Rare Cancer News & Clinical Trials

Sarcoma (218 unread)

Permalink for 'HPV51-associated Leiomyosarcoma: A Novel Class of TP53/RB1-Wildtype Tumor With Predilection for the Female Lower Reproductive Tract'

HPV51-associated Leiomyosarcoma: A Novel Class of TP53/RB1-Wildtype Tumor With Predilection for the Female Lower Reproductive Tract

Fetched: January 17th, 2022, 5:02am GMT by Erik A Williams

Am J Surg Pathol. 2022 Jan 17. doi: 10.1097/PAS.0000000000001862. Online ahead of print.

ABSTRACT

Inactivating mutations in tumor suppressor genes TP53 and RB1 are considered central drivers in leiomyosarcomas (LMSs). In high-risk human papillomavirus (HPV)-related tumors, a similar functional outcome is achieved through oncoproteins E6 and E7, which inactivate the p53 and RB1 proteins, respectively. Here, we hypothesized that HPV infection could provide an alternative mechanism for tumorigenesis in a subset of TP53/RB1-wildtype LMS. We evaluated tumor samples from 2585 consecutive unique patients carrying a diagnosis of gynecologic or soft tissue LMS. Tumor DNA and available RNA were analyzed by hybrid-capture-based next-generation sequencing/comprehensive genomic profiling of 406 genes and transcripts (FoundationOneHeme). Of the initial 2585 cases, we excluded 16 based on the presence of molecular alterations that are considered defining for sarcomas other than LMS. In the remaining 2569 cases, we searched for LMS that were TP53/RB1-wildtype (n=486 of 2569; 18.9%). We also searched LMS tumors for HPV sequences that we then classified into genotypes by de novo assembly of nonhuman sequencing reads followed by alignment to the RefSeq database. Among TP53/RB1-wildtype LMS, we identified 18 unique cases harboring HPV sequences. Surprisingly, most (n=11) were HPV51-positive, and these 11 represented all HPV51-positive tumors in our entire LMS database (n=11 of 2569; 0.4%). The absence of genomic alterations in TP53 or RB1 in HPV51-positive LMS represented a marked difference from HPV51-negative LMS (n=2558; 0% vs. 72% [P<0.00001], 0% vs. 53% [P=0.0002]). In addition, compared with HPV51-negative LMS, HPV51-positive LMS were significantly enriched for genomic alterations in ATRX (55% vs. 24%, P=0.027) and TSC1 (18% vs. 0.6%, P=0.0047). All HPV51-positive LMS were in women; median age was 54 years at surgery (range: 23 to 74 y). All known primary sites were from the gynecologic tract or adjacent anogenital area, including 5 cases of vaginal primary site. Histology was heterogeneous, with evaluable cases showing predominant epithelioid (n=5) and spindle (n=5) morphology. In situ hybridization confirmed the presence of high-risk HPV E6/E7 mRNA in tumor cells in three of three evaluable cases harboring HPV51 genomic sequences. Overall, in our pan-LMS analysis, HPV reads were identified in a subset of TP53/RB1-wildtype LMS. For all HPV51-associated LMS, the striking absence of any detectable TP53 or RB1 mutations and predilection for the female lower reproductive tract supports our hypothesis that high-risk HPV can be an alternative tumorigenic mechanism in this distinct class of LMS.

PMID:35034043 | DOI:10.1097/PAS.0000000000001862

Bone Infarct Associated Osteosarcoma: Epidemiologic and Survival Trends

Fetched: January 17th, 2022, 5:02am GMT by Roberta Laranga

Oncol Res Treat. 2022 Jan 14. doi: 10.1159/000521986. Online ahead of print.

ABSTRACT

Background Bone tumors are not a frequent occurrence and bone-infarct-associated sarcomas are even rarer. The prognosis of this disease is poor and its treatment remains a challenge. Nevertheless, hardly any analyses in literature report on secondary osteosarcoma (SO) on bone infarct and most of the data available do not provide sufficient details. We evaluated whether this condition could be further characterized and if prognosis could be influenced by the chemotherapy treatment. In particular, we sought to determine (1) the main features of this rare disease; (2) the overall survival (OS) rate; (3) the OS rate associated to chemotherapy treatment; (4) the correspondence between our results and published data in terms of survival. Methods We retrospectively reviewed patients admitted at Rizzoli Orthopedic Institute of Bologna between 1992 and 2018 (1465 total cases of osteosarcoma). We identified a list of 11 cases of SO on bone infarct (cohort 1). We conferred about the epidemiology, surgical and chemotherapy (ChT) treatment and surveillance of infarct-associated osteosarcoma showing the correlation to data present in literature, corresponding to 15 case reports published within 1962-2018 (cohort 2). Results (1) cohort 1 was made of 11 patients: six females and five males, median age was 55 years. Nine (81%) were grade 4, two (19%) grade 3. Tumor predominantly arise on distal femur (64%). Most of patients had localized osteosarcoma at the diagnosis (81%); resection surgery was the elective treatment (73%) followed by amputation (18%). Of 11 patients, seven received also ChT (64%). (2) Five-years OS was 62% (95% CI: 28-84). Median OS was 74 months (95% CI: 12-not reached). The cumulative incidence of cancer-related deaths (CICRD) was 37.7% (95% CI: 11.4-64.5) at 120 months. (3) In the group treated with only surgery OS was 50% at 5 years. For patients treated with any form of ChT, OS was 71% at 5 years (p=0.4773) and hazard ratio (HR) 0.56. The CICRD was 29% (at 2 years of follow-up. Instead, it was of 50% for patients treated only with surgery. (4) Median survival was 74 months and 19 months for cohort 1 and cohort 2 respectively (p=0.09). Data analysis showed a decreased HR for cohort 1 compared to cohort 2 (HR 0.378). Results confirmed also stratifying for age and ChT administration (HR 0.355). Conclusions Based on this work, our opinion is that the choice to undergo the patients to ChT combined to surgery could improve their prognosis.

PMID:35034028 | DOI:10.1159/000521986

Permalink for 'Characteristics of TP53 germline variants and their correlation with Li-Fraumeni syndrome or Li-Fraumeni-like syndrome in Chinese tumor patients'

Characteristics of TP53 germline variants and their correlation with Li-Fraumeni syndrome or Li-Fraumeni-like syndrome in Chinese tumor patients

Fetched: January 17th, 2022, 5:02am GMT by Panwen Tian

J Genet Genomics. 2022 Jan 13:S1673-8527(22)00003-0. doi: 10.1016/j.jgg.2021.12.012. Online ahead of print.

ABSTRACT

Li-Fraumeni syndrome (LFS), a rare autosomal-dominant inheritance condition, is associated with a family cancer history as well as pathogenic/likely-pathogenic TP53 germline variants (P/LP TP53 GV). The current clinical methods for detecting LFS are limited. Here, we retrospectively investigate P/LP TP53 GV among Chinese cancer patients by next-generation sequencing and evaluate its relationship with a family cancer history. A total of 270 out of 19,226 cancer patients had TP53 GV, including 53 patients with P/LP TP53 GV. Patients with P/LP TP53 GV were mainly found in male with glioma, lung cancer or sarcoma. The median age of diagnosis for P/LP TP53 GV patients was significantly lower than that of non-P/LP TP53 GV patients (31-years vs. 53-years; P < 0.01). One LFS patient and three Li-Fraumeni-like syndrome (LFL) patients were among the 26 followed-up P/LP TP53 GV patients. Among 25 types of P/LP TP53 GV, the highest variant frequencies occurred at codon 175 and 248. p.M237I, p.R158H, p.C238Y and p.C275R, were firstly identified among the Chinese LFS/LFL patients. This is the first cohort report of (P/LP) TP53 GV characteristics of Chinese pan-cancer patients. These findings suggest analyzing the P/LP TP53 GV in cancer patients is an effective strategy for identifying cancer predisposition syndrome.

PMID:35033608 | DOI:10.1016/j.jgg.2021.12.012

Modern Therapy for Spinal and Paraspinal Ewing Sarcoma: An Update of the XXX Experience

Fetched: January 17th, 2022, 5:02am GMT by Daniel J Indelicato

Int J Radiat Oncol Biol Phys. 2022 Jan 13:S0360-3016(22)00017-7. doi: 10.1016/j.ijrobp.2022.01.007. Online ahead of print.

ABSTRACT

PURPOSE: In 2010, we published a comprehensive review of our institutional outcomes treating children with spinal and paraspinal Ewing sarcoma using photon therapy. Multimodality therapy was associated with fair disease control but also with serious toxicity, including a 37% rate of Grade ≥3 toxicity. We therefore sought to assess our more recent experience treating children with more modern technology and treatment regimens.

METHODS AND MATERIALS: Between 2010 and 2021, 32 pediatric patients with nonmetastatic spinal and paraspinal Ewing sarcoma were treated at XXX and enrolled in a retrospective outcome study. Median age at diagnosis was 9.8 (range, 2.1-21.8) years old. Three, 22, and 7 tumors arose within the cervical, thoracic, and lumbar spine regions, respectively. Median maximum tumor diameter was 5 (range, 3-19) cm. At diagnosis, 28/32 patients had motor, bowel, or bladder deficits. Chemotherapy was delivered per contemporary North American and European interval-compressed regimens. Before radiotherapy, 14 patients underwent gross total resection while 18 underwent a biopsy or subtotal resection with cord decompression. All patients were treated with proton therapy; 6 with hardware stabilization also received a component of intensity-modulated photon therapy. Median prescription dose was 50.4 (range, 45-54) GyRBE. Median maximum dose to the spinal cord was 50.2 (range, 0-54.9) GyRBE.

RESULTS: With a median follow-up of 4.1 (range, 0.7 - 9.4) years, the 5-year local control, progression-free survival, and overall survival rates were 92%, 79%, and 85%, respectively. Ten of 30 living patients have residual motor, bowel, or bladder deficits. Overall, 22% of patients experienced CTCAE grade 3 late toxicity related to multimodality treatment: kyphosis (n=4), esophagitis (n=2) and chronic kidney disease (n=1). No patients developed grade ≥4 toxicity, new neurologic deficits, or second malignancy.

CONCLUSION: Modern treatment advances may offer an improved therapeutic ratio for pediatric spinal and paraspinal Ewing sarcoma. With appropriate management, most patients can be cured with recovery of long-term neurologic function and modest side-effects.

PMID:35033584 | DOI:10.1016/j.ijrobp.2022.01.007

Preoperative Prediction of BRAF Mutation Status in Colorectal Cancer Using a Clinical-radiomics Model

Fetched: January 17th, 2022, 5:02am GMT by Ting Xue

Acad Radiol. 2022 Jan 12:S1076-6332(21)00579-1. doi: 10.1016/j.acra.2021.12.016. Online ahead of print.

ABSTRACT

RATIONALE AND OBJECTIVES: This study aimed to develop a clinically practical model to predict V-raf murine sarcoma viral oncogene homolog B1 (BRAF) mutation in colorectal cancer according to radiomic signatures based on computed tomography (CT) and clinical risk factors, and to determine the model's diagnostic accuracy for BRAF mutation status.

MATERIALS AND METHODS: This retrospective study included 140 patients with colorectal cancer. The significant clinical risk factors were used to build the clinical model; the least absolute shrinkage and selection operator algorithm was adopted to construct a radiomics signature according to imaging features of the tumor lesion, and stepwise logistic regression was applied to select the significant variables to develop the clinical-radiomics model. The predictive performance was evaluated by receiver operating characteristic curve analysis, calibration curve analysis, and decision curve analysis.

RESULTS: The radscore, generated by 5 selected radiomics features, demonstrated a favorable ability to predict BRAF mutation in both the training (area under the receiver operating characteristic curve [AUC] 0.93) and validation (AUC 0.87) cohorts. Subsequently, integrating two independent predictors (including the radscore and clinical risk factors) into a nomogram exhibited more favorable discriminatory performance, with the AUC improved to 0.95 and 0.88 in both cohorts. Moreover, the accuracy for predicting BRAF mutations was higher than that of the clinical model, ranging from 0.70 to 0.89.

CONCLUSION: The proposed CT-based radiomics signature is associated with BRAF mutations. The present study also proposes a combined model can potentially be applied in the individual preoperative prediction of BRAF mutation status in colorectal cancer.

ADVANCES IN KNOWLEDGE: CT-based radiomics showed satisfactory diagnostic significance for the BRAF status in colorectal cancer, the clinical-combined model may be applied in the individual preoperative prediction of BRAF mutation.

PMID:35033450 | DOI:10.1016/j.acra.2021.12.016

Permalink for '5-ALA-mediated fluorescence of musculoskeletal tumors in a chick chorio-allantoic membrane model: preclinical in vivo qualification analysis as a fluorescence-guided surgery agent in Orthopedic Oncology'

5-ALA-mediated fluorescence of musculoskeletal tumors in a chick chorio-allantoic membrane model: preclinical in vivo qualification analysis as a fluorescence-guided surgery agent in Orthopedic Oncology

Fetched: January 17th, 2022, 5:02am GMT by Wiebke K Guder

J Orthop Surg Res. 2022 Jan 15;17(1):34. doi: 10.1186/s13018-022-02931-x.

ABSTRACT

BACKGROUND: Fluorescence-guided surgery (FGS) with 5-aminolevulinic acid (5-ALA) and other contrast agents has shown its efficacy in improving resection margins, local recurrence and survival rates in several medical disciplines. It is the objective of this study to analyze the engraftment rate of musculoskeletal tumor specimens on the chick chorio-allantoic membrane (CAM), the rate of tumor fluorescence (PDD), and the effects of photodynamic therapy (PDT) after exposure of tumors to 5-ALA in an in vivo environment.

METHODS: A total of 486 CAMs were inoculated with macroscopic tumor grafts (n = 26; n = 478 eggs) and primary cell culture suspensions (n = 2; n = 8 eggs) from 26 patients on day 10 of egg development. On day 16, 2 mg/200 µl 5-ALA were topically applied per egg. After 4 h of incubation, Protoporphyrin IX was excited using blue light (420 ± 10 nm). Tumor fluorescence (PDD) was photo-documented. A subgroup of specimens was additionally exposed to red light (635 nm ± 10 nm; PDT). After the termination of the experiment, CAM-grown tumors were histopathologically analyzed.

RESULTS: Benign and borderline tumors (chondroblastoma, giant cell tumor of bone and atypical chondrogenic tumor) presented with high rates of detectable fluorescence. Comparable results were found for chondrosarcoma, osteosarcoma and Ewing's sarcoma among bone and dedifferentiated liposarcoma, myxofibrosarcoma and undifferentiated pleomorphic sarcoma among soft tissue sarcomas. Overall, tumor fluorescence was negative for 20.2%, single-positive (+) for 46.9% and double-positive (++) for 32.9% of macroscopic xenografts, and negative in 20% and (+) in 80% of primary cell culture tumors. Macroscopic tumor xenografts (n = 478) were identified as viable in 14.8%, partially viable in 2.9% and partially to completely regressive in 45.2%. All (n = 8) tumors grown from primary cell culture were viable. After PDT, tumor samples were found viable in 5.5%, partially viable in 5.5% and partially to completely regressive in 68%. Egg survival increased with decreasing PDT doses.

CONCLUSIONS: The CAM model proves to be a suitable in vivo model for the investigation of short-term observation questions in musculoskeletal tumors. The findings of this study warrant further investigation of PDT effects on musculoskeletal tumors and a possible incorporation of 5-ALA FGS in clinical Orthopedic Oncology care.

PMID:35033148 | DOI:10.1186/s13018-022-02931-x

Indication for Proximal Femoral Replacement is Associated with Risk of Failure

Fetched: January 16th, 2022, 5:01am GMT by Athan G Zavras

J Arthroplasty. 2022 Jan 12:S0883-5403(22)00014-6. doi: 10.1016/j.arth.2022.01.013. Online ahead of print.

ABSTRACT

BACKGROUND: Proximal femoral replacement (PFR) is reserved as a salvage procedure following failed total hip arthroplasty (THA) or after wide margin resection of tumors involving the proximal femur. Although failure of the PFR construct remains a significant problem, indication has not previously been investigated as a risk factor for failure.

METHODS: This study retrospectively evaluated patients who underwent PFR over a consecutive fifteen-year period for primary sarcoma or metastatic disease of the proximal femur, compared to conversion to PFR after failed THA. PFR failure was defined as recurrent prosthetic dislocations, periprosthetic fracture, aseptic loosening, or infection that ultimately resulted in revision surgery.

RESULTS: Overall, 99 patients were evaluated including 58 in the neoplasm and 41 in the failed THA cohorts. Failed THA patients were older (p<.001), with a greater proportion having comorbid hypertension (p=.008), cardiac disease (p=.014), and history of prior ipsilateral and intracapsular surgeries (p<.001). The failure rate was significantly higher in failed THA patients (39.0% vs 10.3%, p<.001) with significantly shorter implant survivorship on Kaplan-Meier analysis (p=.003). A multivariate Cox proportional hazards model showed that THA failure was the only independent predictor for PFR failure (HR: 4.26, 95% CI: 1.66 - 10.94; p=.003).

CONCLUSION: This study revealed significantly worse PFR implant survivorship in patients undergoing PFR for the indication of failed THA compared to neoplasm. While the underlying etiology of this relationship remains to be explicitly outlined, poor bone quality and soft tissue integrity, multiple prior surgeries, and comorbid conditions are likely contributing factors.

PMID:35032605 | DOI:10.1016/j.arth.2022.01.013

Delayed Gastric Emptying After Multivisceral Resection for Retroperitoneal Sarcoma

Fetched: January 16th, 2022, 5:01am GMT by Marco Baia

Ann Surg Oncol. 2022 Jan 15. doi: 10.1245/s10434-021-11154-z. Online ahead of print.

ABSTRACT

INTRODUCTION: Delayed gastric emptying (DGE) is a common complication in surgery, but incidence and relevance following multivisceral resection are unknown.

METHODS: Data from 100 consecutive patients treated for primary retroperitoneal sarcoma (RPS) were analyzed from our institutional prospectively maintained database from January 2019 to April 2020. DGE severity was graded according to the International Study Group of Pancreatic Surgery and classified as primary or secondary to other complications. The primary outcome was incidence and grade of clinically relevant DGE (grades B-C). Secondary outcomes were correlation with patient, tumor, and treatment characteristics, and non-DGE morbidity [Clavien-Dindo (CD) grade ≥ 3].

RESULTS: Forty-two patients developed DGE and 28 had clinically relevant DGE. DGE was primary in 10 patients and secondary in 18 patients; the most common associated complications were: infections (11/18, 61.1%), pancreatic leak (7/18, 38.9%), bleeding (6/18, 33.3%), and bowel leak (6/18, 33.3%). DGE was related to longer length of hospital stay (P < 0.001), ICU admission (P = 0.004), ICU length of stay (P = 0.001), postoperative complications (CD [Formula: see text] 3 in 14/28 in DGE patients vs 11/72 in no-DGE; P = 0.04), and re-operation (P = 0.03). With multivariate analysis, the independent risk factors for DGE were patient comorbidities (OR 1.05; 95% CI 1.01-1.1; P = 0.04) and tumor size (OR 1.05; 95% CI 1.0-1.1; P = 0.02).

DISCUSSION: Following multivisceral resection, DGE is a clinically relevant event that can be caused by an underlying complication. Prompt diagnosis and treatment of both DGE and any underlying complications led to full recovery in all cases.

PMID:35031920 | DOI:10.1245/s10434-021-11154-z

NTRK-rearranged spindle cell neoplasm of the lower extremity: radiologic-pathologic correlation

Fetched: January 16th, 2022, 5:01am GMT by Cameron J Overfield

Skeletal Radiol. 2022 Jan 14. doi: 10.1007/s00256-022-03995-w. Online ahead of print.

ABSTRACT

Neurotrophic tyrosine receptor kinase (NTRK)-rearranged spindle cell neoplasm is a recently characterized soft tissue tumor and has been classified as provisional by the World Health Organization. Detection of the genetic rearrangement is important because these tumors are amenable to targeted tyrosine kinase inhibitor therapy, which can play a key role in patients with unresectable or advanced disease. Although the spectrum of histopathology associated with this entity is broad, one notable feature is the infiltrative growth pattern, which is most reminiscent of lipofibromatosis-like neural tumor. Description of their diverse histologic attributes has aided recognition, but so far little attention has been paid to correlating the gross appearance and imaging features of these lesions. In this report, we describe the clinical, imaging, histopathological, and genetic features of a soft tissue NTRK-rearranged spindle cell neoplasm. Inclusion of this more recently identified entity into the imaging differential of tumors with intratumoral relatively hypovascular nodules and infiltrative margins is important because testing for NTRK rearrangement is not routinely performed.

PMID:35031835 | DOI:10.1007/s00256-022-03995-w

Permalink for 'Successful Management of Radiation-Associated Insufficiency Fracture of the Tibial Plateau with Low-Intensity Pulsed Ultrasound'

Successful Management of Radiation-Associated Insufficiency Fracture of the Tibial Plateau with Low-Intensity Pulsed Ultrasound

Fetched: January 16th, 2022, 5:01am GMT by Tomoya Matsunobu

Am J Case Rep. 2022 Jan 15;23:e934372. doi: 10.12659/AJCR.934372.

ABSTRACT

BACKGROUND Clinical management of radiation-associated pathological fracture is challenging because of a high nonunion rate and potential for morbidity. We report a case of radiation-associated insufficiency fracture of the tibial plateau after surgery, perioperative chemotherapy, and adjuvant radiation therapy for synovial sarcoma of the proximal calf that was successfully treated with low-intensity pulsed ultrasound (LIPUS). CASE REPORT A healthy 52-year-old Japanese woman presented with a slowly growing, painful soft tissue mass over her proximal calf. Histological examination of core needle biopsy specimens led to a pathological diagnosis of synovial sarcoma. After perioperative ifosfamide and doxorubicin chemotherapy and surgical resection, adjuvant radiation therapy was administered, with a total of 60 Gy in 30 fractions. At 5 months after surgery and 2 months after the completion of radiation therapy, she developed an insufficiency pathological fracture of the proximal tibia without any apparent trauma. The patient was treated with LIPUS for 1 year. There was no collapse or deformity of the knee joint. The patient remained free of symptoms and had no recurrences for 2 years after surgery. CONCLUSIONS This is the first report of radiation-associated pathological fracture that was successfully treated with LIPUS. LIPUS could be a safe and effective treatment option in the management of radiation-associated pathological fractures.

PMID:35031593 | DOI:10.12659/AJCR.934372

Permalink for 'Inoperable scalp cutaneous angiosarcoma: Complete response after definitive external beam radiation therapy - brachytherapy association'

Inoperable scalp cutaneous angiosarcoma: Complete response after definitive external beam radiation therapy - brachytherapy association

Fetched: January 16th, 2022, 5:01am GMT by Charlotte Le Roy

Oral Oncol. 2022 Jan 11;125:105715. doi: 10.1016/j.oraloncology.2022.105715. Online ahead of print.

ABSTRACT

Effective treatments are scarce in non-operable scalp cutaneous angiosarcoma patients. Curative-intent definitive sequential IMRT and plesiobrachytherapy allowed complete response with limited side effect in two elder patients. This could represent a non-invasive therapeutic option for patients with locally advanced presentation.

PMID:35030408 | DOI:10.1016/j.oraloncology.2022.105715

Challenges in diagnosing an extraosseous Ewing sarcoma: A case report

Fetched: January 16th, 2022, 5:01am GMT by Lina Pankratjevaite

Int J Surg Case Rep. 2021 Dec 31;91:106708. doi: 10.1016/j.ijscr.2021.106708. Online ahead of print.

ABSTRACT

INTRODUCTION: Ewing sarcoma is a highly malignant and rare tumour of bones and soft tissue. It may occur at any age, but it is more common in children and teenagers.

CASE REPORT: We report a case of a 56-year-old woman with EES involving the right iliac fossa. Previous abdominal trauma with retroperitoneal hematoma, nonspecific symptoms and unusual age for EES have caused diagnostics difficulties. The first histopathological examination misdiagnosed tumour to be a GIST, and just after the second surgery the accurate diagnosis of EES was made.

CONCLUSIONS: The diagnosis of ES sometimes is complicated and delayed. Prompt detailed examination and imaging studies should be performed to people with long lasting pain without trauma and other nonspecific symptoms, especially followed by a palpable mass. The treatment of EES is multimodal.

PMID:35030406 | DOI:10.1016/j.ijscr.2021.106708

Permalink for 'High-grade transformation of low-grade biphenotypic sinonasal sarcoma: Radiological, morphophenotypic variation and confirmatory molecular analysis'

High-grade transformation of low-grade biphenotypic sinonasal sarcoma: Radiological, morphophenotypic variation and confirmatory molecular analysis

Fetched: January 16th, 2022, 5:01am GMT by Diana Bell

Ann Diagn Pathol. 2022 Jan 10;57:151889. doi: 10.1016/j.anndiagpath.2021.151889. Online ahead of print.

ABSTRACT

Biphenotypic sinonasal sarcoma (BSNS) is a recently described spindle cell sarcoma with neural and myogenic differentiation which arises exclusively in the sinonasal region. A man presented with swelling of left eyelid, and history of resection of a low-grade spindle cell mesenchymal tumor of left sinonasal cavity performed 15 years before. The original diagnosis was synovial sarcoma. Current MRI showed left supraorbital mass with intracranial extension. IR biopsy confirmed recurrence, and a left orbital craniotomy was done. NGS identified PAX3-MAML3 fusion in both, current and original tumor. The sarcoma was reclassified as BSNS, recurrent, with higher grade transformation. While the morphology, phenotype and molecular signature were in keeping with BSNS, the tumor showed biological progression towards a high-grade sarcoma. High-grade transformation of low-grade BSNS has not been described so far. High-grade transformation was not appreciated at the time of initial diagnosis, and it occurred in the local recurrence 15 years after.

PMID:35030392 | DOI:10.1016/j.anndiagpath.2021.151889

Cutaneous Desmoplastic Leiomyosarcoma Development at Smallpox Vaccination Site

Fetched: January 16th, 2022, 5:01am GMT by Meera H Patel

Skinmed. 2021 Dec 1;19(6):462-463. eCollection 2021.

ABSTRACT

An 81-year-old man presented to the clinic with a 2.1 cm firm, skin-colored subcutaneous tumor on the left upper arm (Figure 1). The lesion arose at the site of a past smallpox vaccination and had been slowly enlarging for approximately 4 years. The differential diagnosis included sympastic leiomyoma, and a variety of desmoplastic spindle cell lesions such as desmoplastic melanoma, cutaneous spindle cell carcinoma, and desmoplastic leiomyosarcoma. Punch biopsy and immunohistochemical staining revealed positive spindle cells for desmin and caldesmon (Figures 2 and 3). Immunostain for p53 was also strongly and uniformly positive. Owing to poor circumscription on histopathology, symplastic leiomyosarcoma was ruled out. Demoplastic melanoma was also excluded due to positive immunoreaction to muscle markers (desmin and caldesmon) and negative S-100 staining. Additionally, cutaneous spinde cell carcinoma was also ruled out due to negative p63 and cytokeratin staining. Ultimately, clinicopathologic correlation favored a diagnosis of desmoplastic leiomyosarcoma. Staged excisions were performed to eradicate the lesion.

PMID:35022120

Multidetector CT findings of primary pleural angiosarcoma : a systematic review, an additional cases report

Fetched: January 16th, 2022, 5:01am GMT by Bo Mi Gil

Cancer Imaging. 2022 Jan 12;22(1):6. doi: 10.1186/s40644-021-00435-1.

ABSTRACT

BACKGROUND: To demonstrate and analyze the relatively common imaging findings in this rare primary pleural angiosarcoma (PPA).

CASE PRESENTATION: Three cases of PPA, proven by video-assisted thoracic surgery biopsies are retrospectively reviewed. Patients were all male. Age ranges from 65 to 75 years old age (mean; 69). Major chief complaints were dyspnea and chest pain. One has a history of colon cancer, the other has a tuberculosis history and the other has no known history. Multidetector chest CT and PET CT were all done. Immunohistochemical studies were performed including CD31, CD34, or factor VIII-related antigen, vimentin, and cytokeratin. We also review the literatures on recently published PPA. All masses were from 1 to 10 cm. All three patients had multiple pleural based masses, which were ovoid in shape with relatively sharp margin in unilateral hemithorax. Multiple small circumscribed pleural masses are limited in the pleural space in two patients, whereas two, huge lobulated masses about up to 10 cm were present with pleural and extrapleural involvement in one patient. In two patients with pleural mass only, multiple pleural masses were only seen in parietal pleura in one patient and were in both visceral and parietal pleura in one patient. Pleural effusion were found in one side in one patient and in both sides in one patient. One angiosarcoma was arised from chronic tuberculotic pleurisy sequelae. All pleural masses are heterogenous with irregular internal low densities in all patients. Hematogenous metastases were found in liver, vertebra, rib in one patient, and were in lungs with mediastinal lymph node metastases in the other patient. Three patients survived for longer than 3months after diagnosis, but continued to deteriorate rapidly. Two patients underwent chemotherapy after surgical excision, and the other one with multiple metastases treated chemotherapy after CT-guided biopsy, but eventually all died. As a result of comparative analysis of a total of 13 patients' images including 10 cases previously published, there was pleural effusion in all except 2 cases.

CONCLUSIONS: PPA were all necrotic without any vascularized enhancing nature, and manifested as unilateral circumscribed or localized pleural-based masses.

PMID:35022068 | DOI:10.1186/s40644-021-00435-1

Mesenchymal Neoplasms of the Prostate and Seminal Vesicles: Spectrum of Disease with Radiologic-Pathologic Correlation

Fetched: January 15th, 2022, 5:02am GMT by Leonardo P Marcal

Radiographics. 2022 Jan 14:210084. doi: 10.1148/rg.210084. Online ahead of print.

ABSTRACT

There is a wide spectrum of benign and malignant mesenchymal neoplasms of the prostate, which account for less than 1% of all prostatic tumors. These include distinctive tumors that arise from the specialized prostatic stroma and site-agnostic neoplasms such as smooth muscle tumors, fibrous or myofibroblastic neoplasms, neurogenic tumors, vascular tumors, and a plethora of sarcomas. Select tumors show classic sites of origin within the prostate. While stromal tumors of uncertain malignant potential (STUMPs) commonly involve the peripheral zone at the prostate base, leiomyomas typically originate from the central prostate toward the apex. Some "prostatic" neoplasms such as gastrointestinal stromal tumors, solitary fibrous tumor (SFT), paragangliomas, and neurogenic tumors arise primarily from periprostatic soft tissues. Most mesenchymal tumors of the prostate and seminal vesicles manifest as large tumors that cause nonspecific symptoms; prostate-specific antigen level is not typically elevated. Diverse mesenchymal neoplasms demonstrate characteristic histopathologic and immunocytochemical features and variable cross-sectional imaging findings. While leiomyoma and SFT typically display low signal intensity on T2-weighted images, synovial sarcomas commonly show hemorrhage. Diagnosis is difficult because of the rarity and lack of awareness of the tumors and the significant overlap in histopathologic features. Select tumors show characteristic genetic abnormalities that allow the diagnosis to be established. For example, more than 90% of SFTs are characterized by a unique NAB2-STAT6 gene fusion, and more than 95% of synovial sarcomas are associated with a distinctive SYT-SSX chimeric transcript. Accurate diagnosis is imperative for optimal management owing to markedly different tumor biology as well as attendant therapeutic and prognostic implications. While STUMPs commonly recur, sarcomas typically charter an aggressive course with poor prognosis. Online supplemental material is available for this article. ^©RSNA, 2022.

PMID:35030067 | DOI:10.1148/rg.210084

Biphasic synovial sarcoma with a striking morphological divergence from the main mass to lymph node metastasis: A case report

Fetched: January 15th, 2022, 5:02am GMT by Ha Young Woo

Medicine (Baltimore). 2022 Jan 7;101(1):e28481. doi: 10.1097/MD.0000000000028481.

ABSTRACT

RATIONALE: Synovial sarcoma accounts for 5% to 10% of all soft tissue sarcomas and involves almost any anatomic site, particularly the deep soft tissue of the extremities of young adults. The incidence rate of lymph node metastases in synovial sarcoma is 3% to 7%, but the detailed morphological features of the metastatic tumors in the lymph node have not been documented.

PATIENT CONCERNS: A 64-year-old Korean man presented with a huge mass in the left lower thorax and multiple hypermetabolic lymph nodes along the mediastinal, supraclavicular, internal mammary, and retrocrural regions.

DIAGNOSES: The patient was diagnosed with primary pleuropulmonary biphasic synovial sarcoma with lymph node metastases, where the main mass mostly comprised spindle cells (>95%) and the metastatic lymph nodes comprised only epithelial cells.

INTERVENTIONS: Left lower lobe lobectomy with the resection of the chest wall (including left ribs 8-10) and diaphragm and mediastinal lymph node dissection were performed.

OUTCOMES: In the 2-month follow-up period, there have been no complications so far, and the attending physician is currently planning for the adjuvant chemotherapy.

LESSONS: The main mass and the metastatic lesion can be clearly different morphologically. In tumors with biphasic differentiation, such as synovial sarcoma, cells that constitute only a small fraction of the main mass may appear as the dominant cells in metastatic lesions.

PMID:35029897 | PMC:PMC8735718 | DOI:10.1097/MD.0000000000028481

Permalink for 'Impact of locus of care on outcomes in adolescents and young adults with osteosarcoma and Ewing sarcoma treated at pediatric versus adult cancer centers: An IMPACT cohort study'

Impact of locus of care on outcomes in adolescents and young adults with osteosarcoma and Ewing sarcoma treated at pediatric versus adult cancer centers: An IMPACT cohort study

Fetched: January 15th, 2022, 5:02am GMT by Mohammadreza Mortazavi

Pediatr Blood Cancer. 2022 Jan 14:e29458. doi: 10.1002/pbc.29458. Online ahead of print.

ABSTRACT

BACKGROUND: Location of cancer care (LOC; pediatric vs. adult center) impacts outcomes in adolescents and young adults (AYA) with some cancer types. Data on the impact of LOC on survival in AYA with osteosarcoma (OSS) and Ewing sarcoma (EWS) are limited OBJECTIVES: To compare differences in demographics, disease/treatment characteristics, and survival in a population-based cohort of AYA with OSS or EWS treated at pediatric versus adult centers METHODS: The Initiative to Maximize Progress in Adolescent Cancer Therapy (IMPACT) cohort captured demographic, disease, and treatment data for all AYA (15-21 years old) diagnosed with OSS and EWS in Ontario, Canada between 1992 and 2012. Patients were linked to provincial administrative health care databases. Outcomes were compared between patients treated in pediatric versus adult centers.

RESULTS: One hundred thirty-seven AYA were diagnosed with OSS (LOC: 47 pediatric, 90 adult) and 84 with EWS (38 pediatric, 46 adult). AYA treated at pediatric centers were more likely to be enrolled in a clinical trial (OSS 55% vs. 1%, p < .001; EWS 53% vs. 2%, p < .001) and received higher cumulative chemotherapy doses. Five-year event-free survival (EFS ± standard error) in OSS and EWS were 47% ± 4% and 43% ± 5%, respectively. In multivariable analysis, the impact of LOC (pediatric vs. adult center) on EFS in OSS (adjusted hazard ratio [HR] 1.15, 95% confidence interval [CI]: 0.58-2.27, p = .69) and EWS (adjusted HR 1.82, 95% CI: 0.97-3.43, p = .06) was not statistically significant.

CONCLUSION: Despite disparities in trial participation and chemotherapy doses, outcomes did not differ by LOC suggesting that AYA with bone tumors can be treated at either pediatric or adult centers.

PMID:35029312 | DOI:10.1002/pbc.29458

Congenital soft tissue Ewing's sarcoma: A case report of pre- and postnatal magnetic resonance imaging findings

Fetched: January 15th, 2022, 5:02am GMT by Chaoxiang Yang

Medicine (Baltimore). 2022 Jan 14;101(2):e28587. doi: 10.1097/MD.0000000000028587.

ABSTRACT

RATIONALE: Most congenital soft tissue masses are benign. Ewing's sarcoma (ES) is a highly malignant tumor that commonly occurs in children and adolescents and rarely occurs during the fetal period. Cases of congenital soft tissue ES with magnetic resonance imaging (MRI) findings are scarce. To the best of our knowledge, no previous reports have described the pre- and postnatal MRI findings of ES.

PATIENT CONCERNS: We present a case of congenital soft tissue ES arising in the body wall, which was examined using MRI during the prenatal and neonatal periods.

DIAGNOSES: Malignancy was suspected by diffusion-weighted imaging, which demonstrated restricted diffusion within the mass even during the fetal period. ES was confirmed via histopathological examination after birth.

INTERVENTIONS: The patient initially underwent conservative treatment for suspected hemangioma. Tumorrectomy was undergone after three weeks based on previously dissatisfied therapeutic effects.

OUTCOMES: The patient died of multiple distant metastases despite undergoing postoperative chemotherapy and metastasectomies.

LESSONS: Fetal or neonatal soft tissue ES may be clinically misdiagnosed as a hemangioma. It is important to suspect this through an imaging approach such as diffusion-weighted imaging.

PMID:35029239 | DOI:10.1097/MD.0000000000028587

Primary spindle cell sarcoma of gallbladder: An unusual case report and a literature review

Fetched: January 15th, 2022, 5:02am GMT by Xin Long

Medicine (Baltimore). 2022 Jan 14;101(2):e28549. doi: 10.1097/MD.0000000000028549.

ABSTRACT

INTRODUCTION: Primary spindle cell sarcoma of the gallbladder is a rare condition.

PATIENT CONCERNS: A 67-year-old woman was admitted to a local hospital with a chief complaint of abdominal pain in the right upper quadrant for the past 2 months.

DIAGNOSIS AND INTERVENTION: Surgical resection was performed following the diagnosis of primary gallbladder sarcoma with local hepatic metastasis. Histological examination confirmed a diagnosis of primary spindle cell sarcoma and hepatic metastasis with simultaneous cholecystolithiasis.

OUTCOMES: Adjuvant chemoradiation therapy was not performed because the patient refused treatment. Three months after the surgery, a relapsed lesion was diagnosed. The patient underwent transcatheter arterial chemoembolization.

CONCLUSIONS: The disease should be differentially diagnosed from gallbladder carcinoma or carcinosarcoma with hepatic metastasis. An aggressive surgical approach should be based on a balance between the risk of surgery and the outcome.

PMID:35029216 | DOI:10.1097/MD.0000000000028549

Permalink for 'Clinicopathological characteristics and treatment of patients with high-grade endometrial stromal sarcoma: A retrospective study of 40 cases'

Clinicopathological characteristics and treatment of patients with high-grade endometrial stromal sarcoma: A retrospective study of 40 cases

Fetched: January 15th, 2022, 5:02am GMT by Huimin Bai

Medicine (Baltimore). 2022 Jan 14;101(2):e28490. doi: 10.1097/MD.0000000000028490.

ABSTRACT

To investigate the clinicopathological characteristics of patients with high-grade endometrial stromal sarcoma (HG-ESS).The clinicopathological characteristics, treatments, and prognostic information of consecutive HG-ESS patients were collected from medical records and then evaluated.A total of 40 women were included in the analysis. The immunohistochemical profiles indicated that HG-ESS tumors tend to be locally or weakly positive for vimentin (100%) and CD10 (72.0%) but mostly negative for desmin (7.7%) and AE1/AE3 (9.1%). The progression-free survival intervals and the clinical benefit rates of patients receiving radiotherapy and/or chemotherapy were slightly longer and higher than those receiving simple observation (progression-free survival: 6 and 5 months vs 2 months; clinical benefit rate: 83.3% and 75.0% vs 28.6%). The 1-year disease-specific survival (DSS) rate was 62.7%. Tumor size, myometrial invasion, lymphovascular space invasion, cervical involvement, Federation International of Gynecology and Obstetrics (FIGO) stage, and residual disease all significantly affected the DSS rate (P < .001, =.002, <.001, =.004, <.001, and <.001, respectively). For patients with stage I disease, the 1-year DSS rate was as high as 91.7%, in contrast to 66.7%, 26.7%, and 0% for those with stage II, III, and IV disease, respectively.HG-ESS is associated with an adverse prognosis. FIGO stage could effectively predict the prognosis of patients with this lethal disease. Immunohistochemical markers, vimentin+/CD10+ (local or very weak), in combination with desmin-/AE1/AE3-, may be helpful for improving the diagnostic accuracy of this lethal condition. The therapeutic roles of adjuvant chemotherapy and radiotherapy warrant further investigation.

PMID:35029198 | DOI:10.1097/MD.0000000000028490

Fibrinogen-like protein 2 in gastrointestinal stromal tumour

Fetched: January 15th, 2022, 5:02am GMT by Olli-Pekka Pulkka

J Cell Mol Med. 2022 Jan 14. doi: 10.1111/jcmm.17163. Online ahead of print.

ABSTRACT

Gastrointestinal stromal tumour (GIST), the most common sarcoma of the gastrointestinal tract, can be treated effectively with tyrosine kinase inhibitors, such as imatinib. Cancer immune therapy has limited efficacy, and little is known about the immune suppressive factors in GISTs. Fibrinogen-like protein 2 (FGL2) is expressed either as a membrane-associated protein or as a secreted soluble protein that has immune suppressive functions. We found that GISTs expressed FGL2 mRNA highly compared to other types of cancer in a large human cancer transcriptome database. GIST expressed FGL2 frequently also when studied using immunohistochemistry in two large clinical series, where 333 (78%) out of the 425 GISTs were FGL2 positive. The interstitial cells of Cajal, from which GISTs may originate, expressed FGL2. FGL2 expression was associated with small GIST size, low mitotic counts and low tumour-infiltrating lymphocyte (TIL) counts. Patients whose GIST expressed FGL2 had better recurrence-free survival than patients whose GIST lacked expression. Imatinib upregulated FGL2 in GIST cell lines, and the patients with FGL2-negative GIST appeared to benefit most from long duration of adjuvant imatinib. We conclude that GISTs express FGL2 frequently and that FGL2 expression is associated with low TIL counts and favourable survival outcomes.

PMID:35029030 | DOI:10.1111/jcmm.17163

Pulmonary artery sarcoma: An important mimic of pulmonary embolism-Case reports and literature review

Fetched: January 15th, 2022, 5:02am GMT by Colin Tuft

Respirol Case Rep. 2022 Jan 9;10(2):e0897. doi: 10.1002/rcr2.897. eCollection 2022 Feb.

ABSTRACT

Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low d-dimer, troponin T or NT-proBNP, as well as characteristic findings on CT pulmonary angiography such as the 'wall eclipsing sign' and an non-dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2-year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.

PMID:35028154 | PMC:PMC8743422 | DOI:10.1002/rcr2.897

Permalink for 'Sequential allogeneic transplantation and ruxolitinib maintenance for a synchronous PCM1-JAK2 positive myeloid sarcoma and acute B-lymphoblastic leukemia'

Sequential allogeneic transplantation and ruxolitinib maintenance for a synchronous PCM1-JAK2 positive myeloid sarcoma and acute B-lymphoblastic leukemia

Fetched: January 15th, 2022, 5:02am GMT by Giuliana Rizzuto

Clin Case Rep. 2022 Jan 7;10(1):e05212. doi: 10.1002/ccr3.5212. eCollection 2022 Jan.

ABSTRACT

The translocation t(8;9)(p22;p24) results in the production of a chimeric PCM1-JAK2 fusion protein leading to the constitutive activation of the Janus Kinase 2 that renders this disease potentially sensitive to ruxolitinib. Here, we report an interesting case of PCM1-JAK2 myeloproliferative neoplasm evolving in myeloid sarcoma and B precursor ALL.

PMID:35028141 | PMC:PMC8741870 | DOI:10.1002/ccr3.5212

Overview of the Clinical Features of Li-Fraumeni Syndrome and the Current European ERN GENTURIS Guideline

Fetched: January 15th, 2022, 5:02am GMT by Christian Peter Kratz

Geburtshilfe Frauenheilkd. 2021 Oct 25;82(1):42-49. doi: 10.1055/a-1541-7912. eCollection 2022 Jan.

ABSTRACT

Patients with a tumour-risk syndrome have a significantly increased risk of developing cancer during their lifetime. A positive family history of tumour disease or an unusually early age of onset may be indicative of a tumour risk syndrome. With the diagnosis of a tumour risk syndrome it is possible to recommend a risk-adapted tumour surveillance programme for the patient and (asymptomatic) family members at risk. This facilitates early detection of possible tumours and thus often prevents advanced tumour stages. Li-Fraumeni syndrome is associated with a significantly increased risk of sarcoma and breast cancer in particular, but it is often not diagnosed clinically in those affected. This article reviews the clinical picture, genetic cause and special aspects in the diagnosis and care of patients with Li-Fraumeni syndrome. The initiative resulted from the European reference network GENTURIS, which has set itself the task of improving the identification and care of patients with tumour risk syndromes. A first step is the recent publication of a European guideline for Li-Fraumeni syndrome, which is summarised here and discussed in the context of existing recommendations.

PMID:35027859 | PMC:PMC8747895 | DOI:10.1055/a-1541-7912

Robot-assisted versus conventional laparoscopic adrenalectomy: Results from the EUROCRINE Surgical Registry

Fetched: January 15th, 2022, 5:02am GMT by Safa Vatansever

Surgery. 2022 Jan 10:S0039-6060(21)01189-2. doi: 10.1016/j.surg.2021.12.003. Online ahead of print.

ABSTRACT

BACKGROUND: Adrenalectomy is routinely performed via the minimally invasive approach. Safety of adrenalectomy using the robot-assisted technique has been widely demonstrated by several series, but the literature is scarce regarding the comparison of conventional laparoscopic versus robot-assisted approach. We decided to carry out a multicenter study to compare clinical and surgical outcomes between laparoscopic and robotic adrenalectomy.

METHODS: This is a retrospective case-control study, including data from centers affiliated to the Surgical Registry EUROCRINE. Patients undergoing laparoscopic surgery for adrenal tumors and registered between 2015 and 2018 were included. Robot-assisted versus laparoscopic adrenalectomy was compared. All comparisons were carried out in terms of complication rate, conversion rate and duration of stay.

RESULTS: A total of 1,005 patients from 46 clinics underwent robotic or conventional laparoscopic adrenalectomy. Median age was 55 (interquartile range: 45-65) years. Robotic adrenalectomy was performed in 189 (18.8%) patients. According to Clavien-Dindo classification, complication rate was lower in the robotic surgery group (1.6% vs 16.5%, P < .001). Laparoscopic surgery and active hormonal status were significantly correlated with complications, both in univariate and multivariate analysis. There was no significant difference between laparoscopic and robotic surgery groups, in terms of conversion rate (2.1% vs 0.5%, respectively, P = .147). Duration of stay was shorter in the robotic adrenalectomy group (82.1% vs 28.8%, P < .001).

CONCLUSION: Analysis of the EUROCRINE database supports that robotic adrenalectomy resulted in a lower complication rate and shorter duration of stay, compared with laparoscopic adrenalectomy. Granular data to support this is warranted.

PMID:35027208 | DOI:10.1016/j.surg.2021.12.003

Primary low-grade extrauterine endometrial stromal sarcoma: analysis of 10 cases with a review of the literature

Fetched: January 15th, 2022, 5:02am GMT by You Wu

World J Surg Oncol. 2022 Jan 13;20(1):17. doi: 10.1186/s12957-021-02474-1.

ABSTRACT

BACKGROUND: This study aimed to analyze the clinical and pathological features of extrauterine endometrial stromal sarcoma (EESS) and explore an effective therapeutic regimen to reduce the recurrence rate in low-grade EESS patients.

METHODS: Ten LG-EESS patients who were treated at the Chinese Academy of Medical Sciences Cancer Institute and Hospital from June 1999 to June 2019 were collected and analyzed.

RESULTS: (1) Patient demographics are summarized in manuscript. Preoperative CA125 examination showed that 8 patients had a median level of 49.5 U/L (15.4-168.0 U/L). (2) All ten patients underwent tumor cytoreductive surgery. Five patients underwent optimal tumor resection and achieved an R0 resection. After the initial surgery, 7 patients who had multiple metastasis were treated with adjuvant chemotherapy, 2 patients with vaginal ESS were treated with chemotherapy and radiation therapy, and 6 patients with ER/PR positive received hormone therapy with or without chemotherapy. (2) Most EESS patients had multiple tumors. The omentum was the most commonly affected site, followed by the ovaries. (3) The median follow-up was 94 (range: 27-228) months, and recurrence was observed in 3 patients (n = 10, 30%) who underwent non-optimal surgery and no hormone therapy. The 5-year and 10-year DFS rates were both 70%, as shown in Fig. 2. OS was both 100% at 5 and 10 years.

CONCLUSION: As a conclusion, EESS is a rare disease and LG-EESS has a good prognosis. Surgery remains the available treatment for patients. LG-EESS has a risk of late recurrence which requires a long-term follow-up. With a limited sample size, our study shows optimal tumor reductive surgery and adjuvant hormone therapy may significantly reduce the risk of recurrence.

PMID:35027071 | DOI:10.1186/s12957-021-02474-1

Permalink for 'Annual report of the Committee on Gynecologic Oncology, the Japan Society of Obstetrics and Gynecology: Annual Patient Report for 2018 and Annual Treatment Report for 2013'

Annual report of the Committee on Gynecologic Oncology, the Japan Society of Obstetrics and Gynecology: Annual Patient Report for 2018 and Annual Treatment Report for 2013

Fetched: January 14th, 2022, 5:01am GMT by Satoru Nagase

J Obstet Gynaecol Res. 2022 Jan 13. doi: 10.1111/jog.15134. Online ahead of print.

ABSTRACT

To provide information including the trend of gynecological malignancies in Japan, we hereby present the Annual Patient Report for 2018 and the Annual Treatment Report for 2013, on the outcomes of patients who started treatment in 2013. The Japan Society of Obstetrics and Gynecology maintains an annual tumor registry, where information on gynecological malignancies from various participating institutions is gathered. The data of patients whose treatment with gynecologic malignancies was initiated in 2018 were analyzed retrospectively. Survival of the patients who started treatment with cervical, endometrial, and ovarian cancer in 2013 was analyzed by using the Kaplan-Meier, log-rank, and Wilcoxson tests. Treatment was initiated in 2018 for 7304 patients with cervical cancer; 11 230 with endometrial cancer; 7031 with ovarian, tubal, and peritoneal cancer; 2072 with ovarian borderline tumors; and with the others (222 vulvar cancer, 159 vaginal cancer, 413 uterine sarcoma, 54 uterine adenosarcoma, and 135 trophoblastic diseases). This clinicopathological information was summarized as the Patient Annual Report. The 5-year survival rates of the patients with cervical cancer were 93.1%, 75.9%, 59.1%, and 31.2% for Stages I, II, III, and IV, respectively. The 5-year survival rates for the patients with endometrial cancer were 94.1%, 89.2%, 73.6%, and 25.8% for Stages I, II, III, and IV, respectively. The 5-year survival rates for the patients with ovarian cancer (surface epithelial-stromal tumors) were 89.7%, 76.8%, 49.1%, and 32.4% for Stages I, II, III, and IV, respectively. The annual report is important to provide knowledge on gynecological malignancy trends in Japan.

PMID:35026864 | DOI:10.1111/jog.15134

Solitary fibrous tumor of the pleura mimicking a soft tissue sarcoma of the chest wall

Fetched: January 14th, 2022, 5:01am GMT by Kentaro Watanabe

Int J Surg Case Rep. 2021 Dec 30;91:106746. doi: 10.1016/j.ijscr.2021.106746. Online ahead of print.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Solitary fibrous tumors of the pleura (SFTPs) present a diagnostic challenge. We herein report a successful case mimicking a soft tissue sarcoma of the chest wall by a meticulous evaluation of the conventional images.

CASE PRESENTATION: A 51-year-old woman presented with a left thoracic mass. The mass exhibited an extrapleural sign, which suggested a chest wall origin. However, the mass was found to be located more caudally by additional computed tomography. This positional change suggested that the mass was pedunculated from the visceral pleura, and an SFTP was suspected. The mass was found to originate from the visceral pleura of the left lower lobe and a pathological diagnosis of an SFTP was confirmed.

CLINICAL DISCUSSION: Although a positional shift with a postural change or the respiratory phase is a well-known characteristic radiological finding, such an intentional imaging study is available only for suspicious cases of SFTPs.

CONCLUSIONS: SFTPs pose a diagnostic challenge because of their rarity and the lack of specific radiological findings. Even conventional radiological images can be diagnostic by performing a meticulous evaluation regardless of any specific diagnosis being initially assumed.

PMID:35026682 | DOI:10.1016/j.ijscr.2021.106746

Permalink for 'Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case-series analysis from the Italian Rare Cancers Network'

Weekly cisplatin with or without imatinib in advanced chordoma: A retrospective case-series analysis from the Italian Rare Cancers Network

Fetched: January 14th, 2022, 5:01am GMT by Giacomo G Baldi

Cancer. 2022 Jan 13. doi: 10.1002/cncr.34083. Online ahead of print.

ABSTRACT

BACKGROUND: To report on a retrospective case-series analysis of weekly cisplatin (wCDDP) as a single agent or combined with imatinib (wCDDP/I) in patients with advanced chordoma treated within the Italian Rare Cancer Network.

METHODS: Adult patients with a diagnosis of advanced, brachyury-positive chordoma, treated from April 2007 to October 2020 with wCDDP or wCDDP/I were retrospectively identified. Imatinib was withheld at the same time as wCDDP. Response according to Response Evaluation Criteria in Solid Tumors, overall survival (OS), and progression-free survival (PFS) were analyzed.

RESULTS: Thirty-three consecutive patients were identified (wCDDP as front-line n = 8 [24.2%]; wCDDP as a further line n = 25 [75.8%]; prior imatinib n = 25 [75.8%]; evidence of progression before starting wCDDP n = 33). Of 32 patients evaluable for response (wCDDP, n = 22 [68.8%]; wCDDP/I, n = 10 [31.3%]), best response was stable disease (SD) in 27 patients (84.3%) and progression in 5 patients (15.6%). At a median follow-up of 54 months, the median OS (m-OS) was 30.3 months (interquartile range [IQR], 18.1-56.6), the m-PFS was 8.0 months (IQR, 5.1-17.0), the 6-month PFS rate was 65.2%, and the 12-month PFS rate was 30.3%. Of 22 patients who received wCDDP, the best response was SD in 18 patients (81.8%) and progression in 4 patients (18.2%), and the m-PFS was 8.0 months (IQR, 5.1-17.0 months). Of 10 patients who received treatment with wCDDP/I, the best response was SD in 9 patients (90%) and progression in 1 patient (10%), and the m-PFS was 9.3 months (IQR, 4.9-26.5 months).

CONCLUSIONS: This series suggests that wCDDP, both as a single agent and combined with imatinib, has antitumor activity in chordoma. Although no dimensional responses were observed, 65% and 30% of previously progressive patients were progression-free at 6 and 12 months, respectively. A prospective study is warranted.

PMID:35026050 | DOI:10.1002/cncr.34083

Bilateral Testicular Mass Presenting as a Rare Manifestation of Extramedullary Plasmacytoma on 18F-FDG PET/CT

Fetched: January 14th, 2022, 5:01am GMT by Caner Civan

Clin Nucl Med. 2022 Jan 12. doi: 10.1097/RLU.0000000000004043. Online ahead of print.

ABSTRACT

We present the case of a 48-year-old man diagnosed with metastatic Ewing sarcoma who was referred for 18F-FDG PET/CT for treatment response evaluation following chemoradiotherapy. The patient also had complaints of new-onset testicular swelling at the time of imaging. Although the metastatic bone disease showed a complete metabolic response to treatment, 18F-FDG PET/CT demonstrated significantly increased metabolic activity in bilateral testicular mass. Consequently, the patient underwent bilateral orchiectomy, and histopathologic examination revealed bilateral testicular plasmacytoma, a rare manifestation of extramedullary plasmacytoma.

PMID:35025778 | DOI:10.1097/RLU.0000000000004043

Adamantinoma-Like Ewing Sarcoma of the Head and Neck: A Case-Series of a Rare and Challenging Diagnosis

Fetched: January 14th, 2022, 5:01am GMT by Munita Bal

Head Neck Pathol. 2022 Jan 13. doi: 10.1007/s12105-022-01412-1. Online ahead of print.

ABSTRACT

Adamantinoma-like Ewing sarcoma (ALES) is a rare malignant tumor characterized by EWSR1::FLI1 related fusions and complex epithelial differentiation. ALES poses a tremendous diagnostic challenge owing to its resemblance to a wide variety of common head and neck malignancies. We aimed to study the clinicopathologic spectrum of ALES diagnosed at our institute. A retrospective review of the clinical and pathologic features of all EWSR1-rearranged ALES cases was performed after confirming the diagnosis. The cases lacking EWSR1 rearrangement were excluded. A total of 7 patients were analyzed. The median age was 27 years (range 7-42 years). There were 4 males and 3 female patients. Tumors were distributed as follows: maxilla (n = 2), parotid (n = 2), nasal cavity (n = 1), ethmoid/maxilla (n = 1), and thyroid (n = 1). Tumor size ranged from 2.2 to 5.5 cm. On microscopy, tumors displayed nested-lobular architecture, monomorphic cells, and interlobular fibrotic stroma. Other features included: palisading (n = 5), squamous differentiation (n = 2), keratinization (n = 1), colonisation of salivary ducts (n = 1) and thyroid follicles (n = 1), follicle-like cysts (n = 3), calcification (n = 2), necrosis (n = 3). Mitotic rate was 4-15/2 mm². On immunohistochemistry, cytokeratins (100%), p40 (100%), strong/diffuse membranous CD99 (100%), NKX2.2 (100%), Fli-1 (71%), and synaptophysin (71%) was positive. Patients received chemotherapy (n = 7) and radiotherapy (n = 4). Two patients developed recurrence at 6 and 10 months; 3 developed metastases at 0, 6, and 25 months. ALES is a rare and aggressive malignancy that mimics diverse neoplasms common in the head and neck region. Awareness of the morphologic and immunohistochemistry spectrum of this tumor is essential to avoid diagnostic errors.

PMID:35025056 | DOI:10.1007/s12105-022-01412-1

Permalink for 'Peripheral-type primitive neuroectodermal tumor of the ovary with EWSR1-FLI1 fusion transcript: a case report and brief review of literature'

Peripheral-type primitive neuroectodermal tumor of the ovary with EWSR1-FLI1 fusion transcript: a case report and brief review of literature

Fetched: January 14th, 2022, 5:01am GMT by Corina Grigoriu

Rom J Morphol Embryol. 2021 Apr-Jun;62(2):581-586. doi: 10.47162/RJME.62.2.26.

ABSTRACT

Primitive neuroectodermal tumors (PNETs) of the ovary are extremely rare tumors composed of undifferentiated small cells with round nuclei and scant cytoplasm. They are rare in general and extremely rare in the female gynecological tract, where they most commonly affect the ovary, followed by the uterine corpus. The most common presenting symptoms are abdominal pain, bloating and the presence of a pelvic mass. Diagnosis mainly relies on immunohistochemical and fluorescence in situ hybridization (FISH). Due to the rarity of these tumors, there are no standard therapeutic guidelines and treatment consists of surgery, various chemotherapy regimens and/or radiotherapy. In this article, we report the case of a 30-year-old female with peripheral-type PNET (pPNET) of the ovary featuring Ewing sarcoma breakpoint region 1-Friend leukemia integration 1 (EWSR1-FLI1) fusion transcript, confirmed by next-generation sequencing (NGS).

PMID:35024748 | DOI:10.47162/RJME.62.2.26

Neck synovial sarcoma: case presentation

Fetched: January 14th, 2022, 5:01am GMT by Konstantinos Sapalidis

Rom J Morphol Embryol. 2021 Apr-Jun;62(2):575-579. doi: 10.47162/RJME.62.2.25.

ABSTRACT

Head and neck synovial sarcoma (HNSS) is a rare tumor with a few case reports or case series being published in the literature. We present the case of a 68-year-old patient admitted to our department for management of a palpable neck mass. After initial investigation and due to major problems of differential diagnosis, there was performed a wide excision of the tumor. Histopathology examination revealed an HNSS.

PMID:35024747 | DOI:10.47162/RJME.62.2.25

A novel immune-related gene signature predicting survival in sarcoma patients

Fetched: January 14th, 2022, 5:01am GMT by Haoyu Ren

Mol Ther Oncolytics. 2021 Dec 9;24:114-126. doi: 10.1016/j.omto.2021.12.007. eCollection 2022 Mar 17.

ABSTRACT

Sarcomas are a heterogeneous group of rare mesenchymal tumors. The migration of immune cells into these tumors and the prognostic impact of tumor-specific factors determining their interaction with these tumors remain poorly understood. The current risk stratification system is insufficient to provide a precise survival prediction and treatment response. Thus, valid prognostic models are needed to guide treatment. This study analyzed the gene expression and outcome of 980 sarcoma patients from seven public datasets. The abundance of immune cells and the response to immunotherapy was calculated. Immune-related genes (IRGs) were screened through a weighted gene co-expression network analysis (WGCNA). A least absolute shrinkage and selection operator (LASSO) Cox regression was used to establish a powerful IRG signature predicting prognosis. The identified IRG signature incorporated 14 genes and identified high-risk patients in sarcoma cohorts. The 14-IRG signature was identified as an independent risk factor for overall and disease-free survival. Moreover, the IRG signature acted as a potential indicator for immunotherapy. The nomogram based on the risk score was built to provide a more accurate survival prediction. The decision tree with IRG risk score discriminated risk subgroups powerfully. This proposed IRG signature is a robust biomarker to predict outcomes and treatment responses in sarcoma patients.

PMID:35024438 | PMC:PMC8718575 | DOI:10.1016/j.omto.2021.12.007

Dermoscopy of Bacillary Angiomatosis: Utility in Diagnosis and Therapeutic Control

Fetched: January 14th, 2022, 5:01am GMT by Maria Leonor Enei

Dermatol Pract Concept. 2021 Oct 1;11(4):e2021088. doi: 10.5826/dpc.1104a88. eCollection 2021 Oct.

NO ABSTRACT

PMID:35024220 | PMC:PMC8648418 | DOI:10.5826/dpc.1104a88

Permalink for 'A case of a 22-year-old man with primary synovial sarcoma of the parapharyngeal space with an AR somatic mutation: A case report and review of the literature'

A case of a 22-year-old man with primary synovial sarcoma of the parapharyngeal space with an AR somatic mutation: A case report and review of the literature

Fetched: January 14th, 2022, 5:01am GMT by He Jiang

SAGE Open Med Case Rep. 2022 Jan 8;10:2050313X211068646. doi: 10.1177/2050313X211068646. eCollection 2022.

ABSTRACT

This case report describes a 22-year-old man with a pharyngeal foreign body sensation arising from the left side of the postpharyngeal wall. Histological examination showed a biphasic pattern of epithelioid and spindle cells including glandular differentiation. The tumour was positive for vimentin and SS18-SSX, and the spindle cells were positive for bcl-2; in contrast, the epithelioid tumour cells were positive for pan-cytokeratin, epithelial membrane antigen and CD99. There was no INI-loss in tumour cells. Then, the presence of the SYT-SSX gene fusion was demonstrated by fluorescence in situ hybridization. In addition, androgen receptor gene somatic mutations were detected by next-generation sequencing. However, 6 months postoperatively, the patient had neither developed a recurrence nor received adjuvant radiotherapy and chemotherapy. Accurate diagnosis depends on morphological and immunohistochemical examination and a proper molecular analysis, and novel technologies can detect a wide variety of genetic alterations. Although androgen receptor somatic mutations cannot provide addition treatment at present, surgical resection with a clean margin and follow-up is an appropriate approach.

PMID:35024148 | PMC:PMC8743932 | DOI:10.1177/2050313X211068646

Use of 68Ga-FAPI PET/CT for Detecting Myeloid Sarcoma of the Breast and Assessing Early Response to Chemotherapy

Fetched: January 14th, 2022, 5:01am GMT by Simin Wu

Clin Nucl Med. 2022 Jan 12. doi: 10.1097/RLU.0000000000004028. Online ahead of print.

ABSTRACT

Myeloid sarcoma is a rare presentation of acute myeloid leukemia as a solid tumor at various extramedullary sites. In this case, we report 18F-FDG and 68Ga-FAPI PET/CT findings in an 18-year-old woman with biopsy-proven myeloid sarcoma involving the left breast. 68Ga-FAPI PET/CT showed a far better tumor-to-background contrast than 18F-FDG PET/CT in the left breast mass. Moreover, follow-up 68Ga-FAPI PET/CT after 2 cycles of chemotherapy revealed a reduction in tumor size with decreasing activity in the myeloid sarcoma lesions.

PMID:35025784 | DOI:10.1097/RLU.0000000000004028

Permalink for 'Compression Therapy for HIV-Associated Kaposi Sarcoma Leg Lymphedema: Results of the Kenyan Improvised Compression for Kaposi Sarcoma Randomized Controlled Trial'

Compression Therapy for HIV-Associated Kaposi Sarcoma Leg Lymphedema: Results of the Kenyan Improvised Compression for Kaposi Sarcoma Randomized Controlled Trial

Fetched: January 14th, 2022, 5:01am GMT by Aileen Y Chang

JCO Glob Oncol. 2022 Jan;8:e2100329. doi: 10.1200/GO.21.00329.

ABSTRACT

PURPOSE: Evaluate the effectiveness of compression while receiving chemotherapy compared with chemotherapy alone in the treatment of HIV-associated Kaposi sarcoma (KS) lymphedema.

METHODS: A randomized controlled trial was conducted in a single oncology clinic in western Kenya (NCT03404297). A computer-generated randomization schedule was used to allocate treatment arms. Randomized block design was used for stratification by lymphedema stage. Participants were HIV positive adults age ≥ 18 years on antiretroviral therapy with biopsy-proven KS associated with leg lymphedema and being initiated on chemotherapy. The intervention was 10 weeks of weekly clinic-based application of two-component paste compression bandages. The primary outcome was change in the Lower Extremity Lymphedema Index (LELI) score from week 0 to week 14. The secondary outcomes were change in the Lymphedema Quality of Life measure (LYMQOL) and change in the European Organisation for Research and Treatment of Cancer (EORTC) QLQ-C30 score from week 0 to week 14. Blinded outcome assessments were conducted.

RESULTS: Of 30 participants randomly assigned, 25 eligible patients (chemotherapy [control], n = 13; compression plus chemotherapy [intervention], n = 12) returned at week 14. Change in LELI, LYMQOL, and EORTC QLQ-C30 scores between week 14 and week 0 did not significantly differ by arm. The mean (standard deviation) change in LELI score was -25.9 (34.6) for the control arm compared with -13.3 (29.5) for the intervention arm, P = .340. The difference (95% CI) in the change in LELI score was -12.6 (-39.3 to 14.1).

CONCLUSION: Future studies evaluating a 14-week change in LELI for KS lymphedema should assume a standard deviation of approximately 30. Lessons learned from this pilot trial should inform the development of a larger, multicenter trial to evaluate the effectiveness of compression for KS lymphedema.

PMID:35025687 | DOI:10.1200/GO.21.00329

Deubiquitinase JOSD2 stabilizes YAP/TAZ to promote cholangiocarcinoma progression

Fetched: January 14th, 2022, 5:01am GMT by Meijia Qian

Acta Pharm Sin B. 2021 Dec;11(12):4008-4019. doi: 10.1016/j.apsb.2021.04.003. Epub 2021 Jun 25.

ABSTRACT

Cholangiocarcinoma (CCA) has emerged as an intractable cancer with scanty therapeutic regimens. The aberrant activation of Yes-associated protein (YAP) and transcriptional co-activator with PDZ-binding motif (TAZ) are reported to be common in CCA patients. However, the underpinning mechanism remains poorly understood. Deubiquitinase (DUB) is regarded as a main orchestrator in maintaining protein homeostasis. Here, we identified Josephin domain-containing protein 2 (JOSD2) as an essential DUB of YAP/TAZ that sustained the protein level through cleavage of polyubiquitin chains in a deubiquitinase activity-dependent manner. The depletion of JOSD2 promoted YAP/TAZ proteasomal degradation and significantly impeded CCA proliferation in vitro and in vivo. Further analysis has highlighted the positive correlation between JOSD2 and YAP abundance in CCA patient samples. Collectively, this study uncovers the regulatory effects of JOSD2 on YAP/TAZ protein stabilities and profiles its contribution in CCA malignant progression, which may provide a potential intervention target for YAP/TAZ-related CCA patients.

PMID:35024322 | PMC:PMC8727894 | DOI:10.1016/j.apsb.2021.04.003

Proximal-type epithelioid sarcoma in pubic region expressing L-type amino acid transporter 1: A case report

Fetched: January 14th, 2022, 5:01am GMT by Shunsuke Yahiro

SAGE Open Med Case Rep. 2022 Jan 8;10:2050313X211067917. doi: 10.1177/2050313X211067917. eCollection 2022.

ABSTRACT

Proximal-type epithelioid sarcoma is an aggressive malignant soft-tissue neoplasm, a "proximal" variant of epithelioid sarcoma, resistant to multimodal therapy and involved in early tumor-related death. Pertinent treatments are, therefore, continually being explored. A 24-year-old woman with nonmetastatic proximal-type epithelioid sarcoma, originating subcutaneously on the right side of the vulva, underwent surgical resection; the lesion recurred, however, leading to death 3 months after the second surgery. Here described is a case of proximal-type epithelioid sarcoma expressing L-type amino acid transporter 1 (LAT1) that transports essential amino acids and p-borono-L-phenylalanine (BPA)-the chemical compound used in boron neutron capture therapy (BNCT)-and is highly expressed in many malignant tumors. Recently, LAT1 has drawn attention, and relevant treatments have been studied-LAT1 inhibitor and BNCT. LAT1 expression in proximal-type epithelioid sarcoma may lead to cogent treatments for the disease.

PMID:35024147 | PMC:PMC8743976 | DOI:10.1177/2050313X211067917

Permalink for 'Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature'

Primary spinal multifocal intradural-extramedullary Ewing sarcoma in children: presentation of a case and review of the literature

Fetched: January 14th, 2022, 5:01am GMT by Eren Müngen

Turk J Pediatr. 2021;63(6):1084-1090. doi: 10.24953/turkjped.2021.06.018.

ABSTRACT

BACKGROUND: Primary spinal, intradural, extramedullary Ewing sarcoma (PSIEES) is exceptionally uncommon. Here, we present an interesting pediatric case with a PSIEES diagnosis confirmed by the presence of a specific fusion protein in the tumor tissue and who then developed a cerebellar recurrence. We also reviewed the PSIEES cases in childhood reported in the literature.

CASE: An 8.5-year-old boy was admitted to a local hospital with a one-month history of severe back and limb pain, and inability to move his lower limbs. Physical examination revealed paraparesis in the lower extremities. Spinal MRI revealed multiple intradural extramedullary masses at the L2-L3, L4-5 and L5-S1 levels. He underwent surgery and near total excision of all three masses were performed. Histopathological diagnosis of Ewing Sarcoma was confirmed with EWS-ERG gene rearrangement. The patient was treated according to EuroEwing chemotherapy protocol. A total dose of 4500 cGy radiotherapy was applied to the tumor location at L2-S1 paravertebral region. Eighteen months after the end of treatment, a mass in the left cerebellar hemisphere was determined. Gross total excision was performed. Histopathological examination of the tumor showed Ewing sarcoma. Radiological screening revealed isolated central nervous system recurrence. A total of 4500 cGy radiotherapy was applied. He is on a second-line treatment consisting of gemcitabine and docetaxel without any evidence of disease.

CONCLUSIONS: Ewing Sarcoma with spinal intradural region in childhood is very rare. We could only find 17 pediatric cases reported in the literature. Neurological findings occur earlier in tumors of this region. The prognosis is worse than other extraosseous Ewing sarcoma.

PMID:35023660 | DOI:10.24953/turkjped.2021.06.018

Permalink for 'Re-irradiation of canine non-lymphomatous nasal tumors using stereotactic radiation therapy (10 Gy x 3) for both courses: assessment of outcome and toxicity in 11 dogs'

Re-irradiation of canine non-lymphomatous nasal tumors using stereotactic radiation therapy (10 Gy x 3) for both courses: assessment of outcome and toxicity in 11 dogs

Fetched: January 14th, 2022, 5:01am GMT by Tracy Gieger

Vet Comp Oncol. 2022 Jan 13. doi: 10.1111/vco.12801. Online ahead of print.

ABSTRACT

No uniformly beneficial treatments exist for dogs with non-lymphomatous nasal tumors (NLNT) that relapse after radiotherapy (RT). Reirradiation may prolong survival and improve quality of life. In this retrospective study, we describe outcomes for eleven dogs that had CT-confirmed locoregional progression of NLNT after an initial course of stereotactic RT (SRT#1; 10 Gy x 3) and were then re-treated with the same type of protocol (SRT#2, also 10 Gy x 3). The median time between SRT #1 and SRT #2 was 243 days (95% CI: 78-385 days). Ten dogs (91%) had a clinical benefit after SRT#1; five dogs (45%) had clinical benefit after SRT#2. Adverse events after SRT#2 included nasocutaneous or oronasal fistula formation (N = 3 at 180, 270, and 468 days), seizures (N = 2 at 78 and 330 days), bacterial or fungal rhinitis (N = 2 at 240 and 385 days), and facial swelling (N = 1 at 90 days). All 11 dogs have died, due to disease progression, presumed radiotoxicity, or declining quality of life; in most cases, it was difficult to discern between these conditions. The median overall survival time (OST) from SRT#1 was 745 days (95% CI: 360-1132). The median overall survival time (OST) from SRT #2 was 448 days (95% CI: 112-626). For these dogs, survival was prolonged, but adverse events after SRT#2 were common (8/11; 73%). Therefore, before consenting to re-irradiation with this protocol, pet owners should be counseled about survivorship challenges, including risk for severe toxicities, and persistence of clinical signs.

PMID:35023604 | DOI:10.1111/vco.12801

Immediate tendon transfer for functional reconstruction of a dorsal forearm defect after sarcoma resection

Fetched: January 14th, 2022, 5:01am GMT by Ryo Karakawa

J Plast Surg Hand Surg. 2022 Jan 13:1-6. doi: 10.1080/2000656X.2021.2024556. Online ahead of print.

ABSTRACT

In the treatment of sarcoma, the reconstructive surgeon must consider not only limb salvage but also functional reconstruction. The aim of this study was to evaluate a functional reconstruction of a dorsal forearm defect after sarcoma resection using immediate tendon transfer. Patients who underwent reconstruction of a dorsal forearm defect after sarcoma resection with an immediate tendon transfer between 1997 and 2019 at our hospital were included in this retrospective study. Patient demographics, tumor characteristics, surgical characteristics and functional outcomes were examined. Nine patients were included in this study. Tendon transfer of the flexor carpi radialis (FCR) or the flexor carpi ulnaris (FCU) to the extensor digitorum communis (EDC), the brachioradialis (BR) to the EDC, and the palmaris longus (PL) tendon to the extensor pollicis longus (EPL) was performed in seven, two and five patients, respectively. Seven patients underwent reconstruction using a free flap. Neither anastomosis complications nor infections were encountered. Partial flap necrosis and donor site dehiscence were seen in one case each. The mean distal interphalangeal (DIP), proximal interphalangeal (PIP) and metacarpophalangeal (MP) joint active extension were 4.4°, 6.1° and 11.1° postoperatively. The mean Musculoskeletal Tumor Society (MSTS) score was 26. Immediate tendon transfers of the FCR or the FCU to the EDC and the PL tendon to the EPL can be considered an optimal functional reconstruction of a dorsal forearm defect after sarcoma resection.

PMID:35023432 | DOI:10.1080/2000656X.2021.2024556

What is the clinical impact of staging and surveillance PET-CT scan findings in patients with bone and soft tissue sarcoma?

Fetched: January 14th, 2022, 5:01am GMT by Linus Lee

J Surg Oncol. 2022 Jan 13. doi: 10.1002/jso.26789. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Positron emission tomography-computerized tomography (PET-CTs) are becoming increasingly utilized in sarcoma care, workup, and surveillance. This study aimed to describe additional PET-CT findings as well as subsequent workups and changes in the clinical course due to those results.

METHODS: Patient records were retrospectively reviewed, and the additional workups and evaluations triggered by PET-CT findings were qualitatively analyzed to document their results. Additional changes in the clinical course were documented.

RESULTS: A total of 183 bone and soft tissue sarcoma patients underwent PET-CT as part of staging or surveillance. Additional workup was performed in 31.5% (n = 41 of 130) patients who had positive PET-CT findings. Among these, 36.6% (n = 15 of 41) patients had clinically significant findings that altered the clinical course. Overall, 14.8% (n = 27 of 183) experienced a change in the clinical course due to PET-CT.

CONCLUSION: PET-CT often highlights lesions of potential clinical importance. Additional workup, as well as changes in the clinical course, were not infrequent. Future, multi-institutional studies should address the value of PET-CT in sarcoma care.

PMID:35023167 | DOI:10.1002/jso.26789

Effect of Preoperative Treatment on the Performance of Predictive Nomograms in Primary Retroperitoneal Sarcoma

Fetched: January 14th, 2022, 5:01am GMT by Deanna Ng

Ann Surg Oncol. 2022 Jan 13. doi: 10.1245/s10434-021-11156-x. Online ahead of print.

ABSTRACT

BACKGROUND: Retroperitoneal sarcoma (RPS)-specific nomograms provide estimates of survival and recurrence risk following resection in the individual patient. The effect of preoperative treatment on nomogram performance has not been previously examined. Our aim was to evaluate the predictive accuracy of existing RPS-specific nomograms in patients managed at our center, where the majority of patients received preoperative radiation.

PATIENTS AND METHODS: All patients who underwent curative treatment for primary RPS at Mount Sinai Hospital/Princess Margaret Hospital between 1996 and 2016 were identified. The performance of four previously published nomograms was assessed by measuring the agreement between nomogram-predicted and observed outcomes using Harrell's C-Index and level of calibration. Outcomes included in each of the nomograms [overall survival (OS), disease-free survival (DFS), disease-specific death (DSD), local recurrence (LR), distant recurrence (DR)] at each of the specified post-resection timepoints were examined.

RESULTS: In total, 253 patients were included. When observed outcomes were compared with those predicted by each of the four nomograms, the C-Index ranged from 0.60 to 0.81, representing a wide range of predictive accuracy. The lowest C-Index was for prediction of LR. Calibration plots revealed that the Memorial Sloan Kettering Cancer Center (MSKCC) nomogram predicted a 5-year LR of 45%, whereas the observed LR was 24%. Overprediction of LR was detected in patients who had undergone preoperative radiotherapy, but not in patients treated with surgery alone.

CONCLUSIONS: Preoperative radiotherapy appeared to preclude the use of the LR component of existing nomograms for primary RPS. Updated nomograms should be created to reflect this variable, particularly in light of the recently published STRASS trial results.

PMID:35022902 | DOI:10.1245/s10434-021-11156-x

Leiomyosarcoma of inferior vena cava (IVC): do we really need to reconstruct IVC post resection? Single institution experience

Fetched: January 14th, 2022, 5:01am GMT by Mahesh Goel

Langenbecks Arch Surg. 2022 Jan 13. doi: 10.1007/s00423-021-02408-1. Online ahead of print.

ABSTRACT

BACKGROUND: Inferior vena cava (IVC) leiomyosarcomas (LMS) are a rare group of retroperitoneal tumors. R0 surgical resection is the only curative modality of treatment. IVC resection for retroperitoneal sarcoma is a complex surgery with no definitive guidelines for reconstruction.

METHODS: Retrospective review of all patients who underwent surgical resection of primary leiomyosarcoma of the IVC requiring resection from 2010 to 2020 at our tertiary care center was performed.

RESULTS: Among 24 patients who required IVC resection for LMS, only 7 (29%) required reconstruction of IVC. According to Clavien-Dindo classification, there was one grade 3 or more morbidity and 1 post-operative mortality. Seventeen patients underwent R0 resection whereas 7 patients had R1 resection on final histopathology. At a median follow-up of 25 months (range 8-91 months), the median OS was 40 months with median DFS of 28 months. Two patients presented with local recurrence while 13 patients developed systemic recurrence on follow-up.

CONCLUSION: Careful preoperative multidisciplinary planning can make IVC resection without reconstruction feasible with acceptable perioperative morbidity, mortality, and oncological outcomes for IVC LMS.

PMID:35022833 | DOI:10.1007/s00423-021-02408-1

Phase I study of pegylated liposomal doxorubicin and cisplatin in patients with advanced osteosarcoma

Fetched: January 14th, 2022, 5:01am GMT by Xi-Zhi Wen

Cancer Chemother Pharmacol. 2022 Jan 13. doi: 10.1007/s00280-021-04371-6. Online ahead of print.

ABSTRACT

PURPOSE: The repeated use of doxorubicin is limited due to dose-limiting cardiac toxicity. Pegylated liposomal doxorubicin (PEG-LD, Duomeisu) has a reduced cardiac toxicity. This phase I study aimed to investigate the maximum tolerated doses (MTDs) and dose-limiting toxicities (DLTs) of the PEG-LD and cisplatin combination in patients with metastatic and recurrent osteosarcoma.

METHODS: Patients were given PEG-LD at a dose of 40, 50, or 60 mg/m² on day 1 of each 21-day cycle, according to a 3 + 3 approach for dose escalation. Cisplatin was administered as a fixed dose of 100 mg/m² for every cycle. Toxicities and tumor response were observed.

RESULTS: A total of 15 patients were enrolled in this trial, and nine of the patients had received prior doxorubicin. The MTD of PEG-LD was reached at 50 mg/m² in this regimen, with neutropenic fever and stomatitis as DTLs. The main adverse event (AE) was myelosuppression. The most common non-hematological AEs were vomiting, hypoproteinemia, stomatitis and transient sinus arrhythmia. Grade 3-4 toxicity was neutropenia, leukopenia, thrombocytopenia, anemia and stomatitis in the whole cohort. All the AEs were relieved after symptomatic and supportive treatment. Totally, the overall response rate was 13.3% and disease control rate was 66.7%. For the six patients who have not received prior doxorubicin, one partial response and five stable diseases were observed.

CONCLUSION: We provide the data showing that PEG-LD 50 mg/m² combined with cisplatin 100 mg/m² demonstrated an acceptable safety profile and promising clinical activity in advanced osteosarcoma, which merits further evaluation in phase II studies.

TRIAL REGISTRATION: ChiCTR1900021550.

PMID:35022817 | DOI:10.1007/s00280-021-04371-6

Permalink for 'Impact of Bacillus Calmette-Guérin (BCG) vaccination on postoperative mortality in patients with perioperative SARS-CoV-2 infection'

Impact of Bacillus Calmette-Guérin (BCG) vaccination on postoperative mortality in patients with perioperative SARS-CoV-2 infection

Fetched: January 14th, 2022, 5:01am GMT by COVID Surg Collaborative

BJS Open. 2021 Nov 9;5(6):zrab131. doi: 10.1093/bjsopen/zrab131.

ABSTRACT

There is little evidence around the potentially protective role of previous Bacillus Calmette-Guerin (BCG) vaccination on postoperative mortality in patients with perioperative SARS-CoV-2 vaccination. Prior BCG vaccination did not protect SARS-CoV-2 infected patients against postoperative pulmonary complications and 30-day mortality.

PMID:35022673 | PMC:PMC8756090 | DOI:10.1093/bjsopen/zrab131

Permalink for 'BAMM (BRAF Autophagy and MEK inhibition in Melanoma): A phase I/II trial of dabrafenib, trametinib and hydroxychloroquine in advanced BRAFV600-mutant melanoma'

BAMM (BRAF Autophagy and MEK inhibition in Melanoma): A phase I/II trial of dabrafenib, trametinib and hydroxychloroquine in advanced BRAFV600-mutant melanoma

Fetched: January 14th, 2022, 5:01am GMT by Janice M Mehnert

Clin Cancer Res. 2022 Jan 12:clincanres.3382.2021. doi: 10.1158/1078-0432.CCR-21-3382. Online ahead of print.

ABSTRACT

PURPOSE: Autophagy is a resistance mechanism to BRAF/MEK inhibition in BRAFV600-mutant melanoma. Here we used hydroxychloroquine (HCQ) to inhibit autophagy in combination with dabrafenib 150 mg bid and trametenib 2 mg qd (D+T).

PATIENTS AND METHODS: We conducted a phase I/II clinical trial in 4 centers of HCQ+D+T in advanced BRAFV600-mutant melanoma patients. The primary objectives were the recommended phase II dose (RP2D) and the one-year progression-free survival (PFS) rate of >53%.

RESULTS: 34 were evaluable for one-year PFS rate. Patient demographics: elevated LDH: 47%; Stage IV M1c/M1d: 52%; prior immunotherapy: 50%. In phase I, there was no dose limiting toxicity. HCQ 600 mg po bid with D+T was the RP2D. The one-year PFS rate was 48.2% (95% CI = 31.0-65.5%), median PFS was 11.2 months (95% CI = 5.4-16.9 months), and response rate (RR) was 85% (95% CI=64-95%). The complete response rate was 41% and median overall survival (OS) was 26.5 months. In patient with elevated LDH (n=16), the RR was 88% and median PFS and OS were 7.3 and 22 months, respectively.

CONCLUSION: HCQ+D+T was well tolerated and produced a high RR but did not meet criteria for success for the one-year PFS rate. There was a high proportion of pre-treated and elevated LDH patients, an increasingly common demographic in patients receiving targeted therapy. In this difficult to treat population, the the RR and PFS were encouraging. A randomized trial of D+T+HCQ or placebo in BRAFV600-mutant melanoma patients with elevated LDH and previous immunotherapy is being conducted.

PMID:35022320 | DOI:10.1158/1078-0432.CCR-21-3382

The role of oncovascular surgery in gynecologic oncology surgery

Fetched: January 14th, 2022, 5:01am GMT by Matias Jurado

Int J Gynecol Cancer. 2022 Jan 12:ijgc-2021-003129. doi: 10.1136/ijgc-2021-003129. Online ahead of print.

ABSTRACT

Oncovascular surgery is a new term used to define tumor resection with simultaneous reconstruction of the great vessels when the tumor infiltrates or firmly adheres to such vessels. The benefit of oncovascular surgery has been widely described in patients with hepato-biliary-pancreatic cancers, retroperitoneal soft tissue sarcoma, and in other areas of gynecologic oncology, such as the lateral compartment of the pelvis, retroperitoneum, and hepato-biliary-pancreatic region, with an increase in complete resections and without increasing the morbidity and mortality rates. In the latter decades of the past century, several advances and accumulating scientific evidence led gynecologic oncologists to perform more thorough cytoreductive surgeries that included multivisceral resections. But to our knowledge, published studies on the frequency and relevance of vascular surgery in gynecological oncology are scarce. Gynecologic oncologists still do not receive formal training in vascular surgery and additionally, with the current reduction in experience with pelvic and para-aortic lymphadenectomy, as well as other types of radical abdominal and pelvic surgeries, trainees will encounter fewer vascular injuries and the opportunity to deal with a variety of management types required. Well-organized collaboration between each subspecialty with a multidisciplinary approach and adequate pre-operative planning are pivotal. The aim of this review is to pave the way towards the understanding that patients with suspicion of great vessels' infiltration or encasement by tumor require personalized and specialized treatment with the need to form an oncovascular surgery team, and that it is necessary for gynecologic oncology surgeons to take a step forward in surgical training.

PMID:35022310 | DOI:10.1136/ijgc-2021-003129

PET/MR fusion texture analysis for the clinical outcome prediction in soft-tissue sarcoma

Fetched: January 14th, 2022, 5:01am GMT by Wenzhe Zhao

Cancer Imaging. 2022 Jan 12;22(1):7. doi: 10.1186/s40644-021-00438-y.

ABSTRACT

BACKGROUND: Various fusion strategies (feature-level fusion, matrix-level fusion, and image-level fusion) were used to fuse PET and MR images, which might lead to different feature values and classification performance. The purpose of this study was to measure the classification capability of features extracted using various PET/MR fusion methods in a dataset of soft-tissue sarcoma (STS).

METHODS: The retrospective dataset included 51 patients with histologically proven STS. All patients had pre-treatment PET and MR images. The image-level fusion was conducted using discrete wavelet transformation (DWT). During the DWT process, the MR weight was set as 0.1, 0.2, 0.3, 0.4, …, 0.9. And the corresponding PET weight was set as 1- (MR weight). The fused PET/MR images was generated using the inverse DWT. The matrix-level fusion was conducted by fusing the feature calculation matrix during the feature extracting process. The feature-level fusion was conducted by concatenating and averaging the features. We measured the predictive performance of features using univariate analysis and multivariable analysis. The univariate analysis included the Mann-Whitney U test and receiver operating characteristic (ROC) analysis. The multivariable analysis was used to develop the signatures by jointing the maximum relevance minimum redundancy method and multivariable logistic regression. The area under the ROC curve (AUC) value was calculated to evaluate the classification performance.

RESULTS: By using the univariate analysis, the features extracted using image-level fusion method showed the optimal classification performance. For the multivariable analysis, the signatures developed using the image-level fusion-based features showed the best performance. For the T1/PET image-level fusion, the signature developed using the MR weight of 0.1 showed the optimal performance (0.9524(95% confidence interval (CI), 0.8413-0.9999)). For the T2/PET image-level fusion, the signature developed using the MR weight of 0.3 showed the optimal performance (0.9048(95%CI, 0.7356-0.9999)).

CONCLUSIONS: For the fusion of PET/MR images in patients with STS, the signatures developed using the image-level fusion-based features showed the optimal classification performance than the signatures developed using the feature-level fusion and matrix-level fusion-based features, as well as the single modality features. The image-level fusion method was more recommended to fuse PET/MR images in future radiomics studies.

PMID:35022071 | DOI:10.1186/s40644-021-00438-y

Permalink for 'Nanoparticle albumin-bound paclitaxel and PD-1 inhibitor (sintilimab) combination therapy for soft tissue sarcoma: a retrospective study'

Nanoparticle albumin-bound paclitaxel and PD-1 inhibitor (sintilimab) combination therapy for soft tissue sarcoma: a retrospective study

Fetched: January 14th, 2022, 5:01am GMT by Zhichao Tian

BMC Cancer. 2022 Jan 12;22(1):56. doi: 10.1186/s12885-022-09176-1.

ABSTRACT

BACKGROUND: There is increasing evidence that combination therapy with nanoparticle albumin-bound paclitaxel (nab-paclitaxel) and programmed cell death protein 1 (PD-1) inhibitor is safe and efficacious in treating many types of malignant tumors. However, clinical data demonstrating the effect of this treatment combination for patients with metastatic soft tissue sarcoma (STS) are currently limited.

METHODS: The clinical data of patients with metastatic STS who received nab-paclitaxel plus PD-1 inhibitor (sintilimab) therapy between January 2019 and February 2021 were retrospectively analyzed. The effectiveness and safety of the combined treatment were evaluated in terms of the median progression-free survival (PFS), estimated using the Kaplan-Meier method. The univariate Cox proportional hazards model was used to analyze the relationship between clinicopathological parameters and PFS. All statistical analyses were two-sided; P < 0.05 was considered statistically significant.

RESULTS: A total of 28 patients treated with nab-paclitaxel plus sintilimab were enrolled in this study. The objective response rate was 25%, the disease control rate was 50%, and the median PFS was 2.25 months (95% CI = 1.8-3.0 months). The most common grade 1 or 2 adverse events (AEs) were alopecia (89.3%; 25/28), leukopenia (25.0%; 7/28), fatigue (21.4%; 6/28), anemia (21.4%; 6/28), and nausea (21.4%; 6/28). The most common grade 3 AEs were neutropenia (10.7%; 3/28) and peripheral neuropathy (10.7%; 3/28). No grade 4 AEs were observed. Among the present study cohort, patients with angiosarcoma (n = 5) had significantly longer PFS (P = 0.012) than patients with other pathological subtypes, including undifferentiated pleomorphic sarcoma (n = 7), epithelioid sarcoma (n = 5), fibrosarcoma (n = 4), synovial sarcoma (n = 3), leiomyosarcoma (n = 2), pleomorphic liposarcoma (n = 1), and rhabdomyosarcoma (n = 1); those who experienced three or more AEs had significantly longer median PFS than those who experienced less than three AEs (P = 0.018).

CONCLUSION: Nab-paclitaxel plus PD-1 inhibitor is a promising treatment regimen for advanced STS. Randomized controlled clinical trials are required to further demonstrate its efficacy and optimal application scenario.

PMID:35022029 | DOI:10.1186/s12885-022-09176-1

Permalink for 'Pembrolizumab Achieves a Complete Response in an NF-1 Mutated, PD-L1 Positive Malignant Peripheral Nerve Sheath Tumor: A Case Report and Review of the Benchmarks'

Pembrolizumab Achieves a Complete Response in an NF-1 Mutated, PD-L1 Positive Malignant Peripheral Nerve Sheath Tumor: A Case Report and Review of the Benchmarks

Fetched: January 13th, 2022, 5:02am GMT by Kristian Larson

J Immunother. 2022 Jan 12. doi: 10.1097/CJI.0000000000000410. Online ahead of print.

ABSTRACT

Malignant peripheral nerve sheath tumors (MPNSTs) represent a rare subtype of neural crest cell-derived soft tissue sarcomas (STS). Standard of care therapy comprises surgical resection followed by adjuvant radiation, and most clinical studies have demonstrated finite survival benefit of radiation and chemotherapy. In metastatic disease, palliative chemotherapy provides very limited efficacy. We report a 60-year-old male patient with a primary para vertebral tumor at T7-T8 with lung metastases who recurred after surgical resection and later progressed on epirubicin plus ifosfamide. He was an international patient and referred to the phase 1 clinic. Molecular profiling and immunohistochemistry of the tumor revealed a PD-L1 expression of 70% (2+) and pathogenic genetic alterations by next-generation sequencing in ARID1A, CDKN2A, KMT2A, NF1, and TP53. Immune checkpoint therapy (ICT) with pembrolizumab was commenced, and interval computed tomography revealed a complete remission by cycle 6. Randomized clinical trials illustrate that ICTs such as anti-PD-1 and anti-CTLA4 monoclonal antibodies in STS cohorts display low or modest response rates by variable PD-L1 expression. This and 3 other case reports of disparate PD-L1 expression demonstrate complete responses in PD-L1 positive MPNSTs treated with ICT. These case reports necessitate further study of ICT in neural crest cell subtype of STS.

PMID:35020691 | DOI:10.1097/CJI.0000000000000410

Tubercle-Sparing Proximal Tibial Reconstruction in Patients with Primary and Metastatic Bone Disease: A Case Report

Fetched: January 13th, 2022, 5:02am GMT by Mitchell S Fourman

JBJS Case Connect. 2022 Jan 12;12(1). doi: 10.2106/JBJS.CC.21.00483.

ABSTRACT

CASE: Two patients with cancer involving their proximal tibia required proximal tibial replacement (PTR). One had a soft-tissue sarcoma that involved her posterior cortex, and the other had extensive metaphyseal destruction from metastatic breast cancer. Their anterolateral cortex and tibial tubercle were uninvolved, permitting tubercle-sparing PTR. A plate was applied to the bone bridge in the latter patient in anticipation of radiotherapy. Both healed uneventfully and had minimal extensor lag 2 weeks postoperatively.

CONCLUSION: Tubercle-sparing PTR preserves extensor mechanism function with minimal lag. It should be considered in patients with cancer when sparing the anterolateral cortex is oncologically safe.

PMID:35020626 | DOI:10.2106/JBJS.CC.21.00483

ASO Author Reflections: Outcome Improvement Over Time in Extremity and Trunk Wall Soft Tissue Sarcomas: To Whom the Merit?

Fetched: January 13th, 2022, 5:02am GMT by Maria Danieli

Ann Surg Oncol. 2022 Jan 12. doi: 10.1245/s10434-022-11333-6. Online ahead of print.

NO ABSTRACT

PMID:35020095 | DOI:10.1245/s10434-022-11333-6

Diagnostic performance of MRI in detecting locally recurrent soft tissue sarcoma: systematic review and meta-analysis

Fetched: January 13th, 2022, 5:02am GMT by Robert M Kwee

Eur Radiol. 2022 Jan 12. doi: 10.1007/s00330-021-08457-w. Online ahead of print.

ABSTRACT

OBJECTIVE: To systematically review the diagnostic criteria and performance of MRI in detecting locally recurrent soft tissue sarcoma.

METHODS: Medline and Embase were searched for original studies on the diagnostic performance of MRI detecting locally recurrent soft tissue sarcoma. Study quality was assessed using QUADAS-2. Sensitivity and specificity were pooled using a bivariate random-effects model.

RESULTS: Ten studies were included. There was a high risk of bias with respect to patient selection in 2 studies and a high risk of bias with respect to flow and timing in 8 studies. The presence of a mass yielded a pooled sensitivity of 80.9% and a pooled specificity of 77.0%. Hyperintensity at T2-weighted imaging yielded a pooled sensitivity of 82.4% and a pooled specificity of 11.0%. Hypo- or isointensity at T1-weighted imaging yielded a pooled sensitivity of 82.0% and a pooled specificity of 14.3%. Contrast enhancement images yielded a pooled sensitivity of 95.9% and a pooled specificity of 12.3%. Low signal mass on the apparent diffusion coefficient (ADC) map yielded a pooled sensitivity of 67.5% and a pooled specificity of 95.3%. Early and rapid arterial phase enhancement at dynamic contrast-enhanced (DCE) MRI yielded a pooled sensitivity of 91.3% and a pooled specificity of 84.7%.

CONCLUSION: The presence of a mass appears a useful criterion to diagnose locally recurrent soft tissue sarcoma. Signal characteristics at standard T2- and T1-weighted imaging and contrast enhancement seem less useful because they lack specificity. Functional MRI techniques, including DWI with ADC mapping and DCE, may help to make a correct diagnosis.

KEY POINTS: • The presence of a mass at MRI appears useful to diagnose locally recurrent soft tissue sarcoma, because both sensitivity and specificity are fairly high. • Signal characteristics at standard T2- and T1-weighted sequences and contrast enhancement suffer from poor specificity. • DWI with ADC mapping and DCE may help to make a correct diagnosis, but further research is needed to better understand the value of these functional MRI techniques.

PMID:35020015 | DOI:10.1007/s00330-021-08457-w

Permalink for 'Caspase-Mediated Cleavage of the Transcription Factor Sp3: Possible Relevance to Cancer and the Lytic Cycle of Kaposi's Sarcoma-Associated Herpesvirus'

Caspase-Mediated Cleavage of the Transcription Factor Sp3: Possible Relevance to Cancer and the Lytic Cycle of Kaposi's Sarcoma-Associated Herpesvirus

Fetched: January 13th, 2022, 5:02am GMT by Li-Yu Chen

Microbiol Spectr. 2022 Jan 12:e0146421. doi: 10.1128/spectrum.01464-21. Online ahead of print.

ABSTRACT

The open reading frame 50 (ORF50) protein of Kaposi's sarcoma-associated herpesvirus (KSHV) is the master regulator essential for initiating the viral lytic cycle. Previously, we have demonstrated that the ORF50 protein can cooperate with Sp3 to synergistically activate a set of viral and cellular gene promoters through highly conserved ORF50-responsive elements that harbor a Sp3-binding motif. Herein, we show that Sp3 undergoes proteolytic cleavage during the viral lytic cycle, and the cleavage of Sp3 is dependent on caspase activation. Since similar cleavage patterns of Sp3 could be detected in both KSHV-positive and KSHV-negative lymphoma cells undergoing apoptosis, the proteolytic cleavage of Sp3 could be a common event during apoptosis. Mutational analysis identifies 12 caspase cleavage sites in Sp3, which are situated at the aspartate (D) positions D17, D19, D180, D273, D275, D293, D304 (or D307), D326, D344, D530, D543, and D565. Importantly, we noticed that three stable Sp3 C-terminal fragments generated through cleavage at D530, D543, or D565 encompass an intact DNA-binding domain. Like the full-length Sp3, the C-terminal fragments of Sp3 could still retain the ability to cooperate with ORF50 protein to activate specific viral and cellular gene promoters synergistically. Collectively, our findings suggest that despite the proteolytic cleavage of Sp3 under apoptotic conditions, the resultant Sp3 fragments may retain biological activities important for the viral lytic cycle or for cellular apoptosis. IMPORTANCE The ORF50 protein of Kaposi's sarcoma-associated herpesvirus (KSHV) is the key viral protein that controls the switch from latency to lytic reactivation. It is a potent transactivator that can activate target gene promoters via interacting with other cellular DNA-binding transcription factors, such as Sp3. In this report, we show that Sp3 is proteolytically cleaved during the viral lytic cycle, and up to 12 caspase cleavage sites are identified in Sp3. Despite the proteolytic cleavage of Sp3, several resulting C-terminal fragments that have intact zinc-finger DNA-binding domains still retain substantial influence in the synergy with ORF50 to activate specific gene promoters. Overall, our studies elucidate the caspase-mediated cleavage of Sp3 and uncover how ORF50 utilizes the cleavage fragments of Sp3 to transactivate specific viral and cellular gene promoters.

PMID:35019687 | DOI:10.1128/spectrum.01464-21

Permalink for 'Distinctive clinical and pathologic features of immature teratomas arising from induced Pluripotent Stem Cell-derived beta cell injection in a diabetes patient'

Distinctive clinical and pathologic features of immature teratomas arising from induced Pluripotent Stem Cell-derived beta cell injection in a diabetes patient

Fetched: January 13th, 2022, 5:02am GMT by Lei Han

Stem Cells Dev. 2022 Jan 12. doi: 10.1089/scd.2021.0255. Online ahead of print.

ABSTRACT

Induced pluripotent stem cells (iPSCs) are a new potential cure for diabetes, characterised by a capacity for self-renewal and differentiation to pancreatic islet beta cells, which secrete insulin and rebuild blood glucose balance. The safety and validity of iPSC-derived cell therapy for diabetes remain controversial. Teratoma formation arising from undifferentiated stem cells is a serious risk, but clinical reports of this phenomenon are rare. Here, we report a distinctive case of immature teratoma after the patient underwent iPSC-derived cell therapy for diabetes in another hospital, and he wastreated in our soft tissue sarcoma centre. The patient received islet beta cell injection, in which the cells were differentiated from autologous iPSCs, into the deltoid muscle. Two months later, a mass located in the injection area was detected and presented with enlarged axillary lymph nodes. Here, we present the clinical, radiological, and pathological features of this immature teratoma. Distinct from typical immature teratomas, this tumour was characterised by rapid growth and local lymph node metastasis. The tumour did not respond to typical chemotherapy regimens for immature teratomas. Magnetic resonance imaging (MRI) showed heterogeneous enhancement and a rich blood supply to the tumour. Histopathology revealed immature endoderm, mesoderm, and ectoderm tissues composed of osseous, cartilaginous, vascular, and adenoid tissues, which have more cellular atypia than typical teratomas. Staining for both OCT4 and SOX2 was positive in the tumour cell nucleus as revealed by immunofluorescence assay; however, insulin staining was negative. Next-generation sequencing showed many missense mutations, but abnormal gene rearrangement, defects, or changes in copy numbers were not observed. In conclusion, more attention should be given to teratoma formation after iPSC-derived cell therapy for diabetes, because these tumours are more aggressive than typical teratomas. The safety and validity of iPSC-derived cell therapy for diabetes should be explored further in standardised clinical trials.

PMID:35018826 | DOI:10.1089/scd.2021.0255

Synthesis and Evaluation of a Class of Compounds Inhibiting the Growth of Stromal Antigen 2 (STAG2)-Mutant Ewing Sarcoma Cells

Fetched: January 13th, 2022, 5:02am GMT by Scott Richard Gilbertson

ChemMedChem. 2022 Jan 11. doi: 10.1002/cmdc.202100653. Online ahead of print.

ABSTRACT

STAG2 (SA2) is a critical component of the cohesin complex that regulates gene expression and the separation of sister chromatids in cells. Mutations in STAG2 have been identified in over thirty different types of cancers including non-small cell lung, bladder and Ewing sarcoma. Selectively inhibiting cancer cells lacking of STAG2 is an attractive approach for the cancer therapy. Here we report that a small molecule, StagX1, identified through a high-throughput screening, inhibits the growth of Ewing sarcoma cells possessing mutant STAG2 . A new synthetic route to the StagX1 scaffold and new versions of the molecule along with their activity in a cell viability assay are reported.

PMID:35018729 | DOI:10.1002/cmdc.202100653

Highly vascularized uterine myomas (uterine smooth muscle tumors) on ultrasound and correlation to histopathology

Fetched: January 13th, 2022, 5:02am GMT by C Russo

Ultrasound Obstet Gynecol. 2022 Jan 12. doi: 10.1002/uog.24855. Online ahead of print.

ABSTRACT

OBJECTIVES: Since differential diagnosis in ultrasound (US) imaging of myometrial lesions can be challenging particularly in the highly vascularized ones, we aimed to correlate the ultrasound appearance of highly vascularized uterine myomas with the histopathological diagnosis.

METHODS: We recruited patients with a preoperative US-diagnosis of highly vascularized uterine myoma characterized by circumferential and intralesional vascular pattern (color score assessment of 3 or 4, according to the Morphological Uterus Sonographic Assessment), who underwent myomectomy or hysterectomy. Additional US-characteristics including cystic area, echogenicity, borders, shadowing, and size were recorded for each patient at baseline. US features were matched with histological characteristics, namely the mitotic count and the presence of cellular atypia and/or coagulative necrosis, for each patient.

RESULTS: 70 patients with highly vascularized uterine myomas were included; mean (± S.D.) age 46.5 years (46.5 ± 11.4), and 13 (18.5%) were post menopause. At histological examination, 93% of cases were benign lesions (32 myomas were typical leiomyoma, 29 were variants of leiomyoma, and 4 were adenomyomas). Whereas 7% were malignant masses (2 uterine sarcomas, 1 leiomyosarcoma, 1 neuroendocrine tumor, 1 uterine smooth muscle tumors of uncertain malignant potential: STUMP). Ultrasound demonstrated cystic areas within the lesion in 10/32 typical leiomyoma, in 16/29 variants of leiomyoma, in all adenomyomas, and in the STUMP and the leiomyosarcoma patients. Borders were regular in 64/65 benign lesions, and 2/5 malignant lesions (p<0.05). No significant differences in terms of echostructure, shadowing and size were found between the two groups. Endometrium was visible in 55/65 benign lesions and 2/5 malignant lesions (p=0.458). The age of patients with malignant lesions was higher than those with benign lesions (64.8±16.0 vs 42.4±5.1; p<0.001) and all were over 45 years.

CONCLUSION: Despite the low number of high vascularized myomas observed, our results showed that ultrasound features of myomas, such as circumferential and intralesional vascularity, cystic areas, and dimensions are important parameters, especially when combined with the patient's age. Such features could be useful to differentiate typical myomas from benign variants and malignant lesions in a pre-operative setting and to select patients that may benefit from a conservative management rather than surgery. This article is protected by copyright. All rights reserved.

PMID:35018681 | DOI:10.1002/uog.24855

Pleomorphic Sarcoma of the Oral Cavity: A Rare Case Entity

Fetched: January 13th, 2022, 5:02am GMT by Sharada T Rajan

J Pharm Bioallied Sci. 2021 Nov;13(Suppl 2):S1747-S1749. doi: 10.4103/jpbs.jpbs_197_21. Epub 2021 Nov 10.

ABSTRACT

Pleomorphic sarcomas are extremely rare in the oral cavity, representing only 5% of all soft tissue sarcomas in the extremities of adults. They form a group of highly undifferentiated pleomorphic malignant tumors. A 36-year-old male reported to the OP with a chief complaint of a tooth ache in the right lower quadrant for the past 6 months. The patient gave a history of extraction of a mobile tooth in that region. A radiograph of the region was advised. An incisional biopsy was also done and sent for histopathological examination. The microscopy revealed the presence of spindle-shaped cells showing dysplastic features. A panel of immunohistochemical markers were performed to identify the tissue of origin of the lesion. The article details the clinical, radiographic, and microscopic features of a pleomorphic soft tissue lesion along with the array of immunohistochemical markers, leading to the final diagnosis of such enigmatic lesions.

PMID:35018069 | PMC:PMC8686877 | DOI:10.4103/jpbs.jpbs_197_21

APOBEC mediated mutagenesis drives genomic heterogeneity in endometriosis

Fetched: January 13th, 2022, 5:02am GMT by Sundaramoorthy Revathidevi

J Hum Genet. 2022 Jan 12. doi: 10.1038/s10038-021-01003-y. Online ahead of print.

ABSTRACT

Endometriosis is a benign gynecologic condition, acting as a precursor of certain histological subtypes of ovarian cancers. The epithelial cells of endometriotic tissues and normal uterine endometrium accumulated somatic mutations in cancer-associated genes such as phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA) and Kirsten rat sarcoma (KRAS) proto-oncogene. To determine the genomic characteristic of endometriotic epithelial cells and normal uterine endometrium and to identify the predominant mutational process acting on them, we studied the somatic mutation profiles obtained from whole exome sequencing of 14 endometriotic epithelium and 11 normal uterine endometrium tissues and classified them into mutational signatures. We observed that single base substitutions 2/13 (SBS), attributed to Apolipoprotein B mRNA Editing Enzyme Catalytic Subunit (APOBEC) induced mutagenesis, were significant in endometriotic tissues, but not in the normal uterine endometrium. Additionally, the larger number and wider allele frequency distribution of APOBEC signature mutations, compared to cancer-associated driver mutations in endometriotic epithelium suggested APOBEC mutagenesis as an important source of mutational burden and heterogeneity in endometriosis. Further, the relative risk of enriched APOBEC signature mutations was higher in endometriosis patients who were carriers of APOBEC3A/3B germline deletion, a common polymorphism in East Asians which involves the complete loss of APOBEC3B coding region. Our results illustrate the significance of APOBEC induced mutagenesis in driving the genomic heterogeneity of endometriosis.

PMID:35017684 | DOI:10.1038/s10038-021-01003-y

Permalink for 'Blockade of beta-adrenergic receptors reduces cancer growth and enhances the response to anti-CTLA4 therapy by modulating the tumor microenvironment'

Blockade of beta-adrenergic receptors reduces cancer growth and enhances the response to anti-CTLA4 therapy by modulating the tumor microenvironment

Fetched: January 13th, 2022, 5:02am GMT by Klaire Yixin Fjæstad

Oncogene. 2022 Jan 11. doi: 10.1038/s41388-021-02170-0. Online ahead of print.

ABSTRACT

The development of immune checkpoint inhibitors (ICI) marks an important breakthrough of cancer therapies in the past years. However, only a limited fraction of patients benefit from such treatments, prompting the search for immune modulating agents that can improve the therapeutic efficacy. The nonselective beta blocker, propranolol, which for decades has been prescribed for the treatment of cardiovascular conditions, has recently been used successfully to treat metastatic angiosarcoma. These results have led to an orphan drug designation by the European Medicines Agency for the treatment of soft tissue sarcomas. The anti-tumor effects of propranolol are suggested to involve the reduction of cancer cell proliferation as well as angiogenesis. Here, we show that oral administration of propranolol delays tumor progression of MCA205 fibrosarcoma model and MC38 colon cancer model and increases the survival rate of tumor bearing mice. Propranolol works by reducing tumor angiogenesis and facilitating an anti-tumoral microenvironment with increased T cell infiltration and reduced infiltration of myeloid-derived suppressor cells (MDSCs). Using T cell deficient mice, we demonstrate that the full anti-tumor effect of propranolol requires the presence of T cells. Flow cytometry-based analysis and RNA sequencing of FACS-sorted cells show that propranolol treatment leads to an upregulation of PD-L1 on tumor associated macrophages (TAMs) and changes in their chemokine expression profile. Lastly, we observe that the co-administration of propranolol significantly enhances the efficacy of anti-CTLA4 therapy. Our results identify propranolol as an immune modulating agent, which can improve immune checkpoint inhibitor therapies in soft tissue sarcoma patients and potentially in other cancers.

PMID:35017664 | DOI:10.1038/s41388-021-02170-0

Permalink for 'Successful chemotherapy with liposomal doxorubicin for metastatic Kaposi's sarcoma without skin involvement in the kidney transplant recipient'

Successful chemotherapy with liposomal doxorubicin for metastatic Kaposi's sarcoma without skin involvement in the kidney transplant recipient

Fetched: January 13th, 2022, 5:02am GMT by Sylwia Kozak

Saudi J Kidney Dis Transpl. 2021 Mar-Apr;32(2):548-553. doi: 10.4103/1319-2442.335469.

ABSTRACT

Kaposi's sarcoma (KS), one of the most typical malignancies after kidney transplantation, is strongly associated with human herpes virus 8 infection. More than 90% of patients had primary skin changes, which make the diagnosis easier and faster. The lack of skin lesions is considered rare, especially in the iatrogenic type of sarcoma, including patients on immunosuppression and may cause a diagnostic challenge due to the variety of organ involvement, imitating other diseases. The aim of this case presentation is to raise attention to the atypical clinical manifestation of this malignancy. Currently, several different therapeutic options are available for patients with KS, including reduction of immunosuppression, conversion of immunosuppression to mTOR inhibitors, or chemotherapy. Here, we present an unusual case of advanced KS human immunodeficiency virus-negative patient after kidney transplantation without primary skin involvement.

PMID:35017351 | DOI:10.4103/1319-2442.335469

Immunologic constant of rejection signature is prognostic in soft-tissue sarcoma and refines the CINSARC signature

Fetched: January 13th, 2022, 5:02am GMT by Francois Bertucci

J Immunother Cancer. 2022 Jan;10(1):e003687. doi: 10.1136/jitc-2021-003687.

ABSTRACT

BACKGROUND: Soft-tissue sarcomas (STSs) are heterogeneous and aggressive tumors, with high metastatic risk. The immunologic constant of rejection (ICR) 20-gene signature is a signature of cytotoxic immune response. We hypothesized that ICR might improve the prognostic assessment of early-stage STS.

METHODS: We retrospectively applied ICR to 1455 non-metastatic STS and searched for correlations between ICR classes and clinicopathological and biological variables, including metastasis-free survival (MFS).

RESULTS: Thirty-four per cent of tumors were classified as ICR1, 27% ICR2, 24% ICR3, and 15% ICR4. These classes were associated with patients' age, pathological type, and tumor depth, and an enrichment from ICR1 to ICR4 of quantitative/qualitative scores of immune response. ICR1 class was associated with a 59% increased risk of metastatic relapse when compared with ICR2-4 class. In multivariate analysis, ICR classification remained associated with MFS, as well as pathological type and Complexity Index in Sarcomas (CINSARC) classification, suggesting independent prognostic value. A prognostic clinicogenomic model, including the three variables, was built in a learning set (n=339) and validated in an independent set (n=339), showing greater prognostic precision than each variable alone or in doublet. Finally, connectivity mapping analysis identified drug classes potentially able to reverse the expression profile of poor-prognosis tumors, such as chemotherapy and targeted therapies.

CONCLUSION: ICR signature is independently associated with postoperative MFS in early-stage STS, independently from other prognostic features, including CINSARC. We built a robust prognostic clinicogenomic model integrating ICR, CINSARC, and pathological type, and suggested differential vulnerability of each prognostic group to different systemic therapies.

PMID:35017155 | DOI:10.1136/jitc-2021-003687

Sarcomas of the extremities and the pelvis: comparing local recurrence after incisional and after core-needle biopsy

Fetched: January 13th, 2022, 5:02am GMT by Alexander Klein

World J Surg Oncol. 2022 Jan 11;20(1):14. doi: 10.1186/s12957-021-02481-2.

ABSTRACT

BACKGROUND: The degree of contamination of healthy tissue with tumor cells during a biopsy in bone or soft tissue sarcomas is clearly dependant on the type of biopsy. Some studies have confirmed a clinically relevant contamination of the biopsy tract after incisional biopsies, as opposed to core-needle biopsies. The aim of our prospective study was to evaluate the risk of local recurrence depending on the biopsy type in extremity and pelvis sarcomas.

METHODS: We included 162 patients with a minimum follow-up of 6 months after wide resection of extremity sarcomas. All diagnostic and therapeutic procedures were performed at a single, dedicated sarcoma center. The excision of the biopsy tract after an incisional biopsy was performed as a standard with all tumor resections. All patients received their follow-up after the conclusion of therapy at our center by means of regional MRI studies and, at a minimum, CT of the thorax to rule out pulmonary metastatic disease. The aim of the study was the evaluation of the influence of the biopsy type and of several other clinical factors on the rate of local recurrence and on the time of local recurrence-free survival.

RESULTS: One hundred sixty-two patients with bone or soft tissue tumors of the extremities and the pelvis underwent either an incisional or a core-needle biopsy of their tumor, with 70 sarcomas (43.2%) being located in the bone. 84.6% of all biopsies were performed as core-needle biopsies. The median follow-up time was 55.6 months, and 22 patients (13.6%) developed a local recurrence after a median time of 22.4 months. There were no significant differences between incisional and core-needle biopsy regarding the risk of local recurrence in our subgroup analysis with differentiation by kind of tissue, grading of the sarcoma, and perioperative multimodal therapy.

CONCLUSIONS: In a large and homogenous cohort of extremity and pelvic sarcomas, we did not find significant differences between the groups of incisional and core-needle biopsy regarding the risk of local recurrence. The excision of the biopsy tract after incisional biopsy in the context of the definitive tumor resection seems to be the decisive factor for this result.

PMID:35016693 | DOI:10.1186/s12957-021-02481-2

Imaging features of inflammatory pseudotumor-like follicular dendritic cell sarcoma of the spleen

Fetched: January 13th, 2022, 5:02am GMT by Nianyu Xue

Ann Palliat Med. 2021 Dec;10(12):12140-12148. doi: 10.21037/apm-21-2776.

ABSTRACT

BACKGROUND: Inflammatory pseudotumor (IPT)-like follicular dendritic cell sarcoma (FDCS) is an extremely rare malignant neoplasm.

METHODS: Retrospective analysis of imaging features of splenic IPT-like FDCS, including ultrasonography, computed tomography (CT), and magnetic resonance (MR) and contrast-enhanced imaging were performed.

RESULTS: When the masses were small, the ultrasound images showed homogeneous hypoechoic signals, clear boundaries, and complete capsules. Abdominal plain CT scans showed equal density (easy to miss diagnosis), unclear boundaries, and no capsules. Magnetic resonance images (MRI) showed slightly shorter T1, slightly shorter T2, and clear boundaries. When the masses were large, the ultrasound images still showed clear boundaries and complete capsules, but the echoes of the masses were not uniform, and some of the masses showed dendritic hyperechoic centers. Abdominal plain CT scans showed irregular low densities in the center (unclear boundaries) and equal densities in the periphery. MRI showed short T1 and T2, but the central signals were mixed. When the mass was accompanied by extensive necrosis, abdominal plain CT scan showed mostly cystic lesions and slight calcifications in low density lesions. Contrast-enhanced CT showed only moderate enhancement in peripheral and septal areas. MRI showed that T1 and T2 were mainly mixed signals. Contrast-enhanced MR showed moderate enhancement of peripheral areas and septum.

CONCLUSIONS: This is the first report to describe the IPT manifestations of the spleen (ultrasonography, CT, and MR). The diagnosis of IPT can be made by combining three imaging features.

PMID:35016402 | DOI:10.21037/apm-21-2776

FDG PET/CT Demonstrated Epstein-Barr Virus-Associated Leiomyosarcoma in a Pediatric Patient With Wiskott-Aldrich Syndrome

Fetched: January 13th, 2022, 5:02am GMT by Cuicui Li

Clin Nucl Med. 2022 Feb 1;47(2):190-191. doi: 10.1097/RLU.0000000000004020.

ABSTRACT

A 7-year-old boy with Wiskott-Aldrich syndrome underwent FDG PET/CT to evaluate hepatic lesions revealed by an ultrasound. The images demonstrated multiple hypermetabolic lesions in the liver, lung, and lymph nodes. Biopsy of liver and lymph nodes demonstrated Epstein-Barr virus-associated leiomyosarcoma.

PMID:35006111 | DOI:10.1097/RLU.0000000000004020

Small-Cell Malignancies of Thyroid: Challenge Solved?

Fetched: January 12th, 2022, 5:01am GMT by Sule Canberk

Acta Cytol. 2022 Jan 11:1-12. doi: 10.1159/000520876. Online ahead of print.

ABSTRACT

"Small-cell malignancies of thyroid" is an unsolved dilemma. This term represents an umbrella terminology in thyroid, encompassing for a small group of tumors in which some of them are well-recognized tumors like medullary thyroid carcinoma, poorly differentiated thyroid carcinoma, and primary thyroid lymphomas and teratoma, whereas the remaining are less known as primary neuroendocrine carcinoma of thyroid, primary extraskeletal Ewing family tumors, and adamantinoma-like Ewing sarcoma. When the issue comes to evaluate a cytological sample predominantly composed of small-cell morphology, metastatic small-cell carcinomas to thyroid also should be excluded. In this review, our group focused on the main cytomorphological and clinical clues of each entity that help to set up a correct differential diagnosis. The literature discussions were also included for the entities that are not yet recognized by the mother publication WHO. A key point of the issue's simple algorithm based on FNAC with small-cell morphology of thyroid was suggested by the authors.

PMID:35016187 | DOI:10.1159/000520876

Osteoblastoma of the Temporal Bone in a Child

Fetched: January 12th, 2022, 5:01am GMT by Micah Gibson

Otol Neurotol. 2022 Feb 1;43(2):e276-e278. doi: 10.1097/MAO.0000000000003381.

ABSTRACT

This report describes osteoblastoma of the temporal bone found on a well-child visit. The relevant clinical, radiographic, and histologic features are described. The tumor was completely resected via a transtemporal approach. The differential diagnosis for these tumors include osteoma, giant cell tumor, histiocytosis, aneurysmal bone cyst and sarcoma. Histologic findings are critical for determining the proper diagnosis.

PMID:35015753 | DOI:10.1097/MAO.0000000000003381

Permalink for 'Cryotherapy Treatment of Cutaneous Kaposi Sarcoma in a Patient With B-Cell Chronic Lymphocytic Leukemia: A Case Report and Short Review of the Literature'

Cryotherapy Treatment of Cutaneous Kaposi Sarcoma in a Patient With B-Cell Chronic Lymphocytic Leukemia: A Case Report and Short Review of the Literature

Fetched: January 12th, 2022, 5:01am GMT by John Doupis

Wounds. 2022 Jan;34(1):E1-E6.

ABSTRACT

INTRODUCTION: Kaposi sarcoma (KS) is a low-grade mesenchymal tumor involving the blood and the lymphatic vessels that primarily effaces the skin and is mediated by human herpesvirus-8 (HHV-8) in more than 90% of patients. There are 4 distinct types of KS. Compared with the classic and AIDS-related variants, chronic lymphocytic leukemia (CLL) associated with KS is a relatively rare clinical condition; thus, only a few cases have been reported.

CASE REPORT: This report presents a case study of an 87-year-old patient with B-cell CLL and cutaneous KS managed with cryotherapy, along with a short review of the literature.

CONCLUSIONS: Considering that the method is relatively simple and with few adverse effects, cryotherapy may represent a simple and safe treatment method for cutaneous KS. However, more studies should be conducted to further evaluate the effectiveness of cryotherapy as a promising treatment for cutaneous KS.

PMID:35015695

Extremity Soft Tissue Sarcoma: A Multi-Institutional Validation of Prognostic Nomograms

Fetched: January 12th, 2022, 5:01am GMT by Malcolm Hart Squires

Ann Surg Oncol. 2022 Jan 11. doi: 10.1245/s10434-021-11205-5. Online ahead of print.

ABSTRACT

BACKGROUND: Prognostic nomograms for patients with resected extremity soft tissue sarcoma (STS) include the Sarculator and Memorial Sloan Kettering (MSKCC) nomograms. We sought to validate these two nomograms within a large, modern, multi-institutional cohort of resected primary extremity STS patients.

METHODS: Resected primary extremity STS patients from 2000 to 2017 were identified across nine high-volume U.S. institutions. Predicted 5- and 10-year overall survival (OS) and distant metastases cumulative incidence (DMCI), and 4-, 8-, and 12-year disease-specific survival (DSS) were calculated with Sarculator and MSKCC nomograms, respectively. Predicted survival probabilities stratified in quintiles were compared in calibration plots to observed survival assessed by Kaplan-Meier estimates. Cumulative incidence was estimated for DMCI. Harrell's concordance index (C-index) assessed discriminative ability of nomograms.

RESULTS: A total of 1326 patients underwent resection of primary extremity STS. Common histologies included: undifferentiated pleomorphic sarcoma (35%), fibrosarcoma (13%), and leiomyosarcoma (9%). Median tumor size was 8.0 cm (IQR 4.5-13.0). Tumor grade distribution was: Grade 1 (13%), Grade 2 (9%), Grade 3 (78%). Median OS was 172 months, with estimated 5- and 10-year OS of 70% and 58%. C-indices for 5- and 10-year OS (Sarculator) were 0.72 (95% CI 0.70-0.75) and 0.73 (95% CI 0.70-0.75), and 0.72 (95% CI 0.69-0.75) for 5- and 10-year DMCI. C-indices for 4-, 8-, and 12-year DSS (MSKCC) were 0.71 (95% CI 0.68-0.75). Calibration plots showed good prognostication across all outcomes.

CONCLUSIONS: Sarculator and MSKCC nomograms demonstrated good prognostic ability for survival and recurrence outcomes in a modern, multi-institutional validation cohort of resected primary extremity STS patients. External validation of these nomograms supports their ongoing incorporation into clinical practice.

PMID:35015183 | DOI:10.1245/s10434-021-11205-5

Permalink for 'A pyridinium‑type fullerene derivative suppresses primary effusion lymphoma cell viability via the downregulation of the Wnt signaling pathway through the destabilization of β‑catenin'

A pyridinium‑type fullerene derivative suppresses primary effusion lymphoma cell viability via the downregulation of the Wnt signaling pathway through the destabilization of β‑catenin

Fetched: January 12th, 2022, 5:01am GMT by Ayano Kadota

Oncol Rep. 2022 Mar;47(3):46. doi: 10.3892/or.2022.8257. Epub 2022 Jan 11.

ABSTRACT

Primary effusion lymphoma (PEL) is defined as a rare subtype of non‑Hodgkin's B cell lymphoma, which is caused by Kaposi's sarcoma‑associated herpesvirus (KSHV) in immunosuppressed patients. PEL is an aggressive type of lymphoma and is frequently resistant to conventional chemotherapeutics. Therefore, the discovery of novel drug candidates for the treatment of PEL is of utmost importance. In order to discover potential novel anti‑tumor compounds against PEL, the authors previously developed a pyrrolidinium‑type fullerene derivative, 1,1,1',1'‑tetramethyl [60]fullerenodipyrrolidinium diiodide (derivative #1), which induced the apoptosis of PEL cells via caspase‑9 activation. In the present study, the growth inhibitory effects of pyrrolidinium‑type (derivatives #1 and #2), pyridinium‑type (derivatives #3 and #5 to #9) and anilinium‑type fullerene derivatives (derivative #4) against PEL cells were evaluated. This analysis revealed a pyridinium‑type derivative (derivative #5; 3‑5'‑(etho xycarbonyl)‑1',5'‑dihydro‑2'H‑[5,6]fullereno‑C60‑Ih‑[1,9‑c]pyrrol‑2'‑yl]‑1‑methylpyridinium iodide), which exhibited antitumor activity against PEL cells via the downregulation of Wnt/β‑catenin signaling. Derivative #5 suppressed the viability of KSHV‑infected PEL cells compared with KSHV‑uninfected B‑lymphoma cells. Furthermore, derivative #5 induced the destabilization of β‑catenin and suppressed β‑catenin‑TCF4 transcriptional activity in PEL cells. It is known that the constitutive activation of Wnt/β‑catenin signaling is essential for the growth of KSHV‑infected cells. The Wnt/β‑catenin activation in KSHV‑infected cells is mediated by KSHV latency‑associated nuclear antigen (LANA). The data demonstrated that derivative #5 increased β‑catenin phosphorylation, which resulted in β‑catenin polyubiquitination and subsequent degradation. Thus, derivative #5 overcame LANA‑mediated β‑catenin stabilization. Furthermore, the administration of derivative #5 suppressed the development of PEL cells in the ascites of SCID mice with tumor xenografts derived from PEL cells. On the whole, these findings provide evidence that the pyridinium‑type fullerene derivative #5 exhibits antitumor activity against PEL cells in vitro and in vivo. Thus, derivative #5 may be utilized as a novel therapeutic agent for the treatment of PEL.

PMID:35014678 | DOI:10.3892/or.2022.8257

Spindle Variant Primary Diffuse Large B Cell Lymphoma of the Colon: Case Report and Literature Review

Fetched: January 12th, 2022, 5:01am GMT by Serhat Toprak

J Gastrointest Cancer. 2022 Jan 11. doi: 10.1007/s12029-021-00790-z. Online ahead of print.

ABSTRACT

Diffuse large B cell lymphoma (DLBCL) with spindle cell morphology is a rare variant that can be seen in extranodal regions. Because the spindle cell dominant morphology in lymphoma is extremely unusual, the diagnosis can easily be missed in many organ systems. We present a case of an 82-year-old male patient with complaints of abdominal pain and swelling. He operated with the preliminary diagnosis of cecum tumor and ileum perforation. Tumoral proliferation was observed originating from the submucosa and infiltrating the muscularis propria, with the features of mostly spindle-shaped, having round-shaped nuclei in some of the cells, and having relatively narrow cytoplasm. A panel of immunohistochemical stains were performed to rule out the possibilities of sarcoma, carcinoma, or melanoma. Diffuse strong positive reaction was observed for CD45, CD20, CD19, CD22, Pax5, and CD30. The case was reported as spindle cell variant of DLBCL based on the present findings. As far as we know, this is the first case described in the colon. We emphasize that pathologists should be reminded of lymphoma as a differential diagnosis of spindle cell tumors.

PMID:35013921 | DOI:10.1007/s12029-021-00790-z

Prediction of biomarkers and therapeutic combinations for anti-PD-1 immunotherapy using the global gene network association

Fetched: January 12th, 2022, 5:01am GMT by Chia-Chin Wu

Nat Commun. 2022 Jan 10;13(1):42. doi: 10.1038/s41467-021-27651-4.

ABSTRACT

Owing to a lack of response to the anti-PD1 therapy for most cancer patients, we develop a network approach to infer genes, pathways, and potential therapeutic combinations that are associated with tumor response to anti-PD1. Here, our prediction identifies genes and pathways known to be associated with anti-PD1, and is further validated by 6 CRISPR gene sets associated with tumor resistance to cytotoxic T cells and targets of the 36 compounds that have been tested in clinical trials for combination treatments with anti-PD1. Integration of our top prediction and TCGA data identifies hundreds of genes whose expression and genetic alterations that could affect response to anti-PD1 in each TCGA cancer type, and the comparison of these genes across cancer types reveals that the tumor immunoregulation associated with response to anti-PD1 would be tissue-specific. In addition, the integration identifies the gene signature to calculate the MHC I association immunoscore (MIAS) that shows a good correlation with patient response to anti-PD1 for 411 melanoma samples complied from 6 cohorts. Furthermore, mapping drug target data to the top genes in our association prediction identifies inhibitors that could potentially enhance tumor response to anti-PD1, such as inhibitors of the encoded proteins of CDK4, GSK3B, and PTK2.

PMID:35013211 | DOI:10.1038/s41467-021-27651-4

Preliminary effects of toripalimab combined with axitinib in the treatment of advanced renal cell carcinoma

Fetched: January 12th, 2022, 5:01am GMT by L Zhou

Zhonghua Yi Xue Za Zhi. 2022 Jan 11;102(2):136-140. doi: 10.3760/cma.j.cn112137-20210527-01225.

ABSTRACT

Objective: To analyze the efficacy and safety of toripalimab combined with axitinib in the treatment of advanced renal cell carcinoma. Methods: Clinical data of 50 patients with advanced renal cell carcinoma who received axitinib combined with toripalimab were retrospectively collected from the database of Peking University Cancer Hospital. ORR, DCR, PFS, and OS were analyzed. Results: Among the 50 patients, 37 were males; median age was 56 (22-73) years; 38 were pathologically diagnosed as clear cell renal cell carcinoma and 12 were non-clear cell carcinoma. Common metastatic sites included lung, bone, lymph node, liver, and so on. 90% of the patients had received at least one-line of systemic therapy. With a median follow-up time of 11.9 months (0.8-24), 27 of the 50 patients are still on treatment, ORR was 34%, DCR was 86%, median PFS was 13.1 months (95%CI 5.8-20.4), and median OS has not yet reached. One-year OS rate was 84.6%. Common adverse reactions were proteinuria, diarrhea, hypertension, abnormal thyroid function, elevated transaminase, and hand-foot syndrome. Most adverse events were grade 1-2. Conclusion: Toripalimab combined with axitinib was efficient in the treatment of advanced renal cell carcinoma, and had manageable adverse reactions.

PMID:35012303 | DOI:10.3760/cma.j.cn112137-20210527-01225

An in-silico approach to design potential siRNAs against the ORF57 of Kaposi's sarcoma-associated herpesvirus

Fetched: January 12th, 2022, 5:01am GMT by Anisur Rahman

Genomics Inform. 2021 Dec;19(4):e47. doi: 10.5808/gi.21057. Epub 2021 Dec 31.

ABSTRACT

Kaposi's sarcoma-associated herpesvirus (KSHV) is one of the few human oncogenic viruses, which causes a variety of malignancies, including Kaposi's sarcoma, multicentric Castleman disease, and primary effusion lymphoma, particularly in human immunodeficiency virus patients. The currently available treatment options cannot always prevent the invasion and dissemination of this virus. In recent times, siRNA-based therapeutics are gaining prominence over conventional medications as siRNA can be designed to target almost any gene of interest. The ORF57 is a crucial regulatory protein for lytic gene expression of KSHV. Disruption of this gene translation will inevitably inhibit the replication of the virus in the host cell. Therefore, the ORF57 of KSHV could be a potential target for designing siRNA-based therapeutics. Considering both sequence preferences and target site accessibility, several online tools (i-SCORE Designer, Sfold web server) had been utilized to predict the siRNA guide strand against the ORF57. Subsequently, off-target filtration (BLAST), conservancy test (fuzznuc), and thermodynamics analysis (RNAcofold, RNAalifold, and RNA Structure web server) were also performed to select the most suitable siRNA sequences. Finally, two siRNAs were identified that passed all of the filtration phases and fulfilled the thermodynamic criteria. We hope that the siRNAs predicted in this study would be helpful for the development of new effective therapeutics against KSHV.

PMID:35012290 | DOI:10.5808/gi.21057

Epigenetics of Cutaneous Sarcoma

Fetched: January 12th, 2022, 5:01am GMT by Emi Mashima

Int J Mol Sci. 2021 Dec 31;23(1):422. doi: 10.3390/ijms23010422.

ABSTRACT

Epigenetic changes influence various physiological and pathological conditions in the human body. Recent advances in epigenetic studies of the skin have led to an appreciation of the importance of epigenetic modifications in skin diseases. Cutaneous sarcomas are intractable skin cancers, and there are no curative therapeutic options for the advanced forms of cutaneous sarcomas. In this review, we discuss the detailed molecular effects of epigenetic modifications on skin sarcomas, such as dermatofibrosarcoma protuberans, angiosarcoma, Kaposi's sarcoma, leiomyosarcoma, and liposarcoma. We also discuss the application of epigenetic-targeted therapy for skin sarcomas.

PMID:35008848 | DOI:10.3390/ijms23010422

New Perspectives for Eye-Sparing Treatment Strategies in Primary Uveal Melanoma

Fetched: January 12th, 2022, 5:01am GMT by Krzysztof Bilmin

Cancers (Basel). 2021 Dec 28;14(1):134. doi: 10.3390/cancers14010134.

ABSTRACT

Uveal melanoma is the most common intraocular malignancy and arises from melanocytes in the choroid, ciliary body, or iris. The current eye-sparing treatment options include surgical treatment, plaque brachytherapy, proton beam radiotherapy, stereotactic photon radiotherapy, or photodynamic therapy. However, the efficacy of these methods is still unsatisfactory. This article reviews several possible new treatment options and their potential advantages in treating localized uveal melanoma. These methods may be based on the physical destruction of the cancerous cells by applying ultrasounds. Two examples of such an approach are High-Intensity Focused Ultrasound (HIFU)-a promising technology of thermal destruction of solid tumors located deep under the skin and sonodynamic therapy (SDT) that induces reactive oxygen species. Another approach may be based on improving the penetration of anti-cancer agents into UM cells. The most promising technologies from this group are based on enhancing drug delivery by applying electric current. One such approach is called transcorneal iontophoresis and has already been shown to increase the local concentration of several different therapeutics. Another technique, electrically enhanced chemotherapy, may promote drug delivery from the intercellular space to cells. Finally, new advanced nanoparticles are developed to combine diagnostic imaging and therapy (i.e., theranostics). However, these methods are mostly at an early stage of development. More advanced and targeted preclinical studies and clinical trials would be needed to introduce some of these techniques to routine clinical practice.

PMID:35008296 | DOI:10.3390/cancers14010134

Efficacy of Neoadjuvant Targeted Therapy for Borderline Resectable III B-D or IV Stage BRAF V600 Mutation-Positive Melanoma

Fetched: January 12th, 2022, 5:01am GMT by Anna M Czarnecka

Cancers (Basel). 2021 Dec 27;14(1):110. doi: 10.3390/cancers14010110.

ABSTRACT

Neoadjuvant therapy for locally advanced disease or potentially resectable metastatic melanoma is expected to improve operability and clinical outcomes over upfront surgery and adjuvant treatment as it is for sarcoma, breast, rectal, esophageal, or gastric cancers. Patients with locoregional recurrence after initial surgery and those with advanced regional lymphatic metastases are at a high risk of relapse and melanoma-related death. There is an unmet clinical need to improve the outcomes for such patients. Patients with resectable bulky stage III or resectable stage IV histologically confirmed melanoma were enrolled and received standard-dose BRAFi/MEKi for at least 12 weeks before feasible resection of the pre-therapy target and then received at least for the next 40 weeks further BRAFi/MEKi. Of these patients, 37 were treated with dabrafenib and trametinib, three were treated with vemurafenib and cobimetinib, five with vemurafenib, and one with dabrafenib alone. All patients underwent surgery with 78% microscopically margin-negative resection (R0) resection. Ten patients achieved a complete pathological response. In patients with a major pathological response with no, or less than 10%, viable cells in the tumor, median disease free survival and progression free survival were significantly longer than in patients with a minor pathological response. No patient discontinued neoadjuvant BRAFi/MEKi due to toxicity. BRAFi/MEKi pre-treatment did not result in any new specific complications of surgery. Fourteen patients experienced disease recurrence or progression during post-operative treatment. We confirmed that BRAFi/MEKi combination is an effective and safe regimen in the perioperative treatment of melanoma. Pathological response to neoadjuvant treatment may be considered as a surrogate biomarker of disease recurrence.

PMID:35008274 | DOI:10.3390/cancers14010110

Permalink for 'Tipping Growth Inhibition into Apoptosis by Combining Treatment with MDM2 and WIP1 Inhibitors in p53WT Uterine Leiomyosarcoma'

Tipping Growth Inhibition into Apoptosis by Combining Treatment with MDM2 and WIP1 Inhibitors in p53WT Uterine Leiomyosarcoma

Fetched: January 12th, 2022, 5:01am GMT by Victoria Chamberlain

Cancers (Basel). 2021 Dec 21;14(1):14. doi: 10.3390/cancers14010014.

ABSTRACT

As there is no optimal therapeutic strategy defined for women with advanced or recurrent uLMS, there is an urgent need for the discovery of novel, targeted approaches. One such area of interest is the pharmacological inhibition of the MDM2-p53 interaction with small-molecular-weight MDM2 inhibitors. Growth inhibition and cytotoxic assays were used to evaluate uLMS cell line responses to MDM2 inhibitors as single agents and in combination, qRT-PCR to assess transcriptional changes and Caspase-Glo 3/7 assay to detect apoptosis. RG7388 and HDM201 are potent, selective antagonists of the MDM2-p53 interaction that can effectively stabilise and activate p53 in a dose-dependent manner. GSK2830371, a potent and selective WIP1 phosphatase inhibitor, was shown to significantly potentiate the growth inhibitory effects of RG7388 and HDM201, and significantly increase the mRNA expression of p53 transcriptional target genes in a p53^WT cell line at a concentration that has no growth inhibitory effects as a single agent. RG7388, HDM201 and GSK2830371 failed to induce apoptosis as single agents; however, a combination treatment tipped cells into apoptosis from senescence. These data present the possibility of MDM2 and WIP1 inhibitor combinations as a potential treatment option for p53^WT uLMS patients that warrants further investigation.

PMID:35008180 | DOI:10.3390/cancers14010014

Bilateral renal metastases from extraskeletal mesenchymal chondrosarcoma of thigh

Fetched: January 12th, 2022, 5:01am GMT by Himanshu Pruthi

BMJ Case Rep. 2022 Jan 7;15(1):e246375. doi: 10.1136/bcr-2021-246375.

ABSTRACT

Mesenchymal chondrosarcoma (MC) is a rare cartilaginous tumour that occurs in the extraskeletal locations in about one-third of cases. It is aggressive in behaviour and may involve the lower extremities, central nervous system or spine. Mesenchymal tumours are known for distant metastasis; however, metastasis to bilateral kidneys after treatment has not been reported earlier. We present a case of a soft-tissue intramuscular MC of the thigh in a 38-year-old patient which had been surgically excised after neoadjuvant chemotherapy. The patient presented with bilateral dense calcified renal masses after 6 years, which were cytologically proven as MC metastases. In the evaluation of bilateral calcified renal masses in patients with a history of MC, metastasis should be considered.

PMID:34996769 | DOI:10.1136/bcr-2021-246375

Leukemia Cutis

Fetched: January 12th, 2022, 5:01am GMT by Meghana Parsi

2022 Jan 9. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan–.

ABSTRACT

Leukemia cutis characteristically demonstrates the infiltration of the skin by neoplastic leukocytes. While the extramedullary collection of leukemic cells is generally regarded as myeloid sarcoma (previously chloroma/granulocytic sarcoma), leukemia cutis is a generic term to describe specific cutaneous involvement. Although any subtype of leukemia can involve the skin, the most common types seen in clinical practice are chronic lymphocytic leukemia (CLL) and acute myeloid leukemia (AML) with monocytic or myelomonocytic morphology. Involvement by chronic myeloid leukemia (CML) is extremely rare and may be indicative of the blast phase. Patients usually have a prior diagnosis of systemic leukemia or myelodysplasia. However, cutaneous lesions may present as the primary manifestation of systemic disease in rare cases. Cutaneous involvement is generally indicative of advanced disease and should stipulate the investigation of other body sites for extramedullary involvement.

Subtypes of Leukemia Involving Skin:

Myeloid/monocytic disorders:

Acute myeloid leukemia (monocytic or myelomonocytic) (AML)
Chronic myeloid leukemia
Chronic myelomonocytic leukemia (transformation)
Myelodysplastic syndrome (transformation)

Lymphoproliferative disorders:

B- cell leukemia/lymphomas
1. Precursor B-cell acute lymphoblastic leukemia
2. Chronic lymphocytic leukemia (CLL)
3. Hairy cell leukemia

T- cell leukemia/lymphomas
1. Precursor T-cell acute lymphoblastic leukemia
2. Adult T-cell leukemia/lymphoma (ATLL)
3. T- cell prolymphocytic leukemia

PMID:31082180 | Bookshelf:NBK541136

Permalink for 'Cytotoxicity of Nucleotide-Stabilized Graphene Dispersions on Osteosarcoma and Healthy Cells: On the Way to Safe Theranostics Agents'

Cytotoxicity of Nucleotide-Stabilized Graphene Dispersions on Osteosarcoma and Healthy Cells: On the Way to Safe Theranostics Agents

Fetched: January 11th, 2022, 5:02am GMT by Mónica Cicuéndez

ACS Appl Bio Mater. 2021 May 17;4(5):4384-4393. doi: 10.1021/acsabm.1c00144. Epub 2021 May 4.

ABSTRACT

An appealing strategy that overcomes the hydrophobicity of pristine graphene and favors its interaction with biological media is colloidal stabilization in aqueous medium with the support of a biomolecule, such as flavin mononucleotide (FMN), as exfoliating/dispersing agent. However, to establish FMN-stabilized graphene (PG-FMN) as suitable for use in biomedicine, its biocompatibility must be proved by a complete assessment of cytotoxicity at the cellular level. Furthermore, if PG-FMN is to be proposed as a theranostic agent, such a study should include both healthy and tumoral cells and its outcome should reveal the nanomaterial as selectively toxic to the latter. Here, we provide an in-depth comparative in vitro analysis of the response of Saos-2 human sarcoma osteoblasts (model tumor cells) and MC3T3-E1 murine preosteoblasts (undifferentiated healthy cells) upon incubation with different concentrations (10-50 μg mL^-1) of PG-FMN dispersions constituted by flakes with different average lateral size (90 and 270 nm). Specifically, the impact of PG-FMN on the viability and cell proliferation, reactive oxygen species (ROS) production, and the cellular incorporation process, cell-cycle progression, and apoptosis has been evaluated. PG-FMN was found to be toxic to both types of cells by increasing ROS production and triggering cell-cycle arrest. The present results constitute a cautionary tale on the need to establish the effect of a nanomaterial not only on tumor cells but also on healthy ones before proposing it as anticancer agent.

PMID:35006850 | DOI:10.1021/acsabm.1c00144

ASO Video Abstract: Delayed Gastric Emptying After Multivisceral Resection for Retroperitoneal Sarcoma

Fetched: January 11th, 2022, 5:02am GMT by Marco Baia

Ann Surg Oncol. 2022 Jan 10. doi: 10.1245/s10434-021-11261-x. Online ahead of print.

NO ABSTRACT

PMID:35006511 | DOI:10.1245/s10434-021-11261-x

Permalink for 'Score for the Risk and Overall Survival of Lung Metastasis in Patients First Diagnosed With Soft Tissue Sarcoma: A Novel Nomogram-Based Risk Assessment System'

Score for the Risk and Overall Survival of Lung Metastasis in Patients First Diagnosed With Soft Tissue Sarcoma: A Novel Nomogram-Based Risk Assessment System

Fetched: January 11th, 2022, 5:02am GMT by Zhiyi Fan

Technol Cancer Res Treat. 2022 Jan-Dec;21:15330338211066240. doi: 10.1177/15330338211066240.

ABSTRACT

Background: Metastatic soft tissue sarcoma (STS) patients have a poor prognosis with a 3-year survival rate of 25%. About 30% of them present lung metastases (LM). This study aimed to construct 2 nomograms to predict the risk of LM and overall survival of STS patients with LM. Materials and Methods: The data of patients were derived from the Surveillance, Epidemiology, and End Results database during the period of 2010 to 2015. Logistic and Cox analysis was performed to determine the independent risk factors and prognostic factors of STS patients with LM, respectively. Afterward, 2 nomograms were, respectively, established based on these factors. The performance of the developed nomogram was evaluated with receiver operating characteristic curves, area under the curve (AUC) calibration curves, and decision curve analysis (DCA). Results: A total of 7643 patients with STS were included in this study. The independent predictors of LM in first-diagnosed STS patients were N stage, grade, histologic type, and tumor size. The independent prognostic factors for STS patients with LM were age, N stage, surgery, and chemotherapy. The AUCs of the diagnostic nomogram were 0.806 in the training set and 0.799 in the testing set. For the prognostic nomogram, the time-dependent AUC values of the training and testing set suggested a favorable performance and discrimination of the nomogram. The 1-, 2-, and 3-year AUC values were 0.698, 0.718, and 0.715 in the training set, and 0.669, 0.612, and 0717 in the testing set, respectively. Furthermore, for the 2 nomograms, calibration curves indicated satisfactory agreement between prediction and actual survival, and DCA indicated its clinical usefulness. Conclusion: In this study, grade, histology, N stage, and tumor size were identified as independent risk factors of LM in STS patients, age, chemotherapy surgery, and N stage were identified as independent prognostic factors of STS patients with LM, these developed nomograms may be an effective tool for accurately predicting the risk and prognosis of newly diagnosed patients with LM.

PMID:35006028 | DOI:10.1177/15330338211066240

Intraoperative angiography imaging correlates with wound complications following soft tissue sarcoma resection

Fetched: January 11th, 2022, 5:02am GMT by Alexander L Lazarides

J Orthop Res. 2022 Jan 10. doi: 10.1002/jor.25270. Online ahead of print.

ABSTRACT

BACKGROUND: For soft tissue sarcoma patients receiving preoperative radiation therapy, wound complications are common and potentially devastating. The purpose of this study was to assess the feasibility of intraoperative indocyanine green fluorescent angiography (ICGA) as a predictor of wound complications in these patients.

METHODS: A consecutive series of patients of patients with soft tissue sarcoma of the extremities or pelvis who received neoadjuvant radiation and a subsequent radical resection received intraoperative ICGA with the SPY PHI device (Stryker Inc, Kalamazoo MI) at the time of closure. Retrospective analysis of fluorescence signal along multiple points of the wound length was performed and quantified. The primary endpoint was wound complication, defined as delayed wound healing or wound dehiscence, within 3 months of surgery.

RESULTS: 14 patients with preoperative irradiated soft-tissue sarcoma were consecutively imaged. There were 6 patients with wound complications classified as "aseptic" in 5 cases. Using the ICGA, blinded surgeons correctly predicted wound complications in 75% of cases. During the inflow phase, a mean ratio of normal of 0.62 maximized the area under the curve (AUC=0.90) for predicting wound complications with a sensitivity of 100% and specificity of 77.4%. During the peak phase, a mean ratio of normal of 0.55 maximized the area under the curve (AUC=0.95) for predicting wound complications with a sensitivity of 88.9% and a specificity 100%.

CONCLUSIONS: Intraoperative use of indocyanine green fluorescent angiography may help to predict wound complications in patients undergoing resection of preoperatively irradiated soft tissue sarcomas of the extremities and pelvis. This article is protected by copyright. All rights reserved.

PMID:35005805 | DOI:10.1002/jor.25270

Why Rotationplasty? A Qualitative Study of Decision-Making by Families of Patients With Primary Bone Sarcoma

Fetched: January 11th, 2022, 5:02am GMT by Chung M Chan

J Patient Exp. 2022 Jan 4;9:23743735211069818. doi: 10.1177/23743735211069818. eCollection 2022.

ABSTRACT

Rotationplasty is an established technique that is indicated as part of the surgical reconstruction for certain patients with primary bone tumors around the knee who undergo tumor resection. There is considerable variation in the application of rotationplasty by surgeons as well as acceptance of the procedure by patients who may be candidates for this procedure. We qualitatively studied the decision-making process of families of patients who had undergone rotationplasty by interviewing 4 patients and their families using semi-structured interviews. Thematic analysis identified the following themes that were important in the decision-making process: (1) the desire for good information sources, (2) finding value in meeting with other patients who had been faced with a similar decision, (3) prioritizing function over cosmesis, (4) a desire to limit the need for revision surgeries, and (5) accepting that a return to normalcy is not an option with a surgery. Physicians and patients faced with a similar decision can benefit from a better understanding of the process, and by the normalization of anxieties and concerns that they may experience.

PMID:35005220 | PMC:PMC8733356 | DOI:10.1177/23743735211069818

Chronic lymphocytic leukemia/small lymphocytic lymphoma complicated with skin Langerhans cell sarcoma: A case report

Fetched: January 11th, 2022, 5:02am GMT by Shao-Yan Li

World J Clin Cases. 2021 Dec 6;9(34):10715-10722. doi: 10.12998/wjcc.v9.i34.10715.

ABSTRACT

BACKGROUND: Langerhans cell sarcoma (LCS) is a rare malignancy with poor prognosis. LCS and chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) can occur in the same diseased tissues, such as lymph nodes or skin.

CASE SUMMARY: A 48-year-old female Han Chinese patient was admitted for generalized lymph node enlargement for 6 years and abdominal distension for 1 wk. She was diagnosed with small B-cell lymphoma (stage IV)/CLL (Benet stage B) and received chemotherapy. She started oral ibrutinib in February 2019. She was hospitalized on June 11, 2019, and a 1.5 cm × 1.5 cm dark-red nodule with ulceration scalp lesion was found. Biopsy revealed LCS but without CLL/SLL. She was diagnosed with CLL/SLL (Binet stage C, Rai stage IV) accompanied by secondary histiocytic sarcomas and skin LCS and received cyclophosphamide, doxorubicin, vincristine, dexamethasone, and etoposide but developed severe cytopenia. She ultimately refused treatments and discharged spontaneously. She died on September 12, 2019. The literature review showed that in patients with CLL/SLL, skin lesions of LCS are accompanied by CLL/SLL. This patient was different from the previously reported cases of skin LCS in patients with CLL/SLL.

CONCLUSION: In this patient, the skin lesion of LCS showed no concomitant CLL/SLL.

PMID:35005006 | PMC:PMC8686146 | DOI:10.12998/wjcc.v9.i34.10715

Nodal Langerhans cell neoplasm: detailing the diagnostic quandaries

Fetched: January 11th, 2022, 5:02am GMT by Zachariah Chowdhury

Autops Case Rep. 2021 Dec 10;11:e2021344. doi: 10.4322/acr.2021.344. eCollection 2021.

ABSTRACT

Langerhans cells, found in the supra-basal region of the mucous membranes in the epidermis of the skin, in lymph nodes and thymus, function as antigen-presenting cells within the histiocyte system. Tumors derived from Langerhans cells (LC) can be divided according to the degree of cytological atypia and clinical behavior into Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS). LCS is rare, and the nodal presentation is even rarer with challenging histological characteristics. LCS has a dismal overcome despite intensive chemotherapy. Herein, we report a case of a 29-year-old male who presented with generalized lymphadenopathy initially considered as a lymphoma. An outright definitive diagnosis could not be attained in the initial histomorphological and immunohistochemical evaluation, fraught with differential diagnoses. The key to decoding the precise neoplasm was a combination of the cytopathologic features, review of the histomorphology, and extensive immunohistochemical assessment in conjunction with the clinical and positron emission tomography (PET) scan findings. The best diagnosis proffered was a Langerhans cell histiocytosis progressing to Langerhans cell sarcoma. This case highlights the grey zone areas in LC neoplasms, the diagnostic conundrums encountered, the indispensable role of meticulous pathological analysis, and the importance of ancillary studies in hammering out the final diagnosis.

PMID:35004363 | PMC:PMC8722817 | DOI:10.4322/acr.2021.344

Permalink for 'Case Report: Phenotypic Switch in High-Grade B-Cell Lymphoma With MYC and BCL6 Rearrangements: A Potential Mechanism of Therapeutic Resistance in Lymphoma?'

Case Report: Phenotypic Switch in High-Grade B-Cell Lymphoma With MYC and BCL6 Rearrangements: A Potential Mechanism of Therapeutic Resistance in Lymphoma?

Fetched: January 11th, 2022, 5:02am GMT by Hui Liu

Front Oncol. 2021 Dec 23;11:795330. doi: 10.3389/fonc.2021.795330. eCollection 2021.

ABSTRACT

Lineage switch between myeloid and lymphoid in acute leukemia is well established as a mechanism for leukemic cells to escape chemotherapy. Cross-lineage transformation is also recognized in some solid tumors on targeted therapy, such as adenocarcinomas of the lung and prostate that transforms to neuroendocrine carcinoma on targeted therapy. Now lineage plasticity is increasingly recognized in mature lymphomas, such as small B-cell lymphomas transforming to histiocytic/dendritic cell sarcoma. However, there is no report of aggressive mature B-cell lymphoma switching from one histologic category to another upon targeted therapy. We report here a case of high-grade B-cell lymphoma with MYC and BCL6 rearrangements relapsing as a high-grade plasmablastic neoplasm with MYC and BCL6 rearrangements after R-CHOP and R-EPOCH therapy. Being aware of this rare scenario will help improve our understanding of the underlying mechanisms of therapeutic resistance and progression of lymphoma.

PMID:35004320 | PMC:PMC8733465 | DOI:10.3389/fonc.2021.795330

The Renaissance of KRAS Targeting in Advanced Non-Small-Cell Lung Cancer: New Opportunities Following Old Failures

Fetched: January 11th, 2022, 5:02am GMT by Miriam Grazia Ferrara

Front Oncol. 2021 Dec 23;11:792385. doi: 10.3389/fonc.2021.792385. eCollection 2021.

ABSTRACT

Non-small cell lung cancer (NSCLC) represents the perfect paradigm of 'precision medicine' due to its complex intratumoral heterogeneity. It is truly characterized by a range of molecular alterations that can deeply influence the natural history of this disease. Several molecular alterations have been found over time, paving the road to biomarker-driven therapy and radically changing the prognosis of 'oncogene addicted' NSCLC patients. Kirsten rat sarcoma (KRAS) mutations are present in up to 30% of NSCLC (especially in adenocarcinoma histotype) and have been identified decades ago. Since its discovery, its molecular characteristics and its marked affinity to a specific substrate have led to define KRAS as an undruggable alteration. Despite that, many attempts have been made to develop drugs capable of targeting KRAS signaling but, until a few years ago, these efforts have been unsuccessful. Comprehensive genomic profiling and wide-spectrum analysis of genetic alterations have only recently allowed to identify different types of KRAS mutations. This tricky step has finally opened new frontiers in the treatment approach of KRAS-mutant patients and might hopefully increase their prognosis and quality of life. In this review, we aim to highlight the most interesting aspects of (epi)genetic KRAS features, hoping to light the way to the state of art of targeting KRAS in NSCLC.

PMID:35004317 | PMC:PMC8733471 | DOI:10.3389/fonc.2021.792385

Expert consensus on resection of chest wall tumors and chest wall reconstruction

Fetched: January 11th, 2022, 5:02am GMT by Lei Wang

Transl Lung Cancer Res. 2021 Nov;10(11):4057-4083. doi: 10.21037/tlcr-21-935.

ABSTRACT

Chest wall tumors are a relatively uncommon disease in clinical practice. Most of the published studies about chest wall tumors are usually single-center retrospective studies, involving few patients. Therefore, evidences regarding clinical conclusions about chest wall tumors are lacking, and some controversial issues have still to be agreed upon. In January 2019, 73 experts in thoracic surgery, plastic surgery, science, and engineering jointly released the Chinese Expert Consensus on Chest Wall Tumor Resection and Chest Wall Reconstruction (2018 edition). After that, numerous experts put forward new perspectives on some academic issues in this version of the consensus, pointing out the necessity to further discuss the points of contention. Thus, we conducted a survey through the administration of a questionnaire among 85 experts in the world. Consensus has been reached on some major points as follows. (I) Wide excision should be performed for desmoid tumor (DT) of chest wall. After excluding the distant metastasis by multi-disciplinary team, solitary sternal plasmacytoma can be treated with extensive resection and adjuvant radiotherapy. (II) Wide excision with above 2 cm margin distance should be attempted to obtain R0 resection margin for chest wall tumor unless the tumor involves vital organs or structures, including the great vessels, heart, trachea, joints, and spine. (III) For patients with chest wall tumors undergoing unplanned excision (UE) for the first time, it is necessary to carry out wide excision as soon as possible within 1-3 months following the previous surgery. (IV) Current Tumor Node Metastasis staging criteria (American Joint Committee on Cancer) of bone tumor and soft tissue sarcoma are not suitable for chest wall sarcomas. (V) It is necessary to use rigid implants for chest wall reconstruction once the maximum diameter of the chest wall defect exceeds 5 cm in adults and adolescents. (VI) For non-small cell lung cancer (NSCLC) invading the chest wall, wide excision with neoadjuvant and/or adjuvant therapy are recommended for patients with stage T3-4N0-1M0. As clear guidelines are lacking, these consensus statements on controversial issues on chest wall tumors and resection could possibly serve as further guidance in clinical practice during the upcoming years.

PMID:35004239 | PMC:PMC8674598 | DOI:10.21037/tlcr-21-935

Permalink for 'The Role of Tissue and Liquid Biopsy in the Clinical Management of Adult Lung Cancer Patients in King Abdul-Aziz Medical City in Riyadh, Saudi Arabia'

The Role of Tissue and Liquid Biopsy in the Clinical Management of Adult Lung Cancer Patients in King Abdul-Aziz Medical City in Riyadh, Saudi Arabia

Fetched: January 11th, 2022, 5:02am GMT by Lafi Alanazi

Cureus. 2022 Jan 3;14(1):e20914. doi: 10.7759/cureus.20914. eCollection 2022 Jan.

ABSTRACT

Background Lung cancer is the most fatal malignancy worldwide, characterized by uncontrolled growth in the tissue of the lung(s). The diagnosis of lung cancer depends on the medical history of the patient, along with the physical examination, and various imaging studies. Furthermore, sputum cytology, thoracentesis, or a tissue and liquid biopsy can be examined. The TNM (tumor size, lymph nodes, and metastasis) system is used for staging and grading lung cancer. This study aimed to evaluate the role of tissue vs liquid biopsy in the clinical management of adenocarcinoma, at King Abdulaziz Medical City, Riyadh. Methods In this cross-sectional study, all adenocarcinoma patients treated between January 2016 to December 2018 were included using consecutive sampling. The participants were ≥ 18 years old patients with histologically confirmed adenocarcinoma (stage IIIb/IV) regardless of the mutation status. This data was collected through chart review. Data analysis was performed using the IBM Statistical Software for Social Sciences (SPSS) software, version 22 (IBM SPSS Statistics for Windows, Armonk, NY). Results A total of 58 participants were included in the analysis. All of them had undergone a tissue biopsy, while only 16 patients underwent liquid biopsy. Out of all patients, 26% of patients had tissue biopsy-related complications (TBRC), with pneumothorax being the most common complication. Single gene testing for epidermal growth factor receptor (EGFR) for patients who underwent tissue biopsy showed a 35% mutation rate. For the anaplastic lymphoma kinase (ALK) gene, 13% were found to be mutated; for the ROS proto-oncogene 1 (ROS1) gene, only 7% were seen to be mutated. For a panel of 12 genes, 25% had the tumor protein 53 (TP53) gene mutation and 39% had the gene Kirsten rat sarcoma viral oncogene homolog (KRAS) mutations. For patients who underwent a liquid biopsy, 20% had the TP53 mutation, 43% had the EGFR mutations on a single gene test and 42% on a panel test, and 10% had the KRAS mutation. Conclusion We found that tissue and liquid biopsy showed genetic mutations, particularly with EGFR, TP53, and KRAS genes, among adenocarcinoma patients. Identifying genetic changes in adenocarcinoma patients is essential for charting a targeted therapy. Primary EGFR mutations and rearrangements of ALK or ROS1 are the only gene mutations that can be done with specific tyrosine kinase inhibitors available for clinical practice. Therefore, we recommend further studies to evaluate the role of tissue and liquid biopsy in clinical practice.

PMID:35004079 | PMC:PMC8724526 | DOI:10.7759/cureus.20914

Omental Flap to Non-healing Posterior Trunk Wound: A Case Report

Fetched: January 11th, 2022, 5:02am GMT by Jake Laun

Cureus. 2021 Dec 5;13(12):e20178. doi: 10.7759/cureus.20178. eCollection 2021 Dec.

ABSTRACT

Large posterior trunk wounds often require flap reconstruction. One option for posterior truncal reconstruction not readily considered, often due to the combined anterior and posterior approaches required for harvesting and coverage, is the omental flap; however, the omental flap stands as a robust backup in non-healing wounds when local flap options have been exhausted. We present a case of a posterior trunk wound that had previously undergone multiple unsuccessful local and regional flaps for reconstruction and was ultimately treated with a pedicled omental flap which went on to heal without any post-operative complications.

PMID:35004003 | PMC:PMC8723772 | DOI:10.7759/cureus.20178

Permalink for 'Prevalence and Risk Factors of Kaposi's Sarcoma-Associated Herpesvirus Infection among Han and Uygur Populations in Xinjiang, China'

Prevalence and Risk Factors of Kaposi's Sarcoma-Associated Herpesvirus Infection among Han and Uygur Populations in Xinjiang, China

Fetched: January 11th, 2022, 5:02am GMT by Zhi Wen

Can J Infect Dis Med Microbiol. 2021 Dec 31;2021:2555865. doi: 10.1155/2021/2555865. eCollection 2021.

ABSTRACT

Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of Kaposi's sarcoma (KS), which is endangering human health worldwide, especially in Africa, Europe, the United States, and parts of Asia. The aim of this study was to investigate the prevalence of KSHV in Xinjiang. Three KSHV recombinant proteins (ORF65, ORF73, and K8.1) were used to detect KSHV infection. The serum samples to be tested were detected by an indirect ELISA method. The overall infection rate of KSHV in Xinjiang was 25.60%, with a higher infection rate in the Uygur population of 29.79%. After adjusting for possible confounders, Uygur (OR = 3.95, 95% CI 2.64-6.12, P < 0.001), agriculture and livestock (OR = 1.60, 95% CI 1.20-2.17, P = 0.002), age ≤ 50 years (OR = 1.50, 95% CI 1.13-2.00, P = 0.006), and predominantly meat-based diet (OR = 1.72, 95% CI 1.11-2.78, P = 0.018) were significantly associated with the odds of KSHV seropositivity correlation. Three unique sequences of KSHV were obtained in this study; genotypic analysis showed that the three unique sequences were all subtype A2.

PMID:35003405 | PMC:PMC8741394 | DOI:10.1155/2021/2555865

m6A Modification Patterns With Distinct Immunity, Metabolism, and Stemness Characteristics in Soft Tissue Sarcoma

Fetched: January 11th, 2022, 5:02am GMT by Zhen-Dong Huang

Front Immunol. 2021 Dec 24;12:765723. doi: 10.3389/fimmu.2021.765723. eCollection 2021.

ABSTRACT

N6-methyladenosine (m6A) RNA methylation has been shown to have prognostic value in cancer. Nonetheless, its potential role regarding immunity, metabolism, and stemness in soft tissue sarcoma (STS) remains unknown. We comprehensively estimated the m6A modification patterns and corresponding immunity, metabolism, and stemness characteristics based on 568 STS samples and 21 m6A regulators. The m6Ascore was constructed to quantify m6A modification patterns in individuals using machine learning algorithms. Two distinct m6A modification patterns among the STS patients were identified, which exhibited differences in prognosis, immune cell infiltration, metabolic pathways, stemness, somatic mutation, and copy number variation. Thereafter, immunity-, metabolism-, and stemness phenotype-related genes associated with m6A modification were identified. Furthermore, patients with lower m6Ascores had increased antitumor immune responses, survival benefit under immunotherapy, tumor mutation burden, immunogenicity, and response to anti-PD-1/L1 immunotherapy. Immunotherapy sensitivity was validated using the IMvigor210 dataset. STS patients with lower m6Ascore might be more sensitive to docetaxel and gemcitabine. Finally, pan-cancer analysis illustrated the significant correlations of m6Ascore with clinical outcomes, immune cell infiltration, metabolism, and stemness. This study revealed that m6A modification plays an important role in immunity, metabolism, and stemness in STS. Evaluating the m6A modification pattern and development of m6Ascore may help to guide more effective immunotherapy and chemotherapy strategies.

PMID:35003079 | PMC:PMC8739240 | DOI:10.3389/fimmu.2021.765723

Permalink for 'A Deletion of the Nuclear Localization Signal Domain in the Fus Protein Induces Stable Post-stress Cytoplasmic Inclusions in SH-SY5Y Cells'

A Deletion of the Nuclear Localization Signal Domain in the Fus Protein Induces Stable Post-stress Cytoplasmic Inclusions in SH-SY5Y Cells

Fetched: January 11th, 2022, 5:02am GMT by Antonietta Notaro

Front Neurosci. 2021 Dec 23;15:759659. doi: 10.3389/fnins.2021.759659. eCollection 2021.

ABSTRACT

Mutations in Fused-in-Sarcoma (FUS) gene involving the nuclear localization signal (NLS) domain lead to juvenile-onset Amyotrophic Lateral Sclerosis (ALS). The mutant protein mislocalizes to the cytoplasm, incorporating it into Stress Granules (SG). Whether SGs are the first step to the formation of stable FUS-containing aggregates is still unclear. In this work, we used acute and chronic stress paradigms to study the SG dynamics in a human SH-SY5Y neuroblastoma cell line carrying a deletion of the NLS domain of the FUS protein (homozygous: ΔNLS^-/-; heterozygous: ΔNLS^+/-). Wild-type (WT) cells served as controls. We evaluated the subcellular localization of the mutant protein through immunoblot and immunofluorescence, in basal conditions and after acute stress and chronic stress with sodium arsenite (NaAsO2). Cells were monitored for up to 24 h after rescue. FUS was expressed in both nucleus and cytoplasm in the ΔNLS^+/- cells, whereas it was primarily cytoplasmic in the ΔNLS^-/-. Acute NaAsO2 exposure induced SGs: at rescue,>90% of ΔNLS cells showed abundant FUS-containing if compared to less than 5% of the WT cells. The proportion of FUS-positive SGs remained 15-20% at 24 h in mutant cells. Cycloheximide did not abolish the long-lasting SGs in mutant cells. Chronic exposure to NaAsO2 did not induce significant SGs formation. A wealth of research has demonstrated that ALS-associated FUS mutations at the C-terminus facilitate the incorporation of the mutant protein into SGs. We have shown here that mutant FUS-containing SGs tend to fail to dissolve after stress, facilitating a liquid-to-solid phase transition. The FUS-containing inclusions seen in the dying motor neurons might therefore directly derive from SGs. This might represent an attractive target for future innovative therapies.

PMID:35002600 | PMC:PMC8733393 | DOI:10.3389/fnins.2021.759659

Mesenchymal Tumors Involving the Pancreas: A Clinicopathologic Analysis and Review of the Literature

Fetched: January 11th, 2022, 5:02am GMT by Gokce Askan

Turk Patoloji Derg. 2022;38(1):46-53. doi: 10.5146/tjpath.2022.01567.

ABSTRACT

OBJECTIVE: Most pancreatic tumors are epithelial, and, among these, more than 90% are of ductal origin. However, a variety of mesenchymal tumors may involve the pancreas and may manifest different clinicopathological characteristics. The literature on mesenchymal tumors in the pancreas is largely limited to individual case reports or analyses of small series, predominantly focusing on radiologic features.

MATERIAL AND METHOD: Authors' institutional and consultation databases were reviewed to identify the mesenchymal tumors involving the pancreas.

RESULTS: Forty cases were identified; twenty-five (63%) tumors were benign/borderline, and the remaining fifteen (37%) were malignant. Of the benign/borderline tumors; 9 were solitary fibrous tumors, 6 gastrointestinal stromal tumors (GISTs), 4 schwannomas, 2 desmoid type fibromatosis, 1 lymphangioma, 1 ganglioneuroma, 1 inflammatory myofibroblastic tumor, and 1 low grade mesenchymal neoplasm. Malignant tumors included 6 cases of leiomyosarcomas, 4 liposarcomas, 2 rhabdomyosarcomas, 1 epithelioid angiosarcoma, 1 malignant peripheral nerve sheet tumor, and 1 undifferentiated pleomorphic sarcoma. Four cases (multicystic schwannoma, desmoid fibromatosis, lymphangioma and inflammatory myofibroblastic tumor) were preoperatively misdiagnosed as a primary epithelial tumor of the pancreas.

CONCLUSION: Mesenchymal tumors rarely involve the pancreas. They are usually benign/borderline neoplasms but may be diagnostically challenging, especially clinically/radiologically, as they may form cystic and/or large lesions in the pancreas. Mesenchymal tumors should be considered in both the clinical/radiological and pathological differential diagnosis of pancreatic lesions.

PMID:35001360 | DOI:10.5146/tjpath.2022.01567

A Phase II Study for 609A in the Treatment of Advanced Undifferentiated Pleomorphic Sarcoma

Posted: January 14th, 2022, 3:00pm GMT

Condition: Sarcoma, Soft Tissue
Intervention: Drug: Recombinant anti-PD-1 humanized monoclonal antibody injection
Sponsor: Sunshine Guojian Pharmaceutical (Shanghai) Co., Ltd.
Not yet recruiting

A Study of Anlotinib Combined With or Without PD-1 Antibody on Unresectable High-grade Chondrosarcoma

Posted: January 14th, 2022, 3:00pm GMT

Condition: Chondrosarcoma
Interventions: Drug: Anlotinib hydrochloride; Drug: PD-1 inhibitor
Sponsor: Second Affiliated Hospital, School of Medicine, Zhejiang University
Not yet recruiting

Albumin-bound Paclitaxel Plus Camrelizumab for Advanced Soft Tissue Sarcoma.

Posted: January 12th, 2022, 3:00pm GMT

Condition: Soft Tissue Sarcoma
Interventions: Drug: Albumin-Bound Paclitaxel; Drug: Camrelizumab
Sponsor: Henan Cancer Hospital
Recruiting

Triplex Checkpoint Inhibitors Therapy for Advanced Solid Tumors

Posted: January 11th, 2022, 3:00pm GMT

Conditions: Lung Cancer; Liver Cancer; Colorectal Cancer; Pancreas Cancer; Ovary Cancer; Head and Neck Cancer; Breast Cancer; Gastric Cancer; Cervical Cancer; Esophageal Cancer; Sarcoma
Intervention: Drug: ipilimumab +pembrolizumab +durvalumab
Sponsor: Second Affiliated Hospital of Guangzhou Medical University
Recruiting

Combination of Pembrolizumab and Cabozantinib in Patients With Advanced Sarcomas

Posted: January 7th, 2022, 3:00pm GMT

Conditions: Soft Tissue Sarcoma Adult; Advanced Soft-tissue Sarcoma; Ewing Sarcoma; Osteosarcoma; Undifferentiated Pleomorphic Sarcoma
Intervention: Drug: Association of pembrolizumab + cabozantinib
Sponsors: Institut Bergonié; Ipsen; MSD France
Not yet recruiting

HDM201 and Pazopanib in Patients With P53 Wild-type Advanced/Metastatic Soft Tissue Sarcomas

Posted: January 6th, 2022, 3:00pm GMT

Conditions: Advanced Soft-tissue Sarcoma; Metastatic Soft-tissue Sarcoma
Interventions: Drug: Pazopanib; Drug: HDM201
Sponsors: Centre Leon Berard; Novartis; National Cancer Institute, France
Not yet recruiting

Computer Aided Diagnostic Tool on Computed Tomography Images for Diagnosis of Retroperitoneal Tumor in Children

Posted: January 5th, 2022, 3:00pm GMT

Conditions: Wilms' Tumor; Neuroblastoma; Teratoma; Lymphoma; Sarcoma; Germ Cell Tumor
Intervention: Diagnostic Test: Radiomic Algorithm
Sponsor: West China Hospital
Recruiting

Niraparib for the Treatment of Leiomyosarcoma

Posted: December 30th, 2021, 3:00pm GMT

Conditions: Locally Advanced Leiomyosarcoma; Metastatic Leiomyosarcoma; Stage III Retroperitoneal Sarcoma AJCC v8; Stage III Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8; Stage IIIA Retroperitoneal Sarcoma AJCC v8; Stage IIIA Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8; Stage IIIB Retroperitoneal Sarcoma AJCC v8; Stage IIIB Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8; Stage IV Retroperitoneal Sarcoma AJCC v8; Stage IV Soft Tissue Sarcoma of the Trunk and Extremities AJCC v8; Unresectable Leiomyosarcoma
Intervention: Drug: Niraparib Tosylate Monohydrate
Sponsor: Ohio State University Comprehensive Cancer Center
Not yet recruiting

Stereotactic Ablative Radiotherapy for Oligometastatic Hepatocellular Carcinoma

Posted: December 30th, 2021, 3:00pm GMT

Condition: Oligometastasis Hepatocellular Carcinoma
Intervention: Radiation: Stereotactic body radiotherapy
Sponsor: Yonsei University
Recruiting

Predictive Models of Treatment Responses and Survival Outcomes in Patients With Soft Tissue Sarcoma

Posted: December 28th, 2021, 3:00pm GMT

Condition: Soft Tissue Sarcoma
Intervention: Other: Prediction model
Sponsor: West China Hospital
Recruiting

Permalink for 'Mobility and Physical Activity in Adolescent and Young Adult Cancer Patients or Survivors at Risk for Cardiovascular Morbidity and Frailty, The MOBILE AYA Study'

Mobility and Physical Activity in Adolescent and Young Adult Cancer Patients or Survivors at Risk for Cardiovascular Morbidity and Frailty, The MOBILE AYA Study

Posted: December 23rd, 2021, 3:00pm GMT

Conditions: Central Nervous System Neoplasm; Hodgkin Lymphoma; Malignant Solid Neoplasm; Non-Hodgkin Lymphoma; Sarcoma
Intervention: Other: Smartphone Application
Sponsors: M.D. Anderson Cancer Center; National Cancer Institute (NCI)
Not yet recruiting

Preoperative IMRT With Concurrent Anlotinib for Localised Extremity or Trunk Sarcoma

Posted: December 22nd, 2021, 3:00pm GMT

Conditions: Sarcoma,Soft Tissue; Extremity; Trunk; Anlotinib; Intensity-modulated Radiotherapy; Major Wound Complications
Intervention: Drug: Anlotinib hydrochloride
Sponsor: Chinese Academy of Medical Sciences
Recruiting

Permalink for 'Nanatinostat Plus Valganciclovir in Patients With Advanced EBV+ Solid Tumors, and in Combination With Pembrolizumab in EBV+ RM-NPC'

Nanatinostat Plus Valganciclovir in Patients With Advanced EBV+ Solid Tumors, and in Combination With Pembrolizumab in EBV+ RM-NPC

Posted: December 22nd, 2021, 3:00pm GMT

Conditions: Nasopharyngeal Carcinoma; EBV-Related Gastric Carcinoma; EBV-Related Leiomyosarcoma; EBV Related Carcinoma; EBV-Related Sarcoma
Interventions: Drug: Nanatinostat; Drug: Pembrolizumab; Drug: Valganciclovir
Sponsor: Viracta Therapeutics, Inc.
Recruiting

A Study of PRT2527 in Patients With Advanced Solid Tumors

Posted: December 16th, 2021, 3:00pm GMT

Conditions: Sarcomas; Castrate Resistant Prostate Cancer; Hormone Receptor-positive Breast Cancer; Triple Negative Breast Cancer; Solid Tumors With Known MYC Amplification
Intervention: Drug: PRT2527
Sponsor: Prelude Therapeutics
Not yet recruiting

Study of Pembrolizumab and M032 (NSC 733972)

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

Conditions: Glioblastoma Multiforme; Anaplastic Astrocytoma; Gliosarcoma
Interventions: Drug: M032; Drug: Pembrolizumab
Sponsor: University of Alabama at Birmingham
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
Recruiting

Permalink for 'Evaluation of Hyperbaric Oxygen Therapy on Wound Healing Following Management of Soft Tissue Sarcoma With Neo-Adjuvant Radiation and Surgical Resection'

Evaluation of Hyperbaric Oxygen Therapy on Wound Healing Following Management of Soft Tissue Sarcoma With Neo-Adjuvant Radiation and Surgical Resection

Posted: May 8th, 2017, 2:00pm GMT

Conditions: Sarcoma; Hyperbaric Oxygen Therapy
Intervention: Drug: Hyperbaric oxygen
Sponsor: Duke University
Recruiting

sEphB4-HSA in Treating Patients With Kaposi Sarcoma

Posted: June 15th, 2016, 2:00pm GMT

Condition: Skin Kaposi Sarcoma
Interventions: Other: Laboratory Biomarker Analysis; Other: Pharmacological Study; Behavioral: Quality-of-Life Assessment; Biological: Recombinant EphB4-HSA Fusion Protein
Sponsors: AIDS Malignancy Consortium; National Cancer Institute (NCI); Vasgene Therapeutics, Inc; The Emmes Company, LLC; University of Arkansas
Recruiting

Rare Cancer News & Clinical Trials » Sarcoma

Feeds

Sarcoma (218 unread)

Fetched: January 17th, 2022, 5:02am GMT by Erik A Williams

Fetched: January 17th, 2022, 5:02am GMT by Roberta Laranga

Fetched: January 17th, 2022, 5:02am GMT by Panwen Tian

Fetched: January 17th, 2022, 5:02am GMT by Daniel J Indelicato

Fetched: January 17th, 2022, 5:02am GMT by Ting Xue

Fetched: January 17th, 2022, 5:02am GMT by Wiebke K Guder

Fetched: January 16th, 2022, 5:01am GMT by Athan G Zavras

Fetched: January 16th, 2022, 5:01am GMT by Marco Baia

Fetched: January 16th, 2022, 5:01am GMT by Cameron J Overfield

Fetched: January 16th, 2022, 5:01am GMT by Tomoya Matsunobu

Fetched: January 16th, 2022, 5:01am GMT by Charlotte Le Roy

Fetched: January 16th, 2022, 5:01am GMT by Lina Pankratjevaite

Fetched: January 16th, 2022, 5:01am GMT by Diana Bell

Fetched: January 16th, 2022, 5:01am GMT by Meera H Patel

Fetched: January 16th, 2022, 5:01am GMT by Bo Mi Gil

Fetched: January 15th, 2022, 5:02am GMT by Leonardo P Marcal

Fetched: January 15th, 2022, 5:02am GMT by Ha Young Woo

Fetched: January 15th, 2022, 5:02am GMT by Mohammadreza Mortazavi

Fetched: January 15th, 2022, 5:02am GMT by Chaoxiang Yang

Fetched: January 15th, 2022, 5:02am GMT by Xin Long

Fetched: January 15th, 2022, 5:02am GMT by Huimin Bai

Fetched: January 15th, 2022, 5:02am GMT by Olli-Pekka Pulkka

Fetched: January 15th, 2022, 5:02am GMT by Colin Tuft

Fetched: January 15th, 2022, 5:02am GMT by Giuliana Rizzuto

Fetched: January 15th, 2022, 5:02am GMT by Christian Peter Kratz

Fetched: January 15th, 2022, 5:02am GMT by Safa Vatansever

Fetched: January 15th, 2022, 5:02am GMT by You Wu

Fetched: January 14th, 2022, 5:01am GMT by Satoru Nagase

Fetched: January 14th, 2022, 5:01am GMT by Kentaro Watanabe

Fetched: January 14th, 2022, 5:01am GMT by Giacomo G Baldi

Fetched: January 14th, 2022, 5:01am GMT by Caner Civan

Fetched: January 14th, 2022, 5:01am GMT by Munita Bal

Fetched: January 14th, 2022, 5:01am GMT by Corina Grigoriu

Fetched: January 14th, 2022, 5:01am GMT by Konstantinos Sapalidis

Fetched: January 14th, 2022, 5:01am GMT by Haoyu Ren

Fetched: January 14th, 2022, 5:01am GMT by Maria Leonor Enei

Fetched: January 14th, 2022, 5:01am GMT by He Jiang

Fetched: January 14th, 2022, 5:01am GMT by Simin Wu

Fetched: January 14th, 2022, 5:01am GMT by Aileen Y Chang

Fetched: January 14th, 2022, 5:01am GMT by Meijia Qian

Fetched: January 14th, 2022, 5:01am GMT by Shunsuke Yahiro

Fetched: January 14th, 2022, 5:01am GMT by Eren Müngen

Fetched: January 14th, 2022, 5:01am GMT by Tracy Gieger

Fetched: January 14th, 2022, 5:01am GMT by Ryo Karakawa

Fetched: January 14th, 2022, 5:01am GMT by Linus Lee

Fetched: January 14th, 2022, 5:01am GMT by Deanna Ng

Fetched: January 14th, 2022, 5:01am GMT by Mahesh Goel

Fetched: January 14th, 2022, 5:01am GMT by Xi-Zhi Wen

Fetched: January 14th, 2022, 5:01am GMT by COVID Surg Collaborative

Fetched: January 14th, 2022, 5:01am GMT by Janice M Mehnert

Fetched: January 14th, 2022, 5:01am GMT by Matias Jurado

Fetched: January 14th, 2022, 5:01am GMT by Wenzhe Zhao

Fetched: January 14th, 2022, 5:01am GMT by Zhichao Tian

Fetched: January 13th, 2022, 5:02am GMT by Kristian Larson

Fetched: January 13th, 2022, 5:02am GMT by Mitchell S Fourman

Fetched: January 13th, 2022, 5:02am GMT by Maria Danieli

Fetched: January 13th, 2022, 5:02am GMT by Robert M Kwee

Fetched: January 13th, 2022, 5:02am GMT by Li-Yu Chen

Fetched: January 13th, 2022, 5:02am GMT by Lei Han

Fetched: January 13th, 2022, 5:02am GMT by Scott Richard Gilbertson

Fetched: January 13th, 2022, 5:02am GMT by C Russo

Fetched: January 13th, 2022, 5:02am GMT by Sharada T Rajan

Fetched: January 13th, 2022, 5:02am GMT by Sundaramoorthy Revathidevi

Fetched: January 13th, 2022, 5:02am GMT by Klaire Yixin Fjæstad

Fetched: January 13th, 2022, 5:02am GMT by Sylwia Kozak

Fetched: January 13th, 2022, 5:02am GMT by Francois Bertucci

Fetched: January 13th, 2022, 5:02am GMT by Alexander Klein

Fetched: January 13th, 2022, 5:02am GMT by Nianyu Xue

Fetched: January 13th, 2022, 5:02am GMT by Cuicui Li

Fetched: January 12th, 2022, 5:01am GMT by Sule Canberk

Fetched: January 12th, 2022, 5:01am GMT by Micah Gibson

Fetched: January 12th, 2022, 5:01am GMT by John Doupis

Fetched: January 12th, 2022, 5:01am GMT by Malcolm Hart Squires