident for patients with advanced stages and were also reflected in the declining mortality rates.

PMID:34492587 | DOI:10.1016/j.ejca.2021.08.001

Permalink for 'Kaposi's sarcoma-associated herpesvirus promotes mesenchymal-to-endothelial transition by resolving the bivalent chromatin of PROX1 gene'

Kaposi's sarcoma-associated herpesvirus promotes mesenchymal-to-endothelial transition by resolving the bivalent chromatin of PROX1 gene

Fetched: September 8th, 2021, 5:02am GMT by Yao Ding

PLoS Pathog. 2021 Sep 7;17(9):e1009847. doi: 10.1371/journal.ppat.1009847. Online ahead of print.

ABSTRACT

Increasing evidence suggests that Kaposi's sarcoma (KS) arises from Kaposi's sarcoma-associated herpesvirus (KSHV)-infected mesenchymal stem cells (MSCs) through mesenchymal-to-endothelial transition (MEndT). KSHV infection promotes MSC differentiation of endothelial lineage and acquisition of tumorigeneic phenotypes. To understand how KSHV induces MEndT and transforms MSCs to KS cells, we investigated the mechanism underlying KSHV-mediated MSC endothelial lineage differentiation. Like embryonic stem cells, MSC differentiation and fate determination are under epigenetic control. Prospero homeobox 1 (PROX1) is a master regulator that controls lymphatic vessel development and endothelial differentiation. We found that the PROX1 gene in MSCs harbors a distinctive bivalent epigenetic signature consisting of both active marker H3K4me3 and repressive marker H3K27me3, which poises expression of the genes, allowing timely activation upon differentiation signals or environmental stimuli. KSHV infection effectively resolves the bivalent chromatin by decreasing H3K27me3 and increasing H3K4me3 to activate the PROX1 gene. vIL-6 signaling leads to the recruitment of MLL2 and SET1 complexes to the PROX1 promoter to increase H3K4me3, and the vGPCR-VEGF-A axis is responsible for removing PRC2 from the promoter to reduce H3K27me3. Therefore, through a dual signaling process, KSHV activates PROX1 gene expression and initiates MEndT, which renders MSC tumorigenic features including angiogenesis, invasion and migration.

PMID:34492084 | DOI:10.1371/journal.ppat.1009847

Paraneoplastic pemphigus associated with interdigitating dendritic cell sarcoma

Fetched: September 8th, 2021, 5:02am GMT by Jing Jing

Indian J Dermatol Venereol Leprol. 2021 Aug 28:1-3. doi: 10.25259/IJDVL_173_2021. Online ahead of print.

NO ABSTRACT

PMID:34491664 | DOI:10.25259/IJDVL_173_2021

Permalink for 'Neurosarcoidosis Pathologically Diagnosed Via Biopsy of a Normal-Sized Inguinal Lymph Node With Fluorodeoxyglucose Accumulation on Positron Emission Tomography/Computed Tomography in a Patient With a History of Brain Ewing's Sarcoma'

Neurosarcoidosis Pathologically Diagnosed Via Biopsy of a Normal-Sized Inguinal Lymph Node With Fluorodeoxyglucose Accumulation on Positron Emission Tomography/Computed Tomography in a Patient With a History of Brain Ewing's Sarcoma

Fetched: September 8th, 2021, 5:02am GMT by Masayuki Fuwa

Mod Rheumatol Case Rep. 2021 Aug 28:rxab019. doi: 10.1093/mrcr/rxab019. Online ahead of print.

ABSTRACT

Neurosarcoidosis is a rare disease and is often difficult to diagnose. Herein, we report a case of neurosarcoidosis in a patient with a history of Ewing's sarcoma of the brain. He presented with fever of unknown origin, and a pathological diagnosis was obtained via biopsy of a normal-sized inguinal lymph node with fluorodeoxyglucose (FDG) accumulation on positron emission tomography/computed tomography (PET/CT). The condition could not have been diagnosed without FDG-PET/CT.

PMID:34491365 | DOI:10.1093/mrcr/rxab019

High SHP2 expression determines the efficacy of PD-1/PD-L1 inhibitors in advanced KRAS mutant non-small cell lung cancer

Fetched: September 8th, 2021, 5:02am GMT by Hui-Bo Feng

Thorac Cancer. 2021 Sep 6. doi: 10.1111/1759-7714.14137. Online ahead of print.

ABSTRACT

BACKGROUND: Src homology region 2 domain-containing phosphatase 2 (SHP2) is a novel target for Kirsten rat sarcoma oncogene (KRAS) mutant cancer. We retrospectively studied the significance of SHP2 in KRAS mutant non-small cell lung cancer (NSCLC) treated with immunotherapy and its relationship with tumor microenvironment (TME).

METHODS: Sixty-one advanced KRAS mutant NSCLC patients who underwent immunotherapy were enrolled. Next-generation sequencing (NGS) was used to profile mutation status. The expression of SHP2, phospho-SHP2 (pSHP2), and programmed death ligand 1 (PD-L1) were analyzed by immunohistochemistry (IHC). Quantitative multiplexed immunofluorescence cytochemistry (mIFC) analysis was conducted to describe the TME.

RESULTS: SHP2 was heterogeneously expressed in 32 samples in both tumor cells and immune cells and highly expressed (H-score >10) in 25 (78.1%) samples. The expression levels of SHP2 and pSHP2 were positively correlated. Stromal SHP2 (s-SHP2) was higher in tumors with PD-L1 ≥50% versus PD-L1 <50% (p = 0.039). By quantitative mIFC analysis, the expression of s-SHP2 had positive correlation with CD8, CD4, CD68, and PD-L1 levels in stromal area. Patients with high SHP2 expression made up 100.0% of the partial respond (PR) and 80.0% of the stable disease (SD), whereas 50.0% of the progress disease (PD). High SHP2 expression was associated with longer progression-free survival (PFS) and overall survival (OS) (p < 0.001, p = 0.013). Patients with high expression of both SHP2 and PD-L1 had longer PFS (p < 0.001).

CONCLUSION: High SHP2 expression could predict the efficacy of immunotherapy and better survival in advanced KRAS mutant NSCLC. SHP2 may function in both tumor cells and immune cells, warranting further study on the potential diverse effects of SHP2 inhibition in TME.

PMID:34490728 | DOI:10.1111/1759-7714.14137

Micrographic Surgery Allows Fascia Preservation in Dermatofibro-sarcoma Protuberans

Fetched: September 8th, 2021, 5:02am GMT by Maximilian Gassenmaier

Acta Derm Venereol. 2021 Sep 7. doi: 10.2340/00015555-3915. Online ahead of print.

ABSTRACT

Removal of the deep fascia is recommended in therapy for dermatofibrosarcoma protuberans, but its necessity in the context of micrographic surgery is unclear. A retrospective clinicopathological analysis of 48 patients with dermatofibrosarcoma protuberans treated by micrographic surgery was performed, to determine in which tumours fascia preservation was feasible and safe. Histologically, 93% of tumours on the trunk and extremities and 14% of tumours in the head and neck region were fully located above the fascia. Localization on the head and neck was the only significant risk factor for tumour extension beyond the subcutis (p<0.001). Overall, 44% of tumours were completely excised above the fascia and 56% with deeper excisions. Two deeply infiltrating tumours (4%) on the head recurred, but in none of these lesions was the fascia spared. These results show that micrographic surgery allows fascia preservation in superficial tumours outside the head and neck region.

PMID:34490467 | DOI:10.2340/00015555-3915

Beyond the Driver Mutation: Immunotherapies in Gastrointestinal Stromal Tumors

Fetched: September 8th, 2021, 5:02am GMT by Matthieu Roulleaux Dugage

Front Immunol. 2021 Aug 20;12:715727. doi: 10.3389/fimmu.2021.715727. eCollection 2021.

ABSTRACT

Gastrointestinal stromal tumors (GISTs) are a subtype of soft tissue sarcoma (STS), and have become a concept of oncogenic addiction and targeted therapies.The large majority of these tumors develop after a mutation in KIT or platelet derived growth factor receptor α (PDGFRα), resulting in uncontrolled proliferation. GISTs are highly sensitive to imatinib. GISTs are immune infiltrated tumors with a predominance of tumor-associated macrophages (TAMs) and T-cells, including many CD8+ T-cells, whose numbers are prognostic. The genomic expression profile is that of an inhibited Th1 response and the presence of tertiary lymphoid structures and B cell signatures, which are known as predictive to response to ICI. However, the microtumoral environment has immunosuppressive attributes, with immunosuppressive M2 macrophages, overexpression of indoleamine 2,3-dioxygenase (IDO) or PD-L1, and loss of major histocompatibility complex type 1. In addition to inhibiting the KIT oncogene, imatinib appears to act by promoting cytotoxic T-cell activity, interacting with natural killer cells, and inhibiting the expression of PD-L1. Paradoxically, imatinib also appears to induce M2 polarization of macrophages. There have been few immunotherapy trials with anti-CTLA-4 or anti-PD-L1drugs and available clinical data are not very promising. Based on this comprehensive analysis of TME, we believe three immunotherapeutic strategies must be underlined in GIST. First, patients included in clinical trials must be better selected, based on the identified driver mutation (such as PDGFRα D842V mutation), the presence of tertiary lymphoid structures (TLS) or PD-L1 expression. Moreover, innovative immunotherapeutic agents also provide great interest in GIST, and there is a strong rationale for exploring IDO targeting after disease progression during imatinib therapy. Finally and most importantly, there is a strong rationale to combine of c-kit inhibition with immune checkpoint inhibitors.

PMID:34489967 | PMC:PMC8417712 | DOI:10.3389/fimmu.2021.715727

Mithramycin delivery systems to develop effective therapies in sarcomas

Fetched: September 8th, 2021, 5:02am GMT by Óscar Estupiñán

J Nanobiotechnology. 2021 Sep 6;19(1):267. doi: 10.1186/s12951-021-01008-x.

ABSTRACT

BACKGROUND: Sarcomas comprise a group of aggressive malignancies with very little treatment options beyond standard chemotherapy. Reposition of approved drugs represents an attractive approach to identify effective therapeutic compounds. One example is mithramycin (MTM), a natural antibiotic which has demonstrated a strong antitumour activity in several tumour types, including sarcomas. However, its widespread use in the clinic was limited by its poor toxicity profile.

RESULTS: In order to improve the therapeutic index of MTM, we have loaded MTM into newly developed nanocarrier formulations. First, polylactide (PLA) polymeric nanoparticles (NPs) were generated by nanoprecipitation. Also, liposomes (LIP) were prepared by ethanol injection and evaporation solvent method. Finally, MTM-loaded hydrogels (HG) were obtained by passive loading using a urea derivative non-peptidic hydrogelator. MTM-loaded NPs and LIP display optimal hydrodynamic radii between 80 and 105 nm with a very low polydispersity index (PdI) and encapsulation efficiencies (EE) of 92 and 30%, respectively. All formulations show a high stability and different release rates ranging from a fast release in HG (100% after 30 min) to more sustained release from NPs (100% after 24 h) and LIP (40% after 48 h). In vitro assays confirmed that all assayed MTM formulations retain the cytotoxic, anti-invasive and anti-stemness potential of free MTM in models of myxoid liposarcoma, undifferentiated pleomorphic sarcoma and chondrosarcoma. In addition, whole genome transcriptomic analysis evidenced the ability of MTM, both free and encapsulated, to act as a multi-repressor of several tumour-promoting pathways at once. Importantly, the treatment of mice bearing sarcoma xenografts showed that encapsulated MTM exhibited enhanced therapeutic effects and was better tolerated than free MTM.

CONCLUSIONS: Overall, these novel formulations may represent an efficient and safer MTM-delivering alternative for sarcoma treatment.

PMID:34488783 | PMC:PMC8419920 | DOI:10.1186/s12951-021-01008-x

Permalink for 'Postoperative liver metastasis of primary inflammatory pseudotumorous follicular dendritic cell sarcoma of the spleen: a case report'

Postoperative liver metastasis of primary inflammatory pseudotumorous follicular dendritic cell sarcoma of the spleen: a case report

Fetched: September 8th, 2021, 5:02am GMT by Run-Hua Pang

Ann Palliat Med. 2021 Aug;10(8):9297-9303. doi: 10.21037/apm-21-2155.

ABSTRACT

Follicular dendritic cell sarcoma (FDCS) is a rare and low incidence tumor. So far, there is no standard treatment for the disease. Surgery is the main treatment for FDCS. Here, we report the case of a 51-year-old woman who was admitted with the chief complaint of "spleen-occupying lesion detected via physical examination more than 1 month ago". Spiral plain scan and enhanced computed tomography (CT) of the upper abdomen showed a vascular-derived tumor, with a high possibility of hemangioma or hemangiolymphangioma. Later, on 25 December 2019, the patient underwent laparoscopic splenectomy, and the pathological diagnosis of primary splenic FDCS was made. The patient did not receive adjuvant chemotherapy or radiotherapy. At a regular follow-up inspection 11 months later, on 18 December 2020, an abdominal scan + enhanced CT revealed multiple new abnormal intrahepatic nodules, which combined with her history, indicated the possibility of metastases. Subsequently, she was readmitted to hospital and surgical treatment was decided upon after multi-disciplinary consultation and discussion. Laparoscopic S1/S3/S4/S5 hepatectomy plus cholecystectomy was performed on 31 December 2020. The postoperative pathology findings revealed (liver tumor, S3, S4, S5) metastatic inflammatory pseudotumor follicular dendritic sarcoma, tumor diameter 0.6-1.2 cm. Due to the lack of clinical reports on postoperative organ metastasis of this disease, less experience, and controversy in drug selection of radiotherapy and chemotherapy, the patient refused to receive radiotherapy and chemotherapy. She now undertakes regular outpatient reexamination, and has been followed-up until now, during which time she has not progressed, and the efficacy is considered satisfactory.

PMID:34488416 | DOI:10.21037/apm-21-2155

Permalink for 'Targeting the Nuclear Receptor-Binding SET Domain Family of Histone Lysine Methyltransferases for Cancer Therapy: Recent Progress and Perspectives'

Targeting the Nuclear Receptor-Binding SET Domain Family of Histone Lysine Methyltransferases for Cancer Therapy: Recent Progress and Perspectives

Fetched: September 8th, 2021, 5:02am GMT by Aarajana Shrestha

J Med Chem. 2021 Sep 7. doi: 10.1021/acs.jmedchem.1c01116. Online ahead of print.

ABSTRACT

Nuclear receptor-binding SET domain (NSD) proteins are a class of histone lysine methyltransferases (HKMTases) that are amplified, mutated, translocated, or overexpressed in various types of cancers. Several campaigns to develop NSD inhibitors for cancer treatment have begun following recent advances in knowledge of NSD1, NSD2, and NSD3 structures and functions as well as the U.S. FDA approval of the first HKMTase inhibitor (tazemetostat, an EZH2 inhibitor) to treat follicular lymphoma and epithelioid sarcoma. This perspective highlights recent findings on the structures of catalytic su(var), enhancer-of-zeste, trithorax (SET) domains and other functional domains of NSD methyltransferases. In addition, recent progress and efforts to discover NSD-specific small molecule inhibitors against cancer-targeting catalytic SET domains, plant homeodomains, and proline-tryptophan-tryptophan-proline domains are summarized.

PMID:34488340 | DOI:10.1021/acs.jmedchem.1c01116

Mobile Spine Osseous Sarcoma: Descriptive Epidemiological Analysis Based on a National Population-Based Study

Fetched: September 8th, 2021, 5:02am GMT by Adel Ebrahimpour

Arch Iran Med. 2021 Aug 1;24(8):629-635. doi: 10.34172/aim.2021.89.

ABSTRACT

BACKGROUND: Primary osseous sarcomas of the mobile spine are rare bony tumors. Ewing sarcoma, chondrosarcoma, chordoma and osteosarcoma constitute the majority of primary bone sarcomas of the spine; however, other rare sarcoma tumors may also affect the spine. In order to perform an epidemiological study of theses tumors, national registries may help to evaluate data for populations with similar characteristics.

METHODS: A population-based study was designed based on data from the Iran National Cancer Registry (INCR). All morphology codes (M-Code) of primary osseous sarcomas of the mobile spine (C-code 41.2) were derived and analyzed.

RESULTS: Among 186 patients with primary osseous sarcomas of the mobile spine, 67.2% were men and 32.8% were women. The median (IQR) age was 37.0 (20.0-59.0) years and the age-standardized incidence rate (ASIR) was 0.37 per million. The majority of cases of Ewing sarcoma (29.5%) were observed in the age group 20-25 years. Among male patients with chondrosarcoma, the median age was 39.0 (30.0-50.0), while females showed a median age of 56.0 (50.0-59.0). The median age of patients with chordoma was 54.0 (47.0-63.0) years. The crude incidence rate of mobile spine osteosarcoma was 0.04 per million.

CONCLUSION: Ewing sarcoma was the most frequent primary osseous sarcoma of the mobile spine. A male predilection was observed among all major sarcomas of the mobile spine. Ewing sarcoma in Iran affects the mobile spine in slightly older ages compared to other studies. Myxoid chondrosarcoma is the most frequent subtype of the mobile spine chondrosarcoma. Chordoma affects male in older ages compared to females. In contrast with other studies which showed a bimodal distribution of osteosarcoma of the spine including young adult and older age groups, 86% of cases in Iran were in the age group of 10-40 years.

PMID:34488331 | DOI:10.34172/aim.2021.89

Incidence of Hematopoietic Sarcoma in Iranian Population

Fetched: September 8th, 2021, 5:02am GMT by Maryam Mabani

Arch Iran Med. 2021 Jun 1;24(6):461-466. doi: 10.34172/aim.2021.66.

ABSTRACT

BACKGROUND: The heterogeneous nature of hematopoietic sarcoma has restricted the diagnosis and treatment of this disease to the extent that annually, several patients lose their lives. Given the lack of comprehensive epidemiologic information on the incidence of hematopoietic sarcoma in the Iranian population, we designed the present study to evaluate the distribution pattern of this disease.

METHODS: In this national population-based cancer registry study, we collected data from patients diagnosed with hematopoietic sarcoma who were registered in the Iran National Cancer Registry (INCR) between 2009 and 2013. For each patient, the variables of age, sex, province, year of diagnosis, site of involvement and morphology were collected.

RESULTS: In 45 cases from 18 provinces of Iran, we found that the incidence rate of the disease was 0.60 (95% CI: 0.44-0.80) per million persons. Among all provinces, Ilam had the highest incidence of hematopoietic sarcoma with a rate of 2 (95% CI: 0.05- 11.14) per million persons, while Isfahan had the lowest incidence with a rate of 0.21 (95% CI: 0.01-1.16) per million persons. The incidence rate of the disease increased with age and the disease was slightly more common in men (0.63 [95% CI: 0.41-0.94] vs. 0.56 [95% CI: 0.35-0.86] per million persons). The frequency of hematopoietic sarcoma in connective and soft tissues was higher than other anatomical sites and we found that myeloid morphology was the most prevalent morphology.

CONCLUSION: The resulting data provided a valuable perspective on the distribution pattern of hematopoietic sarcoma in Iran; however, further studies are required to confirm these results.

PMID:34488308 | DOI:10.34172/aim.2021.66

Primary Hodgkin's Lymphoma of bone in 7-year-old- an exceptional case report of youngest child in literature

Fetched: September 8th, 2021, 5:02am GMT by Durr-E-Amna Siddiqui

Cancer Treat Res Commun. 2021 Aug 19;29:100448. doi: 10.1016/j.ctarc.2021.100448. Online ahead of print.

ABSTRACT

Hodgkin lymphoma (HL) is mainly considered a nodal disease but extra nodal involvement can also be seen with variable frequency. Solitary bone involvement in HL known as Primary osseous hodgkin lymphoma (POHL) is very rare. It is defined as a lymphoma that is restricted to the bone without any simultaneous organ or lymph node involvement at the time of initial diagnosis. Primary Hodgkin lymphoma of the bone can be very challenging to diagnose because of its rarity especially in children and its variable presentation. Here we report the youngest case of POHL in a girl 7 years of age. She presented with the left leg pain and B-symptoms of fever and weight loss. Initial workup and imaging were suggestive of infection or a bone tumor. Bone biopsy of left hip joint after the multiple courses of antibiotics revealed Reed Sternberg cells in the mixed inflammatory background with CD30 and PAX-5 immunohistochemical positivity confirming classical hodgkin lymphoma, mixed cellularity type. CD99 and CD1a were negative excluding ewing sarcoma and langerhan cell Histiocytosis respectively. Absence of significant lymphadenopathy or visceromegaly on staging computerized tomography (CT) scan confirmed a very rare POHL. She received standard conventional chemotherapy with radiation. Our patient is in remission for five years after treatment.

PMID:34488186 | DOI:10.1016/j.ctarc.2021.100448

Camsirubicin + Pegfilgrastim to Determine MTD in ASTS

Posted: September 14th, 2021, 2:00pm GMT

Condition: Advanced Soft-tissue Sarcoma
Intervention: Drug: Camsirubicin
Sponsor: Monopar Therapeutics
Not yet recruiting

Lurbinectedin With or Without Irinotecan in Treating Patients With Relapsed or High Risk Metastatic Ewing Sarcoma

Posted: September 13th, 2021, 2:00pm GMT

Conditions: Metastatic Ewing Sarcoma; Recurrent Ewing Sarcoma
Interventions: Procedure: Fine-Needle Aspiration; Drug: Irinotecan; Drug: Irinotecan Hydrochloride; Drug: Lurbinectedin
Sponsors: M.D. Anderson Cancer Center; National Cancer Institute (NCI)
Not yet recruiting

IACS-6274 With or Without Pembrolizumab for the Treatment of Advanced Solid Tumors

Posted: September 10th, 2021, 2:00pm GMT

Conditions: Advanced Endometrial Carcinoma; Advanced Head and Neck Squamous Cell Carcinoma; Advanced Malignant Solid Neoplasm; Advanced Melanoma; Advanced Ovarian Clear Cell Adenocarcinoma; Chondrosarcoma; Clinical Stage III Cutaneous Melanoma AJCC v8; Clinical Stage IV Cutaneous Melanoma AJCC v8; Pathologic Stage III Cutaneous Melanoma AJCC v8; Pathologic Stage IIIA Cutaneous Melanoma AJCC v8; Pathologic Stage IIIB Cutaneous Melanoma AJCC v8; Pathologic Stage IIIC Cutaneous Melanoma AJCC v8; Pathologic Stage IIID Cutaneous Melanoma AJCC v8; Pathologic Stage IV Cutaneous Melanoma AJCC v8; Refractory Endometrial Carcinoma; Refractory Head and Neck Squamous Cell Carcinoma; Refractory High Grade Ovarian Serous Adenocarcinoma; Refractory Melanoma; Refractory Ovarian Clear Cell Adenocarcinoma; Stage III Ovarian Cancer AJCC v8; Stage III Uterine Corpus Cancer AJCC v8; Stage IIIA Ovarian Cancer AJCC v8; Stage IIIA Uterine Corpus Cancer AJCC v8; Stage IIIA1 Ovarian Cancer AJCC v8; Stage IIIA2 Ovarian Cancer AJCC v8; Stage IIIB Ovarian Cancer AJCC v8; Stage IIIB Uterine Corpus Cancer AJCC v8; Stage IIIC Ovarian Cancer AJCC v8; Stage IIIC Uterine Corpus Cancer AJCC v8; Stage IIIC1 Uterine Corpus Cancer AJCC v8; Stage IIIC2 Uterine Corpus Cancer AJCC v8; Stage IV Ovarian Cancer AJCC v8; Stage IV Uterine Corpus Cancer AJCC v8; Stage IVA Ovarian Cancer AJCC v8; Stage IVA Uterine Corpus Cancer AJCC v8; Stage IVB Ovarian Cancer AJCC v8; Stage IVB Uterine Corpus Cancer AJCC v8
Interventions: Drug: Glutaminase Inhibitor IPN60090; Biological: Pembrolizumab
Sponsor: M.D. Anderson Cancer Center
Recruiting

Permalink for 'A Phase I Trial of T Cell Receptor Gene Therapy Targeting KK-LC-1 for Gastric, Breast, Cervical, Lung and Other KK-LC-1 Positive Epithelial Cancers'

A Phase I Trial of T Cell Receptor Gene Therapy Targeting KK-LC-1 for Gastric, Breast, Cervical, Lung and Other KK-LC-1 Positive Epithelial Cancers

Posted: September 6th, 2021, 2:00pm GMT

Condition: Kita-kyushu Lung Cancer Antigen 1, Human
Interventions: Drug: IL-2 (Aldesleukin); Drug: Cyclophosphamide; Biological: KK-LC-1 TCR; Drug: Fludarabine
Sponsor: National Cancer Institute (NCI)
Not yet recruiting

Permalink for 'Comparing Carbon Ion Therapy, Surgery, and Proton Therapy for the Management of Pelvic Sarcomas Involving the Bone, the PROSPER Study'

Comparing Carbon Ion Therapy, Surgery, and Proton Therapy for the Management of Pelvic Sarcomas Involving the Bone, the PROSPER Study

Posted: September 2nd, 2021, 2:00pm GMT

Conditions: Bone Sarcoma; Chondrosarcoma; Chordoma; Ewing Sarcoma of Bone; Pelvic Rhabdomyosarcoma
Interventions: Other: Electronic Health Record Review; Other: Quality-of-Life Assessment
Sponsors: Mayo Clinic; National Cancer Institute (NCI)
Not yet recruiting

Molecular Basis of Langerhans and Non-Langerhans Cell Histiocytic Neoplasms and Castleman Disease

Posted: August 31st, 2021, 2:00pm GMT

Conditions: Histiocytic Disorders; Castleman's Disease (CD); Langerhans Cell Histiocytosis (LCH); Erdhiem Chester Disease (ECD); Rosai-Dorfman Disease (RDD); Indeterminate Dendritic Cell Tumor; Interdigitating Dendritic Cell Sarcoma; Follicular Dendritic Cell Sarcoma (FDCS); Fibroblastic Reticular Cell Tumor; Unicentric Castleman Disease; Multicentric Castleman Disease Including TAFRO
Intervention: Diagnostic Test: Genetic testing
Sponsor: Case Comprehensive Cancer Center
Not yet recruiting

Sintilimab for the Treatment of Locally Advanced, Metastatic, or Recurrent Angiosarcoma, the SiARa Cancer Study

Posted: August 30th, 2021, 2:00pm GMT

Conditions: Locally Advanced Angiosarcoma; Metastatic Angiosarcoma; Recurrent Angiosarcoma
Interventions: Other: Quality-of-Life Assessment; Biological: Sintilimab
Sponsor: M.D. Anderson Cancer Center
Not yet recruiting

Perioperative Use of Tranexamic (TXA) in Bone Tumor Surgery Reduce Blood Loss and Transfusion Requirements.

Posted: August 27th, 2021, 2:00pm GMT

Conditions: Ewing Sarcoma of Bone; Osteosarcoma
Interventions: Drug: Tranexamic acid injection; Drug: Saline
Sponsor: Children's Cancer Hospital Egypt 57357
Recruiting

Phase II Study of Tazemetostat in Solid Tumors Harboring an ARID1A Mutation

Posted: August 26th, 2021, 2:00pm GMT

Conditions: Solid Tumor; ARID1A Gene Mutation
Intervention: Drug: Tazemetostat
Sponsors: Prisma Health-Upstate; Epizyme, Inc.
Not yet recruiting

Permalink for 'Atezolizumab and Cabozantinib for the Treatment of Adolescents and Young Adults With Recurrent or Metastatic Osteosarcoma, TACOS Study'

Atezolizumab and Cabozantinib for the Treatment of Adolescents and Young Adults With Recurrent or Metastatic Osteosarcoma, TACOS Study

Posted: August 25th, 2021, 2:00pm GMT

Conditions: Locally Advanced Osteosarcoma; Metastatic Osteosarcoma; Recurrent Osteosarcoma; Refractory Osteosarcoma; Unresectable Osteosarcoma
Interventions: Biological: Atezolizumab; Drug: Cabozantinib
Sponsor: M.D. Anderson Cancer Center
Not yet recruiting

Permalink for 'Sintilimab for the Treatment of Locally Advanced, Metastatic, Recurrent, or Unresectable Undifferentiated Pleomorphic Sarcoma, SiARa Cancer Study'

Sintilimab for the Treatment of Locally Advanced, Metastatic, Recurrent, or Unresectable Undifferentiated Pleomorphic Sarcoma, SiARa Cancer Study

Posted: August 23rd, 2021, 2:00pm GMT

Conditions: Locally Advanced Undifferentiated Pleomorphic Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Recurrent Undifferentiated Pleomorphic Sarcoma; Unresectable Undifferentiated Pleomorphic Sarcoma
Intervention: Biological: Sintilimab
Sponsors: M.D. Anderson Cancer Center; National Cancer Institute (NCI)
Recruiting

Mutant Kirsten Rat Sarcoma (KRAS) -Targeted Long Peptide Vaccine for Patients at High Risk of Developing Pancreatic Cancer

Posted: August 19th, 2021, 2:00pm GMT

Conditions: High Risk Cancer; Pancreatic Cancer
Intervention: Drug: KRAS peptide vaccine
Sponsors: Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins; Stand Up To Cancer
Not yet recruiting

A Phase I, Study Evaluating the Safety, Tolerability, PK, and Efficacy of GH35 in Patients With Solid Tumors With KRAS Mutation

Posted: August 18th, 2021, 2:00pm GMT

Conditions: AdvAdvanced Solid Tumors; Kirsten Rat Sarcoma
Intervention: Drug: GH35 Tablet
Sponsor: Suzhou Genhouse Bio Co., Ltd.
Not yet recruiting

A Clinical Trial to Evaluate the Safety and Efficacy of AL2846 Capsules in Chinese Patients With Type I Neurofibromatosis

Posted: August 18th, 2021, 2:00pm GMT

Condition: Neurofibromatosis and Malignant Peripheral Nerve Sheath Tumors
Intervention: Drug: AL2846 capsules
Sponsor: Chia Tai Tianqing Pharmaceutical Group Co., Ltd.
Not yet recruiting

Permalink for 'Study to Evaluate the Safety, Tolerability, and Pharmacokinetics of GS-9716 as Monotherapy and in Combination With Anticancer Therapies in Adults With Solid Malignancies'

Study to Evaluate the Safety, Tolerability, and Pharmacokinetics of GS-9716 as Monotherapy and in Combination With Anticancer Therapies in Adults With Solid Malignancies

Posted: August 16th, 2021, 2:00pm GMT

Condition: Solid Malignancies
Interventions: Drug: GS-9716; Drug: Docetaxel; Drug: Sacituzumab Govitecan-hziy; Drug: Gemcitabine
Sponsor: Gilead Sciences
Not yet recruiting

Atezolizumab Plus Tivozanib in Immunologically Cold Tumor Types

Posted: August 11th, 2021, 2:00pm GMT

Conditions: Bile Duct Cancer; Gall Bladder Cancer; Breast Cancer; Neuroendocrine Tumors; Ovarian Cancer; Pancreatic Adenocarcinoma; Soft Tissue Sarcoma; Vulvar Cancer; Prostate Cancer
Interventions: Drug: Atezolizumab; Drug: Tivozanib
Sponsors: University of Florida; Genentech, Inc.; Aveo Oncology Pharmaceuticals
Not yet recruiting

A Study to Evaluate ELU001 in Patients With Solid Tumors That Overexpress Folate Receptor Alpha (FRα)

Posted: August 11th, 2021, 2:00pm GMT

Conditions: Ovarian Cancer; Ovarian Neoplasms; Ovarian Carcinoma; Endometrial Cancer; Colorectal Cancer; Gastric Cancer; Gastroesophageal Junction Adenocarcinoma; Gastric Adenocarcinoma; TNBC - Triple-Negative Breast Cancer; Triple Negative Breast Cancer; Cholangiocarcinoma; Bile Duct Cancer; Colon Cancer; Colon Cancer Stage; Colon Neoplasm; Colon Adenocarcinoma; Colon Rectal Cancer; Colon Cancer Liver Metastasis; Colorectal Neoplasms; Colorectal Carcinoma; Colorectal Adenocarcinoma; Colorectal Cancer Metastatic; Colorectal Cancer Stage IV; Rectum Cancer; Rectum Neoplasm; Rectum Carcinoma; Rectal Cancer; Rectal Neoplasms; Rectal Adenocarcinoma; Rectal Cancer Stage; Rectal Cancer Metastatic; Rectal Cancer Stage III; Esophagus Cancer; Esophageal Cancer; Esophageal Neoplasms; Gastric Cancer Stage; Breast Cancer; Breast Neoplasm; Breast Adenocarcinoma; Breast Cancer Stage IV; Breast Cancer Stage; Breast Cancer Metastatic; Breast Cancer Invasive; Triple Negative Breast Neoplasms; Intrahepatic Cholangiocarcinoma; Intrahepatic Cholangiocarcinoma Recurrent; Hilar Cholangiocarcinoma; Distal Cholangiocarcinoma; Ovary Cancer; Ovary Neoplasm; Ovary Disease; Ovary Metastasis; Ovarian Diseases; Ovarian Cancer Stage; Ovarian Epithelial Cancer; Ovarian Adenocarcinoma; Ovarian Serous Adenocarcinoma; Ovarian Neoplasm Epithelial; Ovarian Cancer Recurrent; Endometrial Diseases; Endometrial Adenocarcinoma; Endometrial Carcinosarcoma; Endometrial Clear Cell Adenocarcinoma; Endometrioid Adenocarcinoma; Endometrial Neoplasms; Endometrial Cancer Recurrent; Endometrioid Tumor; Fallopian Tube Cancer; Peritoneal Cancer
Intervention: Drug: ELU001
Sponsor: Elucida Oncology
Recruiting

A Trial of TTI-621 in Combination With Doxorubicin in Patients With Leiomyosarcoma

Posted: August 9th, 2021, 2:00pm GMT

Condition: Leiomyosarcoma
Interventions: Drug: TTI-621; Drug: Doxorubicin
Sponsor: Trillium Therapeutics Inc.
Recruiting

HER2 Chimeric Antigen Receptor (CAR) T Cells in Combination With Checkpoint Blockade in Patients With Advanced Sarcoma

Posted: August 6th, 2021, 2:00pm GMT

Conditions: Sarcoma; HER-2 Protein Overexpression
Interventions: Genetic: cells; Drug: Pembrolizumab Injectable Product; Drug: Nivolumab Injectable Product; Drug: Lymphodepletion Chemotherapy
Sponsors: Baylor College of Medicine; Center for Cell and Gene Therapy, Baylor College of Medicine; The Faris Foundation USA; Stand Up To Cancer; Triumph Over Kid Cancer Foundation; St. Baldrick's Foundation
Not yet recruiting

A Study to Compare Early Use of Vinorelbine and Maintenance Therapy for Patients With High Risk Rhabdomyosarcoma

Posted: August 6th, 2021, 2:00pm GMT

Conditions: Alveolar Rhabdomyosarcoma; Botryoid-Type Embryonal Rhabdomyosarcoma; Embryonal Rhabdomyosarcoma; Rhabdomyosarcoma; Solid Alveolar Rhabdomyosarcoma; Spindle Cell Rhabdomyosarcoma; Spindle Cell/Sclerosing Rhabdomyosarcoma
Interventions: Drug: Cyclophosphamide; Biological: Dactinomycin; Radiation: Radiation Therapy; Drug: Vincristine Sulfate; Drug: Vinorelbine Tartrate
Sponsor: Children's Oncology Group
Not yet recruiting

Analysis of the Molecular Profile of the Mixofibrosarcoma of the Extremities

Posted: August 5th, 2021, 2:00pm GMT

Condition: Sarcoma,Soft Tissue
Intervention: Other: case series review of clinical and histological data
Sponsor: Istituto Ortopedico Rizzoli
Not yet recruiting

Ultrasound Evaluation of the Myometrium Using the MUSA Terminology Comparison With Histology

Posted: August 4th, 2021, 2:00pm GMT

Conditions: Myoma;Uterus; Sarcoma Uterus
Intervention: Procedure: Hysterectomy
Sponsor: Universitaire Ziekenhuizen Leuven
Recruiting

Using QPOP to Predict Treatment for Sarcomas and Melanomas

Posted: August 3rd, 2021, 2:00pm GMT

Conditions: Sarcoma; Melanoma
Intervention:
Sponsor: National Cancer Centre, Singapore
Recruiting

FHD-609 in Subjects With Advanced Synovial Sarcoma

Posted: July 16th, 2021, 2:00pm GMT

Condition: Advanced Synovial Sarcoma
Intervention: Drug: FHD-609
Sponsor: Foghorn Therapeutics Inc.
Recruiting

Exercise Intervention for Bone Tumor Patients

Posted: July 15th, 2021, 2:00pm GMT

Condition: Bone Sarcoma
Interventions: Behavioral: Exercise therapy; Other: Information
Sponsors: Universität Duisburg-Essen; Ruhr University of Bochum
Recruiting

Accuracy of the Preoperative Molecular Diagnosis of Uterine Tumors by Liquid Biopsy

Posted: June 23rd, 2021, 2:00pm GMT

Conditions: Leiomyoma, Uterine; Leiomyosarcoma Uterus
Interventions: Procedure: Collection of Leiomyoma samples; Procedure: Collection of Leiomyosarcoma samples; Procedure: Collection of control samples
Sponsor: Igenomix
Recruiting

Permalink for 'APL-2 and Pembrolizumab Versus APL-2, Pembrolizumab and Bevacizumab Versus Bevacizumab Alone for the Treatment of Recurrent Ovarian, Fallopian Tube, or Primary Peritoneal Cancer and Malignant Effusion'

APL-2 and Pembrolizumab Versus APL-2, Pembrolizumab and Bevacizumab Versus Bevacizumab Alone for the Treatment of Recurrent Ovarian, Fallopian Tube, or Primary Peritoneal Cancer and Malignant Effusion

Posted: June 9th, 2021, 2:00pm GMT

Conditions: Fallopian Tube Carcinosarcoma; Fallopian Tube Adenocarcinoma; Fallopian Tube Serous Adenocarcinoma; Ovarian Carcinosarcoma; Ovarian Clear Cell Adenocarcinoma; Ovarian Endometrioid Adenocarcinoma; Ovarian Serous Adenocarcinoma; Primary Peritoneal Adenocarcinoma; Recurrent Fallopian Tube Carcinoma; Recurrent Ovarian Carcinoma; Recurrent Primary Peritoneal Carcinoma
Interventions: Biological: Bevacizumab; Biological: Pegcetacoplan; Biological: Pembrolizumab; Other: Quality-of-Life Assessment; Other: Questionnaire Administration
Sponsor: Roswell Park Cancer Institute
Not yet recruiting

NT-I7 for Kaposi Sarcoma in Patients With or Without HIV

Posted: May 19th, 2021, 2:00pm GMT

Conditions: AIDS-Related Kaposi Sarcoma; HIV Infection; Kaposi Sarcoma
Intervention: Biological: Efineptakin alfa
Sponsors: Fred Hutchinson Cancer Research Center; Cancer Immunotherapy Trials Network
Not yet recruiting

Study of Liposomal Annamycin for the Treatment of Subjects With Soft-Tissue Sarcomas (STS) With Pulmonary Metastases

Posted: May 14th, 2021, 2:00pm GMT

Conditions: Sarcoma,Soft Tissue; Pulmonary Metastasis
Intervention: Drug: Liposomal Annamycin (L-Annamycin)
Sponsor: Moleculin Biotech, Inc.
Recruiting

RegoNivo vs Standard of Care Chemotherapy in AGOC

Posted: May 10th, 2021, 2:00pm GMT

Condition: Gastro-Oesophageal Cancer
Interventions: Drug: Regorafenib; Biological: Nivolumab; Drug: Docetaxel; Drug: Paclitaxel; Drug: Irinotecan; Drug: Trifluridine/Tipracil
Sponsors: Australasian Gastro-Intestinal Trials Group; Bayer; Bristol-Myers Squibb; University of Sydney; Academic and Community Cancer Research United; Taiwanese Cooperative Oncology Group; Institut für Klinische Krebsforschung IKF GmbH at Krankenhaus Nordwest
Recruiting

$Permalink for 'Tegavivint for the Treatment of Recurrent or Refractory Solid Tumors, Including Lymphomas and Desmoid Tumors'$

Tegavivint for the Treatment of Recurrent or Refractory Solid Tumors, Including Lymphomas and Desmoid Tumors

Posted: April 20th, 2021, 2:00pm GMT

Conditions: Colorectal Carcinoma; Endometrial Carcinoma; Melanoma; Neuroblastoma; Ovarian Carcinoma; Pancreatic Ductal Adenocarcinoma; Recurrent Desmoid Fibromatosis; Recurrent Ewing Sarcoma; Recurrent Hepatoblastoma; Recurrent Hepatocellular Carcinoma; Recurrent Malignant Solid Neoplasm; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Refractory Desmoid Fibromatosis; Refractory Ewing Sarcoma; Refractory Hepatoblastoma; Refractory Hepatocellular Carcinoma; Refractory Malignant Solid Neoplasm; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Solid Pseudopapillary Neoplasm of the Pancreas; Wilms Tumor
Intervention: Drug: Tegavivint
Sponsor: Children's Oncology Group
Not yet recruiting

SARC038: Phase 2 Study of Regorafenib and Nivolumab in Osteosarcoma

Posted: March 18th, 2021, 2:00pm GMT

Conditions: Osteosarcoma; Osteosarcoma in Children; Osteosarcoma Recurrent; Osteosarcoma Metastatic
Interventions: Drug: Regorafenib 40 MG; Drug: Regorafenib 20MG; Drug: Nivolumab
Sponsor: Sarcoma Alliance for Research through Collaboration
Recruiting

Sequential Neoadjuvant Chemotherapy in Soft Tissue Sarcoma

Posted: March 1st, 2021, 3:00pm GMT

Condition: Soft Tissue Sarcoma
Interventions: Drug: Ifosfamide; Drug: Doxorubicin
Sponsors: Oslo University Hospital; Haukeland University Hospital
Recruiting

Permalink for 'A Study to Evaluate the Efficacy, Safety, Pharmacokinetics and Pharmacodynamics of ION363 in Amyotrophic Lateral Sclerosis Participants With Fused in Sarcoma Mutations (FUS-ALS)'

A Study to Evaluate the Efficacy, Safety, Pharmacokinetics and Pharmacodynamics of ION363 in Amyotrophic Lateral Sclerosis Participants With Fused in Sarcoma Mutations (FUS-ALS)

Posted: February 24th, 2021, 3:00pm GMT

Condition: Amyotrophic Lateral Sclerosis
Interventions: Drug: ION363; Drug: Placebo
Sponsor: Ionis Pharmaceuticals, Inc.
Recruiting

A Study of BPM31510 With Vitamin K1 in Subjects With Newly Diagnosed Glioblastoma (GB)

Posted: February 12th, 2021, 3:00pm GMT

Conditions: Glioblastoma; Glioblastoma Multiforme
Interventions: Drug: BPM31510; Other: Vitamin K1; Drug: Temozolomide (TMZ); Radiation: Radiation
Sponsor: Berg, LLC
Recruiting

Permalink for 'Aerosolized Azacytidine as Epigenetic Priming for Bintrafusp Alfa-Mediated Immune Checkpoint Blockade in Patients With Unresectable Pulmonary Metastases From Sarcomas, Germ Cell Tumors, or Epithelial Malignancies'

Aerosolized Azacytidine as Epigenetic Priming for Bintrafusp Alfa-Mediated Immune Checkpoint Blockade in Patients With Unresectable Pulmonary Metastases From Sarcomas, Germ Cell Tumors, or Epithelial Malignancies

Posted: December 2nd, 2020, 3:00pm GMT

Conditions: Sarcomas; Melanomas; Germ Cell Tumors; Epithelial Malignancies (Excluding Lung and Renal Cell Carcinomas); Pulmonary Metastases
Interventions: Device: AeroEclipse II Breath Actuated Nebulizer; Drug: Bintrafusp alfa; Drug: Azacytidine
Sponsor: National Cancer Institute (NCI)
Not yet recruiting

Permalink for 'A Study to Test Different Doses of BI 1701963 in Combination With Irinotecan in People With Advanced Bowel Cancer With Kirsten Rat Sarcoma Viral Oncogene Homologue (KRAS) Mutation'

A Study to Test Different Doses of BI 1701963 in Combination With Irinotecan in People With Advanced Bowel Cancer With Kirsten Rat Sarcoma Viral Oncogene Homologue (KRAS) Mutation

Posted: November 13th, 2020, 3:00pm GMT

Condition: Metastatic Colorectal Cancer (CRC)
Interventions: Drug: BI 1701963; Drug: Camptosar®
Sponsor: Boehringer Ingelheim
Recruiting

Surufatinib in Combination With Tislelizumab in Subjects With Advanced Solid Tumors

Posted: October 8th, 2020, 2:00pm GMT

Conditions: Metastatic Solid Tumor; Colorectal Cancer; Neuroendocrine Tumors; Small Cell Lung Cancer; Gastric Cancer; Soft Tissue Sarcoma
Interventions: Drug: Surufatinib and Tislelizumab _ Part 1; Drug: Surufatinib and Tislelizumab _ Part 2
Sponsors: Hutchison Medipharma Limited; BeiGene
Recruiting

Study of LY3410738 Administered to Patients With Advanced Solid Tumors With IDH1 Mutations

Posted: August 20th, 2020, 2:00pm GMT

Conditions: Cholangiocarcinoma; Chondrosarcoma; Glioma; Any Solid Tumor
Intervention: Drug: LY3410738
Sponsors: Eli Lilly and Company; Loxo Oncology, Inc.
Recruiting

Permalink for 'ENVASARC: Envafolimab And Envafolimab With Ipilimumab In Patients With Undifferentiated Pleomorphic Sarcoma Or Myxofibrosarcoma'

ENVASARC: Envafolimab And Envafolimab With Ipilimumab In Patients With Undifferentiated Pleomorphic Sarcoma Or Myxofibrosarcoma

Posted: July 21st, 2020, 2:00pm GMT

Conditions: Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma
Interventions: Biological: Envafolimab; Drug: Ipilimumab
Sponsor: Tracon Pharmaceuticals Inc.
Recruiting

X-ray Psoralen Activated Cancer Therapy in Head and Neck, Breast, Sarcoma and Melanoma

Posted: May 15th, 2020, 2:00pm GMT

Condition: Advanced Cancer
Intervention: Combination Product: X-PACT
Sponsor: Immunolight, LLC
Not yet recruiting

Trial in Patients With Metastatic or Locally Advanced Leiomyosarcoma

Posted: May 12th, 2020, 2:00pm GMT

Conditions: Leiomyosarcoma of Ovary; Soft Tissue Sarcoma
Interventions: Drug: Trabectedin; Drug: Gemcitabine; Drug: No Intervention: Observational Cohort
Sponsors: Italian Sarcoma Group; PharmaMar
Not yet recruiting

Testing the Addition of Nivolumab to Chemotherapy in Treatment of Soft Tissue Sarcoma

Posted: April 9th, 2020, 2:00pm GMT

Conditions: Skin Angiosarcoma; Skin Radiation-Related Angiosarcoma; Visceral Angiosarcoma
Interventions: Drug: Cabozantinib S-malate; Biological: Nivolumab; Drug: Paclitaxel; Other: Quality-of-Life Assessment; Other: Questionnaire Administration
Sponsor: National Cancer Institute (NCI)
Recruiting

A Study of Pembrolizumab Plus Local Chemotherapy Using Isolated Limb Infusion (ILI) for Patients With Sarcoma in the Arm or Leg

Posted: April 3rd, 2020, 2:00pm GMT

Conditions: Sarcoma; Myxofibrosarcoma; Undifferentiated Pleomorphic Sarcoma; Alveolar Soft Part Sarcoma
Interventions: Procedure: Isolated Limb Infusion; Drug: Pembrolizumab; Drug: infusion of melphalan and dactinomycin
Sponsor: Memorial Sloan Kettering Cancer Center
Recruiting

Permalink for 'Selpercatinib for the Treatment of Advanced Solid Tumors, Lymphomas, or Histiocytic Disorders With Activating RET Gene Alterations, a Pediatric MATCH Treatment Trial'

Selpercatinib for the Treatment of Advanced Solid Tumors, Lymphomas, or Histiocytic Disorders With Activating RET Gene Alterations, a Pediatric MATCH Treatment Trial

Posted: March 25th, 2020, 2:00pm GMT

Conditions: Hematopoietic and Lymphoid Cell Neoplasm; Recurrent Ependymoma; Recurrent Ewing Sarcoma; Recurrent Hepatoblastoma; Recurrent Histiocytic and Dendritic Cell Neoplasm; Recurrent Langerhans Cell Histiocytosis; Recurrent Lymphoma; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Malignant Solid Neoplasm; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Rhabdoid Tumor; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Recurrent WHO Grade II Glioma; Refractory Ependymoma; Refractory Ewing Sarcoma; Refractory Hepatoblastoma; Refractory Histiocytic and Dendritic Cell Neoplasm; Refractory Langerhans Cell Histiocytosis; Refractory Lymphoma; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Malignant Solid Neoplasm; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma; Refractory WHO Grade II Glioma; Wilms Tumor
Intervention: Drug: Selpercatinib
Sponsors: National Cancer Institute (NCI); Children's Oncology Group
Recruiting

Study on Trabectedin in Advanced Rearranged Mesenchymal Chondrosarcoma

Posted: March 12th, 2020, 2:00pm GMT

Condition: Mesenchymal Chondrosarcoma
Intervention: Drug: Trabectedin
Sponsors: Italian Sarcoma Group; PharmaMar
Not yet recruiting

NHS-IL12 Monotherapy and in Combination With M7824 in Advanced Kaposi Sarcoma

Posted: March 10th, 2020, 2:00pm GMT

Condition: Kaposi Sarcoma
Interventions: Drug: NHS-IL12; Drug: NHS-IL12+M7824
Sponsor: National Cancer Institute (NCI)
Recruiting

Study of AMG 650 in Adult Participants With Advanced Solid Tumors

Posted: March 3rd, 2020, 3:00pm GMT

Condition: Advanced Solid Tumors
Intervention: Drug: AMG 650
Sponsor: Amgen
Recruiting

Permalink for 'Tipifarnib for the Treatment of Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With HRAS Gene Alterations, a Pediatric MATCH Treatment Trial'

Tipifarnib for the Treatment of Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With HRAS Gene Alterations, a Pediatric MATCH Treatment Trial

Posted: February 26th, 2020, 3:00pm GMT

Conditions: Recurrent Adrenal Gland Pheochromocytoma; Recurrent Ectomesenchymoma; Recurrent Ependymoma; Recurrent Ewing Sarcoma; Recurrent Hepatoblastoma; Recurrent Kidney Wilms Tumor; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Medulloblastoma; Recurrent Melanoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Rhabdoid Tumor; Recurrent Rhabdoid Tumor of the Kidney; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Recurrent Thyroid Gland Carcinoma; Recurrent WHO Grade II Glioma; Refractory Adrenal Gland Pheochromocytoma; Refractory Ependymoma; Refractory Ewing Sarcoma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Medulloblastoma; Refractory Melanoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Rhabdoid Tumor; Refractory Rhabdoid Tumor of the Kidney; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma; Refractory Thyroid Gland Carcinoma; Refractory WHO Grade II Glioma
Intervention: Drug: Tipifarnib
Sponsor: National Cancer Institute (NCI)
Recruiting

Permalink for 'A Study of Abemaciclib (LY2835219) in Combination With Temozolomide and Irinotecan and Abemaciclib in Combination With Temozolomide in Children and Young Adult Participants With Solid Tumors'

A Study of Abemaciclib (LY2835219) in Combination With Temozolomide and Irinotecan and Abemaciclib in Combination With Temozolomide in Children and Young Adult Participants With Solid Tumors

Posted: January 23rd, 2020, 3:00pm GMT

Conditions: Relapsed Solid Tumor; Refractory Solid Tumor
Interventions: Drug: Abemaciclib; Drug: Irinotecan; Drug: Temozolomide
Sponsor: Eli Lilly and Company
Recruiting

Metarrestin (ML-246) in Subjects With Metastatic Solid Tumors

Posted: January 10th, 2020, 3:00pm GMT

Conditions: Advanced Solid Tumors; Metastatic Pancreatic Cancer; Pediatric Solid Tumor; Advanced Breast Cancer; Malignant Peripheral Nerve Sheath Tumor
Intervention: Drug: Metarrestin
Sponsor: National Cancer Institute (NCI)
Recruiting

Development of a Predictive Model for Early Differential Diagnosis of Uterine Leiomyomas and Leiomyosarcomas

Posted: January 2nd, 2020, 3:00pm GMT

Conditions: Leiomyoma; Leiomyosarcoma
Intervention: Procedure: Biopsy and peripheral blood collection
Sponsor: Igenomix
Recruiting

Permalink for 'Ivosidenib in Treating Patients With Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With IDH1 Mutations (A Pediatric MATCH Treatment Trial)'

Ivosidenib in Treating Patients With Advanced Solid Tumors, Lymphoma, or Histiocytic Disorders With IDH1 Mutations (A Pediatric MATCH Treatment Trial)

Posted: December 12th, 2019, 3:00pm GMT

Conditions: Recurrent Ependymoma; Recurrent Ewing Sarcoma; Recurrent Hepatoblastoma; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Malignant Solid Neoplasm; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Rhabdoid Tumor; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Recurrent WHO Grade II Glioma; Refractory Ependymoma; Refractory Ewing Sarcoma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Malignant Solid Neoplasm; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma; Refractory WHO Grade II Glioma; Wilms Tumor
Intervention: Drug: Ivosidenib
Sponsors: National Cancer Institute (NCI); Children's Oncology Group
Recruiting

Sotorasib Activity in Subjects With Advanced Solid Tumors With KRAS p.G12C Mutation (CodeBreak 101)

Posted: December 4th, 2019, 3:00pm GMT

Conditions: Advanced Solid Tumors; Kirsten Rat Sarcoma (KRAS) pG12C Mutation
Interventions: Drug: Sotorasib; Drug: AMG 404; Drug: trametinib; Drug: RMC-4630; Drug: afatinib; Drug: pembrolizumab; Drug: panitumumab; Drug: carboplatin, pemetrexed, docetaxel; Drug: atezolizumab; Drug: everolimus; Drug: palbociclib; Drug: MVASI® (bevacizumab-awwb); Drug: TNO155; Drug: FOLFIRI; Drug: FOLFOX
Sponsor: Amgen
Recruiting

$Permalink for 'A Study to Compare the Efficacy and Safety of Ifosfamide and Etoposide With or Without Lenvatinib in Children, Adolescents and Young Adults With Relapsed and Refractory Osteosarcoma'$

A Study to Compare the Efficacy and Safety of Ifosfamide and Etoposide With or Without Lenvatinib in Children, Adolescents and Young Adults With Relapsed and Refractory Osteosarcoma

Posted: November 6th, 2019, 3:00pm GMT

Condition: Osteosarcoma
Interventions: Drug: Lenvatinib; Drug: Ifosfamide; Drug: Etoposide
Sponsors: Eisai Inc.; Merck Sharp & Dohme Corp.
Recruiting

A Pilot Study to Explore the Role of Gut Flora in Colorectal Cancer

Posted: November 1st, 2019, 2:00pm GMT

Conditions: Colorectal Cancer; Colorectal Neoplasms; Colorectal Carcinoma; Colorectal Adenocarcinoma; Colorectal Cancer Metastatic; Colorectal Sarcoma
Intervention: Other: No intervention
Sponsor: ProgenaBiome
Recruiting

Permalink for 'Study to Learn More About the Safety and Effectiveness of the Drug VITRAKVI During Routine Use in Patients With TRK Fusion Cancer Which is Locally Advanced or Spread From the Place Where it Started to Other Places in the Body'

Study to Learn More About the Safety and Effectiveness of the Drug VITRAKVI During Routine Use in Patients With TRK Fusion Cancer Which is Locally Advanced or Spread From the Place Where it Started to Other Places in the Body

Posted: October 29th, 2019, 2:00pm GMT

Condition: Locally Advanced or Metastatic Solid Tumor Harboring an NTRK Gene Fusion
Intervention: Drug: larotrectinib(Vitrakvi, BAY2757556)
Sponsor: Bayer
Recruiting

$Permalink for 'Cobimetinib in Refractory Langerhans Cell Histiocytosis (LCH), and Other Histiocytic Disorders'$

Cobimetinib in Refractory Langerhans Cell Histiocytosis (LCH), and Other Histiocytic Disorders

Posted: September 6th, 2019, 2:00pm GMT

Conditions: Langerhan's Cell Histiocytosis; Juvenile Xanthogranuloma; Erdheim-Chester Disease; Rosai Dorfman Disease; Neuro-Degenerative Disease; Histiocytic Sarcoma; Histiocytic Disorders, Malignant
Intervention: Drug: Cobimetinib
Sponsors: Carl Allen; Baylor College of Medicine; North American Consortium for Histiocytosis; Genentech, Inc.
Recruiting

Spearhead 1 Study in Subjects With Advanced Synovial Sarcoma or Myxoid/Round Cell Liposarcoma

Posted: August 5th, 2019, 2:00pm GMT

Conditions: Synovial Sarcoma; Myxoid Liposarcoma
Intervention: Genetic: afamitresgene autoleucel (previously ADP-A2M4)
Sponsor: Adaptimmune
Recruiting

Olaratumab (LY3012207) Patient Access for Soft Tissue Sarcoma

Posted: June 21st, 2019, 2:00pm GMT

Condition: Soft Tissue Sarcoma
Intervention: Drug: Olaratumab
Sponsor: Eli Lilly and Company
Available

A Study of Tivozanib in Combination With Durvalumab in Subjects With Advanced Hepatocellular Carcinoma

Posted: May 31st, 2019, 2:00pm GMT

Condition: Hepatocellular Carcinoma
Interventions: Drug: Tivozanib; Drug: Durvalumab
Sponsors: AVEO Pharmaceuticals, Inc.; AstraZeneca
Recruiting

Permalink for 'A Study in Patients With Different Types of Advanced Cancer (Solid Tumors) to Test Different Doses of BI 907828 in Combination With BI 754091 (Ezabenlimab) and 754111 or BI 907828 in Combination With BI 754091 (Ezabenlimab)'

A Study in Patients With Different Types of Advanced Cancer (Solid Tumors) to Test Different Doses of BI 907828 in Combination With BI 754091 (Ezabenlimab) and 754111 or BI 907828 in Combination With BI 754091 (Ezabenlimab)

Posted: May 28th, 2019, 2:00pm GMT

Condition: Neoplasms
Interventions: Drug: BI 907828; Drug: BI 754091; Drug: BI 754111
Sponsor: Boehringer Ingelheim
Recruiting

Study of Folfiri/Cetuximab in FcGammaRIIIa V/V Stage IV Colorectal Cancer Patients

Posted: March 14th, 2019, 2:00pm GMT

Conditions: Colorectal Cancer; ADCC
Intervention: Drug: Folfiri/Cetuximab
Sponsor: National Cancer Institute, Naples
Recruiting

Rituximab Hyaluronidase in Combination With Chemotherapy in Treating Aggressive B-cell Lymphoma in Uganda

Posted: March 6th, 2019, 3:00pm GMT

Conditions: Burkitt Lymphoma; KSHV-associated Multicentric Castleman Disease; Diffuse Large B-Cell Lymphoma
Interventions: Biological: Rituximab and Hyaluronidase Human; Drug: Cyclophosphamide; Drug: Vincristine; Drug: Methotrexate; Drug: Doxorubicin; Drug: Doxorubicin Hydrochloride; Drug: Prednisone; Drug: Etoposide; Biological: Rituximab
Sponsor: Fred Hutchinson Cancer Research Center
Recruiting

PROactive Evaluation of Function to Avoid CardioToxicity

Posted: March 5th, 2019, 3:00pm GMT

Conditions: Cardiotoxicity; Breast Cancer; Lymphoma; Sarcoma; Leukemia; Myeloma; Lung Cancer
Intervention: Device: MyoStrain®
Sponsors: Washington University School of Medicine; Myocardial Solutions
Recruiting

A Study of APG-115 in Combination With Pembrolizumab in Patients With Metastatic Melanomas or Advanced Solid Tumors

Posted: August 2nd, 2018, 2:00pm GMT

Conditions: Unresectable or Metastatic Melanoma or Advanced Solid Tumors; Melanoma; Uveal Melanoma; Non Small Cell Lung Cancer; ATM Gene Mutation; P53 Mutation; MDM2 Gene Mutation; Liposarcoma; Urothelial Carcinoma; MPNST; Cutaneous Melanoma; Mucosal Melanoma; Malignant Peripheral Nerve Sheath Tumors; STK11 Gene Mutation
Intervention: Drug: APG-115+Pembrolizumab
Sponsors: Ascentage Pharma Group Inc.; Merck Sharp & Dohme Corp.
Recruiting

$Permalink for 'Palbociclib in Treating Patients With Relapsed or Refractory Rb Positive Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating Alterations in Cell Cycle Genes (A Pediatric MATCH Treatment Trial)'$

Palbociclib in Treating Patients With Relapsed or Refractory Rb Positive Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating Alterations in Cell Cycle Genes (A Pediatric MATCH Treatment Trial)

Posted: May 16th, 2018, 2:00pm GMT

Conditions: Advanced Malignant Solid Neoplasm; Recurrent Childhood Ependymoma; Recurrent Ewing Sarcoma; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Kidney Wilms Tumor; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Rhabdoid Tumor; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Refractory Ependymoma; Refractory Ewing Sarcoma; Refractory Glioma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma
Interventions: Other: Laboratory Biomarker Analysis; Drug: Palbociclib; Other: Pharmacological Study
Sponsor: National Cancer Institute (NCI)
Recruiting

Safety, Tolerability, Efficacy and Pharmacokinetics of Copanlisib in Pediatric Patients

Posted: March 8th, 2018, 3:00pm GMT

Condition: Mixed Tumor, Malignant
Intervention: Drug: BAY806946
Sponsor: Bayer
Recruiting

Pediatric PET/MR Image Registry

Posted: March 8th, 2018, 3:00pm GMT

Conditions: Soft Tissue; Lymphoma; Osteosarcoma
Interventions: Diagnostic Test: PET/MR imaging; Diagnostic Test: MR imaging; Diagnostic Test: PET/CT imaging
Sponsor: Stanford University
Recruiting

Permalink for 'A Study of Atezolizumab (Anti-Pd-L1 Antibody) as Adjuvant Therapy After Definitive Local Therapy in Patients With High-Risk Locally Advanced Squamous Cell Carcinoma of the Head and Neck'

A Study of Atezolizumab (Anti-Pd-L1 Antibody) as Adjuvant Therapy After Definitive Local Therapy in Patients With High-Risk Locally Advanced Squamous Cell Carcinoma of the Head and Neck

Posted: March 2nd, 2018, 3:00pm GMT

Condition: Locally Advanced Squamous Cell Carcinoma of the Head and Neck (SCCHN)
Interventions: Drug: Atezolizumab; Drug: Placebo
Sponsor: Hoffmann-La Roche
Recruiting

Phase 2 CAB-AXL-ADC Safety and Efficacy Study in Adult and Adolescent Patients With Sarcoma

Posted: February 7th, 2018, 3:00pm GMT

Conditions: Solid Tumor; Non Small Cell Lung Cancer; Melanoma; Sarcoma; Sarcoma, Ewing; Osteosarcoma; Leiomyosarcoma; Synovial Sarcoma; Liposarcoma; Soft Tissue Sarcoma; Bone Sarcoma; Refractory Sarcoma
Interventions: Biological: CAB-AXL-ADC; Biological: PD-1 inhibitor
Sponsor: BioAtla, Inc.
Recruiting

Project HERO: Health Empowerment & Recovery Outcomes

Posted: November 17th, 2017, 3:00pm GMT

Conditions: Prostate Cancer; Bladder Cancer; Kidney Cancer; Colon Cancer; Rectum Cancer; Thyroid Cancer; Lung Cancer; Small Intestine Cancer; Soft Tissue Cancer
Interventions: Behavioral: Body Mind Training (BMT); Behavioral: Body Training (BT):; Behavioral: Usual Care (UC)
Sponsors: Rutgers, The State University of New Jersey; University of California, Los Angeles; University of New Mexico
Recruiting

Permalink for 'Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease'

Molecular Characterization of Viral-associated Tumors, Tumors Occurring in the Setting of HIV or Other Immune Disorders and Castleman Disease

Posted: October 4th, 2017, 2:00pm GMT

Conditions: Human Immunodeficiency Virus; Castleman's Disease; Kaposi's Sarcoma; Viral-Associated Cancer
Intervention:
Sponsor: National Cancer Institute (NCI)
Recruiting

The Impact of Cancer on the Physical and Psychosocial Well-being Among Childhood Osteosarcoma Survivors

Posted: October 2nd, 2017, 2:00pm GMT

Condition: Childhood Osteosarcoma
Intervention: Other: Questionnaires
Sponsor: The University of Hong Kong
Recruiting

$Permalink for 'Olaparib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Defects in DNA Damage Repair Genes (A Pediatric MATCH Treatment Trial)'$

Olaparib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Defects in DNA Damage Repair Genes (A Pediatric MATCH Treatment Trial)

Posted: July 28th, 2017, 2:00pm GMT

Conditions: Advanced Malignant Solid Neoplasm; Ann Arbor Stage III Childhood Non-Hodgkin Lymphoma; Ann Arbor Stage IV Childhood Non-Hodgkin Lymphoma; Low Grade Glioma; Malignant Glioma; Recurrent Childhood Central Nervous System Neoplasm; Recurrent Childhood Ependymoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Non-Hodgkin Lymphoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Solid Neoplasm; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Osteosarcoma; Refractory Childhood Malignant Germ Cell Tumor; Refractory Ependymoma; Refractory Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Refractory Glioma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Glioma; Refractory Malignant Solid Neoplasm; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Primary Central Nervous System Neoplasm; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma; Rhabdoid Tumor; Wilms Tumor
Intervention: Drug: Olaparib
Sponsor: National Cancer Institute (NCI)
Recruiting

Biology of Osteosarcoma (BOOST) Registry and Biobank

Posted: July 21st, 2017, 2:00pm GMT

Condition: Osteosarcoma
Intervention:
Sponsor: University of Minnesota
Recruiting

$Permalink for 'Vemurafenib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With BRAF V600 Mutations (A Pediatric MATCH Treatment Trial)'$

Vemurafenib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With BRAF V600 Mutations (A Pediatric MATCH Treatment Trial)

Posted: July 18th, 2017, 2:00pm GMT

Conditions: Advanced Malignant Solid Neoplasm; Ann Arbor Stage III Childhood Non-Hodgkin Lymphoma; Ann Arbor Stage IV Childhood Non-Hodgkin Lymphoma; Ependymoma; Ewing Sarcoma; Hepatoblastoma; Langerhans Cell Histiocytosis; Malignant Germ Cell Tumor; Malignant Glioma; Osteosarcoma; Peripheral Primitive Neuroectodermal Tumor; Recurrent Childhood Central Nervous System Neoplasm; Recurrent Childhood Non-Hodgkin Lymphoma; Recurrent Malignant Solid Neoplasm; Recurrent Neuroblastoma; Refractory Malignant Solid Neoplasm; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Primary Central Nervous System Neoplasm; Rhabdoid Tumor; Rhabdomyosarcoma; Soft Tissue Sarcoma; Wilms Tumor
Interventions: Other: Laboratory Biomarker Analysis; Drug: Vemurafenib
Sponsor: National Cancer Institute (NCI)
Recruiting

$Permalink for 'Samotolisib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With TSC or PI3K/MTOR Mutations (A Pediatric MATCH Treatment Trial)'$

Samotolisib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With TSC or PI3K/MTOR Mutations (A Pediatric MATCH Treatment Trial)

Posted: July 11th, 2017, 2:00pm GMT

Conditions: Advanced Malignant Solid Neoplasm; Ann Arbor Stage III Non-Hodgkin Lymphoma; Ann Arbor Stage IV Non-Hodgkin Lymphoma; Malignant Glioma; Recurrent Ependymoma; Recurrent Ewing Sarcoma; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Solid Neoplasm; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Primary Central Nervous System Neoplasm; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Solid Neoplasm; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Primary Central Nervous System Neoplasm; Rhabdoid Tumor; Stage III Osteosarcoma AJCC v7; Stage III Soft Tissue Sarcoma AJCC v7; Stage IV Osteosarcoma AJCC v7; Stage IV Soft Tissue Sarcoma AJCC v7; Stage IVA Osteosarcoma AJCC v7; Stage IVB Osteosarcoma AJCC v7; Wilms Tumor
Interventions: Other: Laboratory Biomarker Analysis; Other: Pharmacological Study; Drug: Samotolisib
Sponsor: National Cancer Institute (NCI)
Recruiting

$Permalink for 'Ensartinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With ALK or ROS1 Genomic Alterations (A Pediatric MATCH Treatment Trial)'$

Ensartinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With ALK or ROS1 Genomic Alterations (A Pediatric MATCH Treatment Trial)

Posted: July 11th, 2017, 2:00pm GMT

Conditions: Hematopoietic and Lymphoid Cell Neoplasm; Malignant Solid Neoplasm; Recurrent Ependymoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Hepatoblastoma; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Recurrent WHO Grade II Glioma; Refractory Ependymoma; Refractory Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma; Wilms Tumor
Interventions: Drug: Ensartinib; Other: Laboratory Biomarker Analysis; Other: Pharmacological Study
Sponsors: National Cancer Institute (NCI); Children's Oncology Group
Recruiting

$Permalink for 'Larotrectinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With NTRK Fusions (A Pediatric MATCH Treatment Trial)'$

Larotrectinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With NTRK Fusions (A Pediatric MATCH Treatment Trial)

Posted: July 11th, 2017, 2:00pm GMT

Conditions: Advanced Malignant Solid Neoplasm; Recurrent Ependymoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Malignant Solid Neoplasm; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Rhabdoid Tumor; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Refractory Ependymoma; Refractory Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Refractory Glioma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Malignant Solid Neoplasm; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Primary Central Nervous System Neoplasm; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Wilms Tumor
Interventions: Drug: Larotrectinib; Drug: Larotrectinib Sulfate
Sponsor: National Cancer Institute (NCI)
Recruiting

$Permalink for 'Erdafitinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With FGFR Mutations (A Pediatric MATCH Treatment Trial)'$

Erdafitinib in Treating Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With FGFR Mutations (A Pediatric MATCH Treatment Trial)

Posted: July 7th, 2017, 2:00pm GMT

Conditions: Advanced Malignant Solid Neoplasm; Ann Arbor Stage III Childhood Non-Hodgkin Lymphoma; Ann Arbor Stage IV Childhood Non-Hodgkin Lymphoma; Low Grade Glioma; Malignant Glioma; Recurrent Childhood Ependymoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Non-Hodgkin Lymphoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Hepatoblastoma; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Solid Neoplasm; Recurrent Neuroblastoma; Recurrent Osteosarcoma; Recurrent Primary Central Nervous System Neoplasm; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Solid Neoplasm; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Primary Central Nervous System Neoplasm; Rhabdoid Tumor; Stage III Soft Tissue Sarcoma AJCC v7; Stage IV Soft Tissue Sarcoma AJCC v7; Wilms Tumor
Interventions: Drug: Erdafitinib; Other: Laboratory Biomarker Analysis; Other: Pharmacological Study
Sponsor: National Cancer Institute (NCI)
Recruiting

$Permalink for 'Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial)'$

Targeted Therapy Directed by Genetic Testing in Treating Pediatric Patients With Relapsed or Refractory Advanced Solid Tumors, Non-Hodgkin Lymphomas, or Histiocytic Disorders (The Pediatric MATCH Screening Trial)

Posted: May 16th, 2017, 2:00pm GMT

Conditions: Advanced Malignant Solid Neoplasm; Ann Arbor Stage III Non-Hodgkin Lymphoma; Ann Arbor Stage IV Non-Hodgkin Lymphoma; Histiocytic Sarcoma; Juvenile Xanthogranuloma; Langerhans Cell Histiocytosis; Malignant Glioma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Ependymoma; Recurrent Ewing Sarcoma; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Solid Neoplasm; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Primary Central Nervous System Neoplasm; Recurrent Rhabdoid Tumor; Recurrent Soft Tissue Sarcoma; Refractory Ewing Sarcoma; Refractory Glioma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Solid Neoplasm; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Primary Central Nervous System Neoplasm; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Rhabdoid Tumor; Stage III Osteosarcoma AJCC v7; Stage III Soft Tissue Sarcoma AJCC v7; Stage IV Osteosarcoma AJCC v7; Stage IV Soft Tissue Sarcoma AJCC v7; Stage IVA Osteosarcoma AJCC v7; Stage IVB Osteosarcoma AJCC v7; Wilms Tumor
Interventions: Procedure: Biopsy; Procedure: Biospecimen Collection; Drug: Ensartinib; Drug: Erdafitinib; Other: Laboratory Biomarker Analysis; Drug: Larotrectinib; Procedure: Mutation Carrier Screening; Drug: Olaparib; Drug: Palbociclib; Other: Pharmacological Study; Drug: Samotolisib; Drug: Selpercatinib; Drug: Selumetinib Sulfate; Drug: Tazemetostat; Drug: Tipifarnib; Drug: Ulixertinib; Drug: Vemurafenib
Sponsor: National Cancer Institute (NCI)
Recruiting

$Permalink for 'Nab-Paclitaxel and Gemcitabine for Recurrent/Refractory Sarcoma'$

Nab-Paclitaxel and Gemcitabine for Recurrent/Refractory Sarcoma

Posted: October 26th, 2016, 2:00pm GMT

Conditions: Osteosarcoma; Ewing Sarcoma; Rhabdomyosarcoma; Soft Tissue Sarcoma
Interventions: Drug: nab-Paclitaxel; Drug: Gemcitabine
Sponsors: H. Lee Moffitt Cancer Center and Research Institute; National Pediatric Cancer Foundation
Recruiting

Permalink for 'Continuous 24h Intravenous Infusion of Mithramycin, an Inhibitor of Cancer Stem Cell Signaling, in People With Primary Thoracic Malignancies or Carcinomas, Sarcomas or Germ Cell Neoplasms With Pleuropulmonary Metastases'

Continuous 24h Intravenous Infusion of Mithramycin, an Inhibitor of Cancer Stem Cell Signaling, in People With Primary Thoracic Malignancies or Carcinomas, Sarcomas or Germ Cell Neoplasms With Pleuropulmonary Metastases

Posted: August 9th, 2016, 2:00pm GMT

Conditions: Esophageal Neoplasms; Lung Neoplasms; Mesothelioma; Thymus Neoplasms; Neoplasms, Germ Cell and Embryonal
Intervention: Drug: Mithramycin
Sponsor: National Cancer Institute (NCI)
Recruiting

Randomized Carbon Ions vs Standard Radiotherapy for Radioresistant Tumors

Posted: July 20th, 2016, 2:00pm GMT

Condition: Malignant Tumors as Chordoma, Adenoid Cystic Carcinoma and Sarcoma
Interventions: Radiation: Carbon ions therapy; Radiation: Advanced external radiotherapy by Xrays or protons
Sponsor: Hospices Civils de Lyon
Recruiting

Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

Posted: July 15th, 2016, 2:00pm GMT

Conditions: Acinar Cell Carcinoma; Adenoid Cystic Carcinoma; Adrenal Cortex Carcinoma; Adrenal Gland Pheochromocytoma; Anal Canal Neuroendocrine Carcinoma; Anal Canal Undifferentiated Carcinoma; Angiosarcoma; Apocrine Neoplasm; Appendix Mucinous Adenocarcinoma; Bartholin Gland Transitional Cell Carcinoma; Basal Cell Carcinoma; Bladder Adenocarcinoma; Breast Metaplastic Carcinoma; Cervical Adenocarcinoma; Cholangiocarcinoma; Chordoma; Colorectal Squamous Cell Carcinoma; Desmoid Fibromatosis; Endometrial Transitional Cell Carcinoma; Endometrioid Adenocarcinoma; Esophageal Neuroendocrine Carcinoma; Esophageal Undifferentiated Carcinoma; Extrahepatic Bile Duct Carcinoma; Extramammary Paget Disease; Fallopian Tube Adenocarcinoma; Fallopian Tube Transitional Cell Carcinoma; Fibromyxoid Tumor; Gallbladder Carcinoma; Gastric Neuroendocrine Carcinoma; Gastric Squamous Cell Carcinoma; Gastric Undifferentiated Carcinoma; Gastrointestinal Stromal Tumor; Gestational Trophoblastic Tumor; Giant Cell Carcinoma; Human Papillomavirus-Independent Cervical Adenocarcinoma, Clear Cell-Type; Intestinal Neuroendocrine Carcinoma; Intrahepatic Cholangiocarcinoma; Lung Carcinoid Tumor; Lung Sarcomatoid Carcinoma; Major Salivary Gland Carcinoma; Malignant Odontogenic Neoplasm; Malignant Peripheral Nerve Sheath Tumor; Malignant Solid Neoplasm; Malignant Testicular Sex Cord-Stromal Tumor; Metastatic Malignant Neoplasm of Unknown Primary; Minimally Invasive Lung Adenocarcinoma; Mixed Mesodermal (Mullerian) Tumor; Mucinous Adenocarcinoma; Mucinous Cystadenocarcinoma; Nasal Cavity Adenocarcinoma; Nasal Cavity Carcinoma; Nasopharyngeal Carcinoma; Nasopharyngeal Papillary Adenocarcinoma; Nasopharyngeal Undifferentiated Carcinoma; Oral Cavity Carcinoma; Oropharyngeal Undifferentiated Carcinoma; Ovarian Adenocarcinoma; Ovarian Germ Cell Tumor; Ovarian Mucinous Adenocarcinoma; Ovarian Squamous Cell Carcinoma; Ovarian Transitional Cell Carcinoma; Pancreatic Acinar Cell Carcinoma; Pancreatic Neuroendocrine Carcinoma; Paraganglioma; Paranasal Sinus Adenocarcinoma; Paranasal Sinus Carcinoma; Parathyroid Gland Carcinoma; PEComa; Peritoneal Mesothelioma; Pituitary Gland Carcinoma; Placental Choriocarcinoma; Primary Peritoneal High Grade Serous Adenocarcinoma; Pseudomyxoma Peritonei; Rare Disorder; Scrotal Squamous Cell Carcinoma; Seminal Vesicle Adenocarcinoma; Seminoma; Serous Cystadenocarcinoma; Small Intestinal Adenocarcinoma; Small Intestinal Squamous Cell Carcinoma; Spindle Cell Neoplasm; Squamous Cell Carcinoma of the Penis; Teratoma With Somatic-Type Malignancy; Testicular Non-Seminomatous Germ Cell Tumor; Thyroid Gland Carcinoma; Tracheal Carcinoma; Transitional Cell Carcinoma; Ureter Adenocarcinoma; Ureter Squamous Cell Carcinoma; Urethral Adenocarcinoma; Urethral Squamous Cell Carcinoma; Vaginal Adenocarcinoma; Vaginal Squamous Cell Carcinoma, Not Otherwise Specified; Vulvar Carcinoma
Interventions: Procedure: Biospecimen Collection; Biological: Ipilimumab; Biological: Nivolumab
Sponsor: National Cancer Institute (NCI)
Recruiting

PLX3397 Plus Sirolimus in Unresectable Sarcoma and Malignant Peripheral Nerve Sheath Tumors

Posted: October 22nd, 2015, 2:00pm GMT

Conditions: Sarcoma; Malignant Peripheral Nerve Sheath Tumors
Interventions: Drug: PLX3397; Drug: Sirolimus
Sponsors: Gulam Manji; Daiichi Sankyo, Inc.
Recruiting

Permalink for 'Basket Study of Entrectinib (RXDX-101) for the Treatment of Patients With Solid Tumors Harboring NTRK 1/2/3 (Trk A/B/C), ROS1, or ALK Gene Rearrangements (Fusions)'

Basket Study of Entrectinib (RXDX-101) for the Treatment of Patients With Solid Tumors Harboring NTRK 1/2/3 (Trk A/B/C), ROS1, or ALK Gene Rearrangements (Fusions)

Posted: October 5th, 2015, 2:00pm GMT

Conditions: Breast Cancer; Cholangiocarcinoma; Colorectal Cancer; Head and Neck Neoplasms; Lymphoma, Large-Cell, Anaplastic; Melanoma; Neuroendocrine Tumors; Non-Small Cell Lung Cancer; Ovarian Cancer; Pancreatic Cancer; Papillary Thyroid Cancer; Primary Brain Tumors; Renal Cell Carcinoma; Sarcomas; Salivary Gland Cancers; Adult Solid Tumor
Intervention: Drug: Entrectinib
Sponsor: Hoffmann-La Roche
Recruiting

Combination Chemotherapy With or Without Temsirolimus in Treating Patients With Intermediate Risk Rhabdomyosarcoma

Posted: October 5th, 2015, 2:00pm GMT

Conditions: Alveolar Rhabdomyosarcoma; Botryoid-Type Embryonal Rhabdomyosarcoma; Embryonal Rhabdomyosarcoma; Rhabdomyosarcoma; Sclerosing Rhabdomyosarcoma; Spindle Cell Rhabdomyosarcoma
Interventions: Drug: Cyclophosphamide; Biological: Dactinomycin; Drug: Irinotecan Hydrochloride; Other: Laboratory Biomarker Analysis; Other: Questionnaire Administration; Radiation: Radiation Therapy; Drug: Temsirolimus; Drug: Vincristine Sulfate; Drug: Vinorelbine
Sponsor: National Cancer Institute (NCI)
Recruiting

Nab-Paclitaxel and Bevacizumab in Treating Patients With Unresectable Stage IV Melanoma or Gynecological Cancers

Posted: December 25th, 2013, 3:00pm GMT

Conditions: Cervical Adenocarcinoma; Cervical Adenosarcoma; Cervical Adenosquamous Carcinoma; Cervical Carcinosarcoma; Cervical Squamous Cell Carcinoma, Not Otherwise Specified; Endometrial Adenosquamous Carcinoma; Endometrial Clear Cell Adenocarcinoma; Endometrial Endometrioid Adenocarcinoma; Endometrial Mixed Cell Adenocarcinoma; Endometrial Mucinous Adenocarcinoma; Endometrial Serous Adenocarcinoma; Endometrial Undifferentiated Carcinoma; Fallopian Tube Adenocarcinoma; Fallopian Tube Carcinosarcoma; Fallopian Tube Clear Cell Adenocarcinoma; Fallopian Tube Endometrioid Adenocarcinoma; Fallopian Tube Mucinous Adenocarcinoma; Fallopian Tube Serous Adenocarcinoma; Fallopian Tube Squamous Cell Carcinoma; Fallopian Tube Transitional Cell Carcinoma; Fallopian Tube Undifferentiated Carcinoma; High Grade Ovarian Serous Adenocarcinoma; Malignant Female Reproductive System Neoplasm; Malignant Ovarian Clear Cell Tumor; Malignant Ovarian Endometrioid Tumor; Malignant Ovarian Epithelial Tumor; Malignant Ovarian Mucinous Tumor; Malignant Peritoneal Neoplasm; Malignant Solid Neoplasm; Ovarian Carcinosarcoma; Ovarian Clear Cell Adenocarcinoma; Ovarian Endometrioid Adenocarcinoma; Ovarian Mucinous Adenocarcinoma; Ovarian Serous Adenocarcinoma; Ovarian Transitional Cell Carcinoma; Ovarian Undifferentiated Carcinoma; Platinum-Resistant Fallopian Tube Carcinoma; Platinum-Resistant Ovarian Carcinoma; Platinum-Resistant Primary Peritoneal Carcinoma; Platinum-Sensitive Ovarian Carcinoma; Primary Peritoneal Carcinosarcoma; Primary Peritoneal Clear Cell Adenocarcinoma; Primary Peritoneal Serous Adenocarcinoma; Primary Peritoneal Transitional Cell Carcinoma; Primary Peritoneal Undifferentiated Carcinoma; Stage IV Cutaneous Melanoma AJCC v6 and v7; Unresectable Melanoma; Uterine Corpus Carcinosarcoma
Interventions: Biological: Bevacizumab; Other: Laboratory Biomarker Analysis; Drug: Nab-paclitaxel; Other: Pharmacological Study
Sponsors: Mayo Clinic; National Cancer Institute (NCI)
Recruiting

Comprehensive Omics Analysis of Pediatric Solid Tumors and Establishment of a Repository for Related Biological Studies

Posted: April 23rd, 2010, 2:00pm GMT

Conditions: Sarcoma; Endocrine Tumors; Neuroblastoma; Retinoblastoma; Renal Cancer
Intervention:
Sponsor: National Cancer Institute (NCI)
Recruiting

Long-Term Follow-Up of Patients Who Have Participated in Children's Oncology Group Studies

Posted: August 18th, 2008, 2:00pm GMT

Conditions: Acute Lymphoblastic Leukemia; Brain Neoplasm; Hematopoietic Cell Transplantation Recipient; Hodgkin Lymphoma; Osteosarcoma; Rhabdomyosarcoma
Interventions: Procedure: Assessment of Therapy Complications; Other: Questionnaire Administration
Sponsors: Children's Oncology Group; National Cancer Institute (NCI)
Recruiting

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Rare Cancer News & Clinical Trials » Sarcoma

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Sarcoma (292 unread)

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