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PubMed - Angiosarcoma (100 unread)

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Contrast-Enhanced Ultrasonographic Features of a case of Primary Splenic Angiosarcoma

Fetched: June 19th, 2021, 5:00am GMT by Chao Hou

Pol Arch Intern Med. 2021 Jun 18. doi: 10.20452/pamw.16034. Online ahead of print.

NO ABSTRACT

PMID:34142767 | DOI:10.20452/pamw.16034

Case report: Haemoperitoneum secondary to acute rupture of primary hepatic angiosarcoma

Fetched: June 18th, 2021, 5:00am GMT by Boyoung Kim

Int J Surg Case Rep. 2021 Jun 10;84:106090. doi: 10.1016/j.ijscr.2021.106090. Online ahead of print.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Primary hepatic angiosarcoma (PHA) is a rare and aggressive liver malignancy of endothelial cell origin and is associated with poor outcome. Pre-operative confirmation of the diagnosis is challenging, as clinical and radiological findings are generally non-specific. Very rarely, spontaneous haemoperitoneum may occur due to the spontaneous rupture of previously undiagnosed PHA.

CASE PRESENTATION: We describe a case of a 28-year-old male with haemoperitoneum due to the rupture of previously undiagnosed PHA. After failing to respond to the non-operative measures, the patient underwent emergency partial liver resection and recovered without any post-operative complications. Histopathological examination of the specimen confirmed the diagnosis of PHA. Two months after the operation, the patient represented with advanced metastatic disease and disseminated intravascular coagulation (DIC). The patient died one month after discharge.

CLINICAL DISCUSSION: A patient with PHA presents a diagnostic challenge due to its rare incidence and non-specific clinical findings. Spontaneous intra-abdominal haemorrhage can occur due to PHA rupture and carries a dismal prognosis. In addition to emergency haemorrhage control, complete surgical resection with clear margins is the definitive treatment to date, however, most cases of PHA are unresectable at diagnosis and recurrence is common even after complete resection.

CONCLUSION: PHA is associated with very poor outcomes, due to its rapid progression, early recurrence, and metastatic nature. The median survival is approximately 5 months. Haemoperitoneum secondary to rupture of previously undiagnosed PHA is uncommon and is a poor prognostic indicator. Complete surgical resection of the disease is challenging and there is no established treatment.

PMID:34139418 | DOI:10.1016/j.ijscr.2021.106090

Multi-disciplinary frameworks for the treatment of primary cardiac sarcoma

Fetched: June 17th, 2021, 5:00am GMT by Randy M Stevens

J Card Surg. 2021 Jun 15. doi: 10.1111/jocs.15742. Online ahead of print.

NO ABSTRACT

PMID:34131956 | DOI:10.1111/jocs.15742

A case of metastatic primary cardiac angiosarcoma

Fetched: June 17th, 2021, 5:00am GMT by C Intrator

J R Coll Physicians Edinb. 2021 Jun;51(2):156-159. doi: 10.4997/JRCPE.2021.213.

ABSTRACT

We report a 49-year-old Southeast Asian woman diagnosed with metastatic primary right atrial angiosarcoma (PCA) and the difficulties encountered in this diagnosis and its subsequent management. Diagnosis of PCA is often delayed due to non-specific clinical presentation of patients. These tumours often present once metastatic spread has occurred, restricting treatment options and leading to very poor prognosis. Patients undergo a multidisciplinary team (MDT) approach involving chemotherapy, radiotherapy and, if eligible, surgery, but evidence-based treatment guidelines have yet to be established due to the rarity of the tumour.

PMID:34131674 | DOI:10.4997/JRCPE.2021.213

Surgical treatment outcomes of primary hepatic sarcomas: A single-center experience

Fetched: June 17th, 2021, 5:00am GMT by Sang Jin Kim

World J Hepatol. 2021 May 27;13(5):584-594. doi: 10.4254/wjh.v13.i5.584.

ABSTRACT

BACKGROUND: Primary hepatic sarcoma is a rare tumor originated from mesenchymal tissue. There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing. Unlike hepatocellular carcinoma, outcome of primary hepatic sarcoma is not well-known due to it's rarity. However, with development of medical technology, surgical treatment may lead to better survival.

AIM: To investigate the surgical outcomes of primary hepatic sarcoma, we gathered and analyzed the cases of a single institute.

METHODS: From August 2001 to September 2016, a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure, early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma. Baseline characteristics, tumor characteristics such as tumor pathology, size and number, surgical and adjuvant treatments were reviewed. Tumor recurrence, and patient survival were analyzed with retrospective approach.

RESULTS: The enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma. All patients experienced tumor recurrence at a median of 52 post-operative days. Only two patients survived and the 5-year survival rate was 29.6%. One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years. One patient with undifferentiated sarcoma received Rt. lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor. Two patients who received living donor liver transplantation due to angiosarcoma died. Only adjuvant therapy was associated with survival gain (P = 0.002).

CONCLUSION: Patients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy, even though the outcome remains relatively poor.

PMID:34131472 | PMC:PMC8173340 | DOI:10.4254/wjh.v13.i5.584

A RETROSPECTIVE STUDY OF NEOPLASIA IN NONDOMESTIC FELIDS IN HUMAN CARE, WITH A COMPARATIVE LITERATURE REVIEW

Fetched: June 17th, 2021, 5:00am GMT by Amélie Mathieu

J Zoo Wildl Med. 2021 Jun;52(2):413-426. doi: 10.1638/2020-0077.

ABSTRACT

This retrospective study of neoplasia in nondomestic felids in human care presents the cases diagnosed at Northwest ZooPath (NWZP), Monroe, Washington, from 1998 to 2017 in conjunction with a scoping literature review. The 554 neoplasms identified in 20 species in the NWZP archive were combined with the 984 neoplasms identified in those same species in the published literature. Some of the cases identified in the literature were from the NWZP archive. Based on this review, mammary adenocarcinoma (183/1,483, 12.3%), lymphoma (89/1,483, 6.0%), squamous cell carcinoma (85/1,483, 5.7%), pheochromocytoma (57/1,483, 3.8%), and thyroid adenoma (57/1,483, 3.8%) are the most frequently reported neoplasms in nondomestic felids in human care. Apparent species predilections for neoplasia include mammary adenocarcinoma in tigers, jaguars, lions, and jungle cats; lymphoma in lions and tigers; squamous cell carcinoma in snow leopards; pheochromocytoma in clouded leopards; ovarian adenocarcinoma in jaguars; cholangiocarcinoma in lions and tigers; multiple myeloma in tigers; bronchoalveolar adenocarcinoma in cougars and lions; hemangiosarcoma, hepatocellular carcinoma, and gastrointestinal adenocarcinoma in lions; mesothelioma in clouded leopards, lions, and tigers; myelolipoma and cutaneous mast cell tumor in cheetahs; soft tissue sarcomas in tigers; and transitional cell carcinoma of the urinary bladder in fishing cats.

PMID:34130383 | DOI:10.1638/2020-0077

Hemoptysis as the first sign of angiosarcoma - an extremely aggressive cardiac tumor

Fetched: June 15th, 2021, 5:00am GMT by Aleksandra Ciepłucha

Kardiol Pol. 2021;79(5):589-590. doi: 10.33963/KP.15920.

NO ABSTRACT

PMID:34125941 | DOI:10.33963/KP.15920

Posterior Mediastinal Epithelioid Angiosarcoma Arising in Schwannoma: A Case Report and Review of the Literature

Fetched: June 15th, 2021, 5:00am GMT by Yingming Xiang

Front Surg. 2021 May 28;8:666389. doi: 10.3389/fsurg.2021.666389. eCollection 2021.

ABSTRACT

Epithelioid angiosarcoma arising in schwannoma is an extremely rare mesenchymal tumor that accounts for only 1 to 2% of all sarcomas. This type of tumor occurs in all parts of the body, most often in the skin and soft tissues and rarely in the mediastinum. The present study describes the case of an asymptomatic, 58-year-old male who presented with epithelioid angiosarcoma in the posterior mediastinum during a physical examination. Enhanced computed tomography of the chest revealed a 3.5 × 3.1-cm mass in the posterior mediastinum. Thoracoscopic mediastinal mass resection was performed under general anesthesia due to the possibility that the tumor was malignant. Pathological examination revealed the presence of angiosarcoma and schwannoma components. Immunohistochemical staining for cluster of differentiation (CD) 31, CD34, early growth response (EGR), vimentin, Sry-related HMG box (SOX)-10 and S-100 was strongly positive. The patient recovered and was discharged on postoperative day 5. Two months postsurgery, the patient returned for evaluation, and no evidence of tumor recurrence was observed.

PMID:34124136 | PMC:PMC8192798 | DOI:10.3389/fsurg.2021.666389

Successful perioperative management of a primary pulmonary arterial angiosarcoma - Case report

Fetched: June 14th, 2021, 5:00am GMT by Ashish Anil Bartakke

Braz J Anesthesiol. 2021 Jun 9:S0104-0014(21)00232-3. doi: 10.1016/j.bjane.2021.05.011. Online ahead of print.

ABSTRACT

INTRODUCTION: Primary pleomorphic pulmonary angiosarcomas are extremely rare tumors which could be easily mistaken for pulmonary emboli.

BACKGROUND AND FINDINGS: We describe the successful perioperative management of a patient with a pulmonary arterial mass which turned out to be a primary pulmonary angiosarcoma. The severe pulmonary hypertension was a particular challenge compounded with the site and adhesions of the tumor, and pulmonary hemorrhage. The procedure was successfully performed with strict hemodynamic control ensuring stable systemic and pulmonary arterial pressures using perioperative transesophageal echocardiography to continuously monitor cardiac function, along with other standard cardiac surgical monitors including depth of anesthesia monitoring.

CONCLUSION: Tight hemodynamic control, ensuring stable pulmonary arterial pressures using perioperative echocardiography, and thorough preparation with measures to reduce and prevent increase in pulmonary arterial pressure along with close communication within the multi-disciplinary team are essential for successful management of patients with this pathology.

PMID:34118262 | DOI:10.1016/j.bjane.2021.05.011

Aortic Angiosarcoma in Association with Endovascular Aneurysm Repair: Case Report and Review of the Literature

Fetched: June 13th, 2021, 5:00am GMT by Kaori Takamura

Am J Case Rep. 2021 Jun 12;22:e931740. doi: 10.12659/AJCR.931740.

ABSTRACT

BACKGROUND Primary aortic sarcoma often poses diagnostic challenges for pathologists and clinicians because of a very low incidence and controversy over nomenclature and definition. We report a case of aortic angiosarcoma in association with a graft. We also conducted a clinicopathological review of cases of primary aortic sarcomas associated with implanted grafts. CASE REPORT The patient was an 82-year-old woman. She underwent thoracic endovascular aneurysm repair (TEVAR) at age 78 because of an aneurysm in the descending aorta. Approximately 4 years after the TEVAR, computed tomography revealed a type II endoleak and expansion of the aneurysm. Her c-reactive protein level rose to 34 mg/dL, and Ga scintigraphy showed 67Ga accumulation at the aneurysm. She had fever up to 39°C, and a stent graft infection was suspected. Despite administration of antibiotics, her condition deteriorated, and she died. Postmortem examination identified epithelioid aortic angiosarcoma at the aorta with aneurysm repair and the graft, and the aortic angiosarcoma invaded the left lower lobe of the lung. CONCLUSIONS Our clinicopathological review revealed that the proper clinical diagnosis was very difficult owing to confusion of aortic sarcoma after the implantation with the infected graft, atypical endoleak, or pseudoaneurysm. The histological diagnosis was ambiguous because immunohistochemical and genetic studies were not adequately conducted. Overall prognosis of aortic sarcoma is poor as most patients die within a year, with no effective treatments. It is hoped that recent projects for genomic medicine will provide useful insights about the diagnosis and treatment of these cancers.

PMID:34118148 | DOI:10.12659/AJCR.931740

The role of radiation therapy in the management of cutaneous malignancies. Part II: When is radiation therapy indicated?

Fetched: June 13th, 2021, 5:00am GMT by Kelly M Wilmas

J Am Acad Dermatol. 2021 Jun 8:S0190-9622(21)01104-X. doi: 10.1016/j.jaad.2021.05.057. Online ahead of print.

ABSTRACT

Radiation therapy may be performed for a variety of cutaneous malignancies depending on patient health status, tumor clinical and histologic features, patient preference, and resource availability. Dermatologists should be able to recognize the clinical scenarios in which radiation therapy is appropriate as this may reduce morbidity, decrease risk of disease recurrence, and improve quality of life. The second article in this 2-part continuing medical education series focuses on the most common indications for radiation therapy in the treatment of basal cell carcinoma, cutaneous squamous cell carcinoma, dermatofibrosarcoma protuberans, Merkel cell carcinoma, Kaposi sarcoma, angiosarcoma, cutaneous lymphoma, melanoma, undifferentiated pleomorphic sarcoma, and sebaceous carcinoma.

PMID:34116100 | DOI:10.1016/j.jaad.2021.05.057

An autopsy case of hepatic angiosarcoma diagnosed with ultrasonography-guided percutaneous needle biopsy of the liver

Fetched: June 11th, 2021, 5:00am GMT by Masayoshi Takami

Nihon Shokakibyo Gakkai Zasshi. 2021;118(6):562-570. doi: 10.11405/nisshoshi.118.562.

ABSTRACT

Contrast medium-enhanced computed tomography revealed a mass in the liver of a 65-year-old man. The edge but not the center of the mass was enhanced. Ultrasonography-guided percutaneous needle biopsy revealed the diagnosis of angiosarcoma of the liver, and it was treated with chemotherapy. Angiosarcoma of the liver has various appearances on imaging and is not often diagnosed while patients are alive. If the tumor is difficult to diagnose by imaging and thought to be unresectable, a biopsy can help in guiding treatment, but treatment should be adapted with caution.

PMID:34108356 | DOI:10.11405/nisshoshi.118.562

Unusual presentation of metastatic radiogenic breast angiosarcoma

Fetched: June 10th, 2021, 5:00am GMT by Fabio Carboni

Jpn J Clin Oncol. 2021 Jun 9:hyab088. doi: 10.1093/jjco/hyab088. Online ahead of print.

NO ABSTRACT

PMID:34105728 | DOI:10.1093/jjco/hyab088

Current Management of Angiosarcoma: Recent Advances and Lessons From the Past

Fetched: June 8th, 2021, 5:00am GMT by Vaia Florou

Curr Treat Options Oncol. 2021 Jun 7;22(7):61. doi: 10.1007/s11864-021-00858-9.

ABSTRACT

Despite their rarity, angiosarcomas are one of the most aggressive soft tissue sarcomas. Management can often be challenging due to their location and infiltrative nature. A multidisciplinary treatment approach is always warranted, but the recurrence remains high even for localized tumors despite multimodality treatment. In the metastatic setting, cytotoxic chemotherapies, targeted therapies, and, more recently, immunotherapy are used. The sequence of systemic therapies remains currently a topic of active investigation. Over the last couple of years, there have been significant advances in understanding angiosarcoma biology, most notably via patient-driven initiatives like the Angiosarcoma Project. The knowledge derived from such translational work has led to identifying potential biomarkers of response to treatments and exploring new therapeutic avenues. More clinical trials are underway to expand treatment options and improve patient outcomes.

PMID:34097172 | DOI:10.1007/s11864-021-00858-9

Permalink for 'Epithelioid Haemangioma of Bone: A Case Series and Comprehensive Literature Review Reappraising the Diagnostic Classification of All Epithelioid Vascular Neoplasms of Bone'

Epithelioid Haemangioma of Bone: A Case Series and Comprehensive Literature Review Reappraising the Diagnostic Classification of All Epithelioid Vascular Neoplasms of Bone

Fetched: June 8th, 2021, 5:00am GMT by Subramaniam Ramkumar

Cureus. 2021 Jun 1;13(6):e15371. doi: 10.7759/cureus.15371.

ABSTRACT

Epithelioid vascular neoplasms of the bone are classified by the World Health Organization (WHO) into only two tiers: low-grade epithelioid hemangioma (EH) and a more malignant category including both epithelioid hemangioendothelioma and epithelioid angiosarcoma. The World Health Organization defines bone EH as a locally aggressive neoplasm with no connotation of benign or intermediate malignancy. We reviewed three cases of EH in our lab archives with the perspective of appraising their histomorphological approach toward diagnosis. Patients were in the age range of 15-25 years. The site of the neoplasms ranged from the carpal bones to the metatarsal bones. Histomorphological examination of the lesions showed a nodular growth pattern of a vascular neoplasm without demonstrable vessel origin. The vasoformative area increased from the center to the periphery, with prominent epithelioid morphology of the endothelial cells at the periphery and an associated inflammatory infiltrate comprising eosinophils, lymphocytes, and plasma cells. The growth pattern was diffuse, with extension into the deeper dermis of overlying skin.

PMID:34094789 | PMC:PMC8168629 | DOI:10.7759/cureus.15371

Cutaneous symplastic hemangioma: a series of four cases

Fetched: June 6th, 2021, 5:00am GMT by Derek Frew

J Cutan Pathol. 2021 Jun 5. doi: 10.1111/cup.14075. Online ahead of print.

ABSTRACT

Symplastic hemangiomas are benign vascular lesions that demonstrate atypia in vascular smooth muscle and interstitial cells with sparing of endothelial cells. We present four cases of this rare tumor. The patients (2M;2F) ranged in age from 57-83 years (median 74); lesions were located on the leg (n=3) and back (n=1), and ranged from 6-8 mm. SH were well-circumscribed and dermal-based, often with an epidermal collarette (3/4). They were characterized by the presence of variably atypical, hyperchromatic/pleomorphic epithelioid to spindled cells within vascular walls (3/4) and/or perivascular stroma (4/4). Atypical multinucleated cells were present in 3 of 4 cases. The overall mitotic rate was low (0-2 mitotic figures per 10 HPFs; mean 0.75 per 10 HPFs), but atypical mitotic figures were seen in 2 of 4 cases. Atypical cells were negative for SMA (0/2), desmin (0/2), AE1/3 (0/2), and CAM5.2 (0/1). ERG, CD31, and CD34 stains were positive in endothelial cells but negative in atypical cells (4/4). Lesional cells and vessels were negative for podoplanin (3/3). Symplastic hemangioma is a benign tumor with bizarre atypia that may be mistaken for malignancy. We present four cases of this rare entity to increase awareness of this tumor and discuss the differential diagnosis. This article is protected by copyright. All rights reserved.

PMID:34089529 | DOI:10.1111/cup.14075

Photodynamic therapy of classic Kaposi's sarcoma with video-fluorescence control

Fetched: June 6th, 2021, 5:00am GMT by A A Shiryaev

Photodiagnosis Photodyn Ther. 2021 Jun 1;35:102378. doi: 10.1016/j.pdpdt.2021.102378. Online ahead of print.

ABSTRACT

BACKGROUND: Kaposi's sarcoma (multiple idiopathic hemorrhagic sarcoma, Kaposi's angiosarcoma) is an angioproliferative neoplasm of endothelial origin associated with human herpes virus 8 (HHV - 8) and human immunodeficiency virus (HIV). The incidence of the classical form of Kaposi's sarcoma (КS) varies significantly in different geographical areas from 0.14/1 million people (both men and women) to 10.5 per 1 million men and 2.7 per 1 million women. The onset of КS is typical at the age of 35-39 in men and 25-39 years in women.

CASE REPORT: A case of successful treatment of a recurrent non-HIV and non-HHV-8 Kaposi's sarcoma with PDT in a 79-year old man.

RESULTS: After the recieved photodynamic treatment, complete pathologic response was achieved, i.e., Grade 5 tumor response according to Miller-Payne histological grading system (1999). The overall condition has significantly improved with no clinical tumor signs.

CONCLUSION: This report describes a good outcome with PDT in the treatment of Kaposi's sarcoma. The results obtained are initial, but encouraging, demonstrating good tolerance, safety and high efficacy of PDT in classical КS. The successful use of PDT in cutaneous manifestations of HIV-associated KS is also described in literature.

PMID:34087467 | DOI:10.1016/j.pdpdt.2021.102378

Rare Mimic of Angiosarcoma: Erythema Ab Igne with Reactive Angiomatosis

Fetched: June 5th, 2021, 5:00am GMT by Nathan M Johnson

J Cutan Pathol. 2021 Jun 4. doi: 10.1111/cup.14072. Online ahead of print.

ABSTRACT

Erythema ab igne is an uncommon physical dermatosis that presents with localized patches of reticulated erythema and hyperpigmentation corresponding with the underlying dermal venous plexus. The rash occurs in response to chronic heat exposure that does not meet the threshold for thermal burn of the skin. The histopathologic findings are characterized by atrophy and thinning of the epidermis, focal hyperkeratosis, and keratinocyte atypia. The dermis displays dilated capillaries, evidence of pigment incontinence, and prominent elastotic material. We report a case of a 65-year-old male who presented to his primary care physician with a one-year history of reticular erythema and hyperpigmentation with focal ulceration on his right lateral leg. Histopathology on biopsy revealed mild hyperkeratosis and focal epidermal atrophy; however, the most striking finding was a proliferation of dermal vascular spaces lined by pleomorphic endothelial cells and numerous mitotic figures, which was morphologically compatible with angiosarcoma. However, clinicopathologic correlation and immunostaining revealed an actual diagnosis of erythema ab igne with reactive angiomatosis. Reactive angiomatosis - morphologically mimicking angiosarcoma - is a rarely reported feature of severe erythema ab igne, and dermatopathologists should be aware of this possibility to avoid misdiagnosis of erythema ab igne as angiosarcoma. This article is protected by copyright. All rights reserved.

PMID:34086363 | DOI:10.1111/cup.14072

PARP inhibition in UV-associated angiosarcoma preclinical models

Fetched: June 5th, 2021, 5:00am GMT by Marije E Weidema

J Cancer Res Clin Oncol. 2021 Jun 3. doi: 10.1007/s00432-021-03678-4. Online ahead of print.

ABSTRACT

PURPOSE: Angiosarcoma (AS) is a rare vasoformative sarcoma, with poor overall survival and a high need for novel treatment options. Clinically, AS consists of different subtypes, including AS related to previous UV exposure (UV AS) which could indicate susceptibility to DNA damage repair inhibition. We, therefore, investigated the presence of biomarkers PARP1 (poly(ADP-ribose)polymerase-1) and Schlafen-11 (SLFN11) in UV AS. Based on experiences in other sarcomas, we examined (combination) treatment of PARP inhibitor (PARPi) olaparib and temozolomide (TMZ) in UV AS cell lines.

METHODS: Previously collected UV AS (n = 47) and non-UV AS (n = 96) patient samples and two UV AS cell lines (MO-LAS and AS-M) were immunohistochemically assessed for PARP1 and SLFN11 expression. Both cell lines were treated with single agents PARPi olaparib and TMZ, and the combination treatment. Next, cell viability and treatment synergy were analyzed. In addition, effects on apoptosis and DNA damage were examined.

RESULTS: In 46/47 UV AS samples (98%), PARP1 expression was present. SLFN11 was expressed in 80% (37/46) of cases. Olaparib and TMZ combination treatment was synergistic in both cell lines, with significantly increased apoptosis compared to single agent treatment. Furthermore, a significant increase in DNA damage marker γH2AX was present in both cell lines after combination therapy.

CONCLUSION: We showed combination treatment of olaparib with TMZ was synergistic in UV AS cell lines. Expression of PARP1 and SLFN11 was present in the majority of UV AS tumor samples. Together, these results suggest combination treatment of olaparib and TMZ is a potential novel AS subtype-specific treatment option for UV AS patients.

PMID:34085099 | DOI:10.1007/s00432-021-03678-4

Living donor liver transplantation for hepatic malignancies in children

Fetched: June 3rd, 2021, 5:00am GMT by Aurore Pire

Pediatr Transplant. 2021 Jun 2:e14047. doi: 10.1111/petr.14047. Online ahead of print.

ABSTRACT

BACKGROUND: Living donor liver transplantation is a treatment option for unresectable hepatic tumors in children.

METHODS: We enrolled 45 living donor transplantations performed between 1993 and 2018 for liver malignacies, which included hepatoblastoma (n = 33), hepatocellular carcinoma (n = 10), hepatic angiosarcoma (n = 1), and rhabdomyosarcoma (n = 1).

RESULTS: No mortality or major morbidities were encountered in any donor, and the complication rate was 9%. In the hepatoblastoma group, 5-year overall and event-free survival rate in recipients was 87.4% and 75.8%, respectively, and mortality was significantly higher in patients after rescue transplantation (p = .001). Inferior vena cava replacement in these recipients appeared to be associated with reduced mortality (p = .034), but this was not confirmed when rescue patients were excluded (p = .629). In hepatocellular carcinoma group, both 5-year overall and event-free survival rates were 75.4% each, and invasion of hepatic veins was significantly associated with increased risk of recurrence and death (p = .028). The patient with rhabdomyosarcoma died from EBV-induced lymphoma 2 months after transplantation. The patient with angiosarcoma was in complete remission at the last follow-up. Overall, 5-year graft survival rate was 81.3%, and one patient underwent re-transplantation due to chronic rejection.

CONCLUSIONS: Pediatric oncological liver transplantation has become a key player in the management of malignancies with cancer cure in 84% of patients in this series. Living donor liver transplantation for pediatric recipients with unresectable tumors might be a beneficial surgical option, which is technically safe for donors and recipients, thus, allowing timely planning according to chemotherapy protocols.

PMID:34076944 | DOI:10.1111/petr.14047

Permalink for 'Plasma Metabolomics Analysis of Polyvinyl Chloride Workers Identifies Altered Processes and Candidate Biomarkers for Hepatic Hemangiosarcoma and Its Development'

Plasma Metabolomics Analysis of Polyvinyl Chloride Workers Identifies Altered Processes and Candidate Biomarkers for Hepatic Hemangiosarcoma and Its Development

Fetched: June 3rd, 2021, 5:00am GMT by John J Guardiola

Int J Mol Sci. 2021 May 11;22(10):5093. doi: 10.3390/ijms22105093.

ABSTRACT

BACKGROUND: High-level occupational vinyl chloride (VC) exposures have been associated with hepatic hemangiosarcoma, which typically develops following a long latency period. Although VC is genotoxic, a more comprehensive mode of action has not been determined and diagnostic biomarkers have not been established. The purpose of this study is to address these knowledge gaps through plasma metabolomics.

METHODS: Plasma samples from polyvinyl chloride polymerization workers who developed hemangiosarcoma (cases, n = 15) and VC exposure-matched controls (n = 17) underwent metabolomic analysis. Random forest and bioinformatic analyses were performed.

RESULTS: Cases and controls had similar demographics and routine liver biochemistries. Mass spectroscopy identified 606 known metabolites. Random forest analysis had an 82% predictive accuracy for group classification. 60 metabolites were significantly increased and 44 were decreased vs. controls. Taurocholate, bradykinin and fibrin degradation product 2 were up-regulated by greater than 80-fold. The naturally occurring anti-angiogenic phenol, 4-hydroxybenzyl alcohol, was down-regulated 5-fold. Top affected ontologies involved: (i) metabolism of bile acids, taurine, cholesterol, fatty acids and amino acids; (ii) inflammation and oxidative stress; and (iii) nicotinic cholinergic signaling.

CONCLUSIONS: The plasma metabolome was differentially regulated in polyvinyl chloride workers who developed hepatic hemangiosarcoma. Ontologies potentially involved in hemangiosarcoma pathogenesis and candidate biomarkers were identified.

PMID:34065028 | PMC:PMC8150673 | DOI:10.3390/ijms22105093

CT Findings of Pulmonary Metastases from Primary Cardiac Angiosarcoma

Fetched: June 2nd, 2021, 5:00am GMT by Yan Chen

Curr Med Imaging. 2021 May 21. doi: 10.2174/1573405617666210521151753. Online ahead of print.

ABSTRACT

BACKGROUND: Primary cardiac angiosarcoma is a rare malignancy with high predilection to involve surrounding structures such as pulmonary metastases. We analysed the chest computed tomography (CT) imaging features of patients diagnosed with primary cardiac angiosarcoma with pulmonary metastases in this study.

METHODS: This study retrospectively reviewed 12 patients with confirmed primary cardiac angiosarcoma, out of which eight (all men) with pulmonary metastasis were included in the analysis. The patients' age ranged from 17 to 74 (mean: 48) years. CT was performed in all patients with unenhanced, contrast-enhanced, and both scans were done in 1, 3 and 4 patients, respectively.

RESULTS: Nodular lesions were observed in 7 patients with multiple solid nodules observed in 6 out of 7 patients. A solitary solid nodule was found in the remaining patient in the upper lobe and apical segment of the right lung with a diameter of 11.7 mm. All solid nodules were distributed along with bronchovascular bundles in the lungs, and their maximum diameter ranged from 2.3 to 19.9 mm. Nodules larger than 10 mm in diameter were heterogeneously enhanced on contrast-enhanced CT images (5/8 patients), whereas those smaller than 10 mm were homogeneously enhanced (3/8 patients). Other imaging features, namely the tree-in-bud pattern, emphysema, pleural effusion, and mediastinal lymph node enlargement, were observed in 4, 3, 3, and 2 patients, respectively.

CONCLUSION: CT enhancement features of pulmonary metastasis in patients with primary cardiac angiosarcoma depend on the size of pulmonary nodules, with larger ones being heterogeneous and smaller ones homogeneous. Other signs are less noticed.

PMID:34061006 | DOI:10.2174/1573405617666210521151753

Tertiary lymphoid structures correlate with better prognosis in cutaneous angiosarcoma

Fetched: June 2nd, 2021, 5:00am GMT by Tetsuya Magara

J Dermatol Sci. 2021 May 25:S0923-1811(21)00112-2. doi: 10.1016/j.jdermsci.2021.05.006. Online ahead of print.

NO ABSTRACT

PMID:34059409 | DOI:10.1016/j.jdermsci.2021.05.006

Intravascular histiocytosis: mimicker of cellulitis, angiosarcoma, inflammatory breast cancer, and others

Fetched: June 1st, 2021, 5:00am GMT by Emma F Johnson

Int J Dermatol. 2021 May 31. doi: 10.1111/ijd.15670. Online ahead of print.

ABSTRACT

BACKGROUND: Intravascular histiocytosis is an underrecognized reactive skin condition characterized by the clinical finding of poorly demarcated erythematous to violaceous patches and plaques. The diagnosis is confirmed by the histologic findings of intraluminal histiocytes on skin biopsy and exclusion of an alternative diagnosis.

METHODS: A review of patients with a histologic diagnosis of intravascular or intralymphatic histiocytosis and seen at Mayo Clinic, Rochester, Minnesota, from January 1, 2010, to October 10, 2020, was performed. Histologic and clinical information was collected from the medical records.

RESULTS: Nine patients were identified. Clinical impressions prior to biopsy varied widely, and no clinician included intravascular histiocytosis in the initial clinical differential diagnosis. Eight patients had preceding trauma to the affected area.

CONCLUSION: Intravascular histiocytosis remains a rare skin condition. Clinical identification remains low. Our cases add support to the hypothesis that intravascular histiocytosis is a reactive condition often preceded by trauma and/or surgery.

PMID:34057196 | DOI:10.1111/ijd.15670

Prognostic implications of PD-L1 expression in patients with angiosarcoma

Fetched: May 29th, 2021, 5:00am GMT by Jii Bum Lee

Future Sci OA. 2021 Mar 2;7(5):FSO691. doi: 10.2144/fsoa-2020-0211.

ABSTRACT

AIM: There are limited data on the feasibility of programmed death ligand-1 (PD-L1) expression as a prognostic biomarker in metastatic angiosarcoma.

PATIENTS & METHODS: We retrospectively collected and analyzed the data on PD-L1 expression in 70 angiosarcoma patients who were diagnosed at our center between 2005 and 2019.

RESULTS: Thirteen (19%) patients had PD-L1 expression. Metastatic angiosarcoma patients who were PD-L1-negative (n = 24) showed longer median progression-free survival (4.9 vs 1.6 months; p = 0.04) and median overall survival (OS; 10.9 vs 5.4 months; p = 0.01) than those who were PD-L1-positive (n = 4). PD-L1 status proved to be a significant factor for OS.

CONCLUSION: Metastatic angiosarcoma patients with PD-L1 expression showed shorter survival. PD-L1 status is an independent prognostic factor for OS in metastatic angiosarcoma patients.

PMID:34046193 | PMC:PMC8147824 | DOI:10.2144/fsoa-2020-0211

Iatrogenic immunosuppression can lead to prolonged viral shedding and absent immune response to COVID-19

Fetched: May 28th, 2021, 5:00am GMT by A Psaros Einberg

Acta Paediatr. 2021 May 27. doi: 10.1111/apa.15955. Online ahead of print.

ABSTRACT

In the era of the COVID-19 pandemic, paediatricians need to remember that immunocompromised children might experience prolonged viral shedding and impaired immunological response to SARS-CoV-2. We report a girl diagnosed with an unresectable angiosarcoma of the liver who underwent liver transplantation in June 2019. The basic immunosuppression protocol consisted of tacrolimus and prednisolone. Additionally, she was on adjuvant treatment with trametinib, a mitogen-activated protein kinase (MEK) inhibitor.

PMID:34043855 | DOI:10.1111/apa.15955

GNA14, GNA11, and GNAQ Mutations Are Frequent in Benign but Not Malignant Cutaneous Vascular Tumors

Fetched: May 28th, 2021, 5:00am GMT by Philipp Jansen

Front Genet. 2021 Apr 30;12:663272. doi: 10.3389/fgene.2021.663272. eCollection 2021.

ABSTRACT

Cutaneous vascular tumors consist of a heterogeneous group of benign proliferations, including a range of hemangiomas and vascular malformations, as well as heterogeneous groups of both borderline and malignant neoplasms such as Kaposi's sarcoma and angiosarcomas. The genetics of these tumors have been assessed independently in smaller individual cohorts making comparisons difficult. In our study, we analyzed a representative cohort of benign vascular proliferations observed in a clinical routine setting as well as a selection of malignant vascular proliferations. Our cohort of 104 vascular proliferations including hemangiomas, malformations, angiosarcomas and Kaposi's sarcoma were screened by targeted next-generation sequencing for activating genetic mutations known or assumed to be potentially relevant in vascular proliferations. An association analysis was performed for mutation status and clinico-pathological parameters. Frequent activating hotspot mutations in GNA genes, including GNA14 Q205, GNA11 and GNAQ Q209 were identified in 16 of 64 benign vascular tumors (25%). GNA gene mutations were particularly frequent (52%) in cherry (senile) hemangiomas (13 of 25). In angiosarcomas, activating RAS mutations (HRAS and NRAS) were identified in three samples (16%). No activating GNA or RAS gene mutations were identified in Kaposi's sarcomas. Our study identifies GNA14 Q205, GNA11 and GNAQ Q209 mutations as being the most common and mutually exclusive mutations in benign hemangiomas. These mutations were not identified in malignant vascular tumors, which could be of potential diagnostic value in distinguishing these entities.

PMID:34040639 | PMC:PMC8141909 | DOI:10.3389/fgene.2021.663272

Ovarian epithelioid angiosarcoma complicating pregnancy: a case report and review of the literature

Fetched: May 28th, 2021, 5:00am GMT by Xiaotong Peng

J Int Med Res. 2021 May;49(5):3000605211019641. doi: 10.1177/03000605211019641.

ABSTRACT

Epithelioid angiosarcoma is a rare and highly aggressive soft tissue angiosarcoma most commonly arising in the deep soft tissues. Given that abundant vascular cavities anastomose with each other, most angiosarcomas prone to metastasis recur quickly, and the overall prognosis is poor. We report a 25-year-old woman at 24 weeks' gestation who presented with a 1-month history of abdominal distension. Ultrasonography suggested a mass in the right adnexa, and she underwent two operations owing to uncontrolled intraperitoneal bleeding with progressive anemia. The right ovarian tumor and right adnexa were removed successively. Biopsy yielded a diagnosis of primary epithelioid angiosarcoma with mature cystic teratoma. The patient died from uncontrolled progressive bleeding 1 week after the second operation. This case revealed that epithelial angiosarcoma is a highly malignant endothelial cell tumor. The results of surgery and chemoradiotherapy tend to be poor, and the recurrence rate is high. The purpose of this study is to raise clinical awareness of epithelial angiosarcoma and its adverse events and to provide new ideas for the treatment of these adverse events. Immunohistochemical staining of pathological specimens can facilitate diagnosis. Pregnancy with malignant tumors may lead to rapid disease progression, extensive lesions, and a poor prognosis.

PMID:34039070 | PMC:PMC8168035 | DOI:10.1177/03000605211019641

Metastatic Cutaneous Angiosarcoma: A Rare Entity

Fetched: May 27th, 2021, 5:00am GMT by Javier Blanco Jimenez

Cureus. 2021 Apr 20;13(4):e14577. doi: 10.7759/cureus.14577.

ABSTRACT

We present a case of a 56-year-old female presented with a chief complaint of four months of dyspnea that had acutely worsened. The patient also reported a chronic right thigh wound. Chest radiograph on initial presentation demonstrated multiple bilateral rounded opacities. CT pulmonary angiogram demonstrated multiple bilateral rounded nodules seen on chest radiograph along with a right upper lobe pulmonary artery with near-complete compression secondary to large right hilar adenopathy. CT of right lower extremity showed evidence of ulceration with multiple conglomerate subcutaneous masses, predominately anteriorly and laterally. She underwent a bronchoscopy; a fine-needle aspiration of the subcarinal lymph node was taken. A surgical biopsy from the right thigh wound was also performed. A histological examination from the right thigh ulcer and subcarinal lymph node demonstrated high-grade spindle cell neoplasm, positive for CD-31 and consistent with angiosarcoma.

PMID:34035999 | PMC:PMC8135628 | DOI:10.7759/cureus.14577

Successful surgical resection of primary cardiac angiosarcoma: a case report

Fetched: May 27th, 2021, 5:00am GMT by Y L Qu

Zhonghua Xin Xue Guan Bing Za Zhi. 2021 May 24;49(5):513-515. doi: 10.3760/cma.j.cn112148-20200617-00490.

NO ABSTRACT

PMID:34034388 | DOI:10.3760/cma.j.cn112148-20200617-00490

Diffuse Cutaneous Angiosarcoma in the Scalp and Face Demonstrated on FDG PET/CT Imaging

Fetched: May 27th, 2021, 5:00am GMT by Liu Xiao

Clin Nucl Med. 2021 May 26. doi: 10.1097/RLU.0000000000003706. Online ahead of print.

ABSTRACT

Diffuse cutaneous angiosarcomas are rare. Herein, we reported FDG PET/CT findings of cutaneous angiosarcoma in a 59-year-old man. FDG PET/CT imaging was performed for staging, showing diffuse intense FDG uptake with an SUVmax of 19 in the scalp and face. Chemotherapy was recommended for this patient because surgery and radiotherapy were not appropriate due to the widespread skin involvement. Our case suggests that cutaneous angiosarcoma can present with diffuse skin involvement, and FDG PET/CT plays an important role in determining the treatment plan to avoid unnecessary surgery and radiotherapy.

PMID:34034311 | DOI:10.1097/RLU.0000000000003706

Zebrafish Tumor Graft Transplantation to Grow Tumors In Vivo That Engraft Poorly as Single Cell Suspensions

Fetched: May 26th, 2021, 5:00am GMT by Amanda Lipsitt

Zebrafish. 2021 May 24. doi: 10.1089/zeb.2021.0006. Online ahead of print.

ABSTRACT

Angiosarcoma is a clinically aggressive tumor with a high rate of mortality. It can arise in vascular or lymphatic tissues, involve any part of the body, and aggressively spread locally or metastasize. Angiosarcomas spontaneously develop in the tp53 deleted (tp53^del/del) zebrafish mutant. However, established protocols for tumor dissection and transplantation of single cell suspensions of angiosarcoma tumors result in inferior implantation rates. To resolve these complications, we developed a new tumor grafting technique for engraftment of angiosarcoma and similar tumors in zebrafish, which maintains the tumor microenvironment and has superior rates of engraftment.

PMID:34030492 | DOI:10.1089/zeb.2021.0006

Primary Angiosarcoma of the Adrenal Gland: Report of 2 Cases and Review of the Literature

Fetched: May 25th, 2021, 5:00am GMT by Alexander Ladenheim

Int J Surg Pathol. 2021 May 24:10668969211020099. doi: 10.1177/10668969211020099. Online ahead of print.

ABSTRACT

Primary adrenal angiosarcoma is a rare, malignant, vascular neoplasm. These neoplasms typically arise in middle age (median age of 60 years) and are more common in males (65%) than in females. Although rare, these neoplasms are aggressive with a propensity for local recurrence and metastasis and a median survival of 18 months. We present 2 cases of primary adrenal angiosarcoma with synchronous, ipsilateral adrenocortical adenomas. We review the cases of adrenal angiosarcoma reported since 1988 and discuss their clinical and histopathologic characteristics.

PMID:34029146 | DOI:10.1177/10668969211020099

Permalink for 'A Rare Cause of Pulmonary Nodules Diagnosed as Angiosarcoma Was Misdiagnosed as Vasculitis and Wegener's Granuloma in an Elderly Man: A Case Report'

A Rare Cause of Pulmonary Nodules Diagnosed as Angiosarcoma Was Misdiagnosed as Vasculitis and Wegener's Granuloma in an Elderly Man: A Case Report

Fetched: May 25th, 2021, 5:00am GMT by Peixia Wang

Front Oncol. 2021 May 5;11:629597. doi: 10.3389/fonc.2021.629597. eCollection 2021.

ABSTRACT

BACKGROUND: Angiosarcoma is a rare, highly malignant tumor prone to recurrence and metastasis. Angiosarcoma is insidious in the initial stage, and its clinical manifestation lacks specificity. The diagnosis is based on histopathology and immunohistochemistry findings.

CASE PRESENTATION: A 73-year-old man was hospitalized following complaints of persistent cough 6 months and hemoptysis for 2 months. Anti-infective treatment was ineffective. A CT-guided percutaneous core needle biopsy of pulmonary lesions revealed organized pneumonia, and the removed skin of purpuric rash area on the left calf revealed vasculitis. Chest CT was used during the patient follow-up. Hormonal therapy combined with immunoglobulins did not lead to improvement, and there was rapid progression of the lung lesions. Subsequently, the patient underwent a surgery, the diseased tissue was separated and removed completely beside the left submandibular gland under local anaesthesia. The immunohistochemical staining indicated CD31 (+) and CD34 (+) confirming a diagnosis of metastatic angiosarcoma. The expression of PD-L1 was 70%, therefore, anlotinib and pembrolizumab treatments were initiated. The patient eventually died.

CONCLUSION: Angiosarcoma is a malignant tumor in the clinic that lacks typical and specific signs and symptoms. The diagnosis depends on immunohistochemistry, which requires repeated biopsies of multiple sites in highly suspected cases.

PMID:34026609 | PMC:PMC8131857 | DOI:10.3389/fonc.2021.629597

KDM2B promotes cell viability by enhancing DNA damage response in canine hemangiosarcoma

Fetched: May 25th, 2021, 5:00am GMT by Kevin Christian Montecillo Gulay

J Genet Genomics. 2021 Mar 10:S1673-8527(21)00044-8. doi: 10.1016/j.jgg.2021.02.005. Online ahead of print.

ABSTRACT

Epigenetic regulators have been implicated in tumorigenesis of many types of cancer; however, their roles in endothelial cell cancers such as canine hemangiosarcoma (HSA) have not been studied. In this study, we find that lysine-specific demethylase 2b (KDM2B) is highly expressed in HSA cell lines compared with normal canine endothelial cells. Silencing of KDM2B in HSA cells results in increased cell death in vitro compared with the scramble control by inducing apoptosis through the inactivation of the DNA repair pathways and accumulation of DNA damage. Similarly, doxycycline-induced KDM2B silencing in tumor xenografts results in decreased tumor sizes compared with the control. Furthermore, KDM2B is also highly expressed in clinical cases of HSA. We hypothesize that pharmacological KDM2B inhibition can also induce HSA cell death and can be used as an alternative treatment for HSA. We treat HSA cells with GSK-J4, a histone demethylase inhibitor, and find that GSK-J4 treatment also induces apoptosis and cell death. In addition, GSK-J4 treatment decreases tumor size. Therefore, we demonstrate that KDM2B acts as an oncogene in HSA by enhancing the DNA damage response. Moreover, we show that histone demethylase inhibitor GSK-J4 can be used as a therapeutic alternative to doxorubicin for HSA treatment.

PMID:34023294 | DOI:10.1016/j.jgg.2021.02.005

Aberrant cytoplasmic connexin43 expression as a helpful marker in vascular neoplasms

Fetched: May 23rd, 2021, 5:00am GMT by Verena Gerlinde Frings

J Cutan Pathol. 2021 May 21. doi: 10.1111/cup.14066. Online ahead of print.

ABSTRACT

BACKGROUND: Gap junctions consisting of connexins (Cx) are fundamental in controlling cell proliferation, differentiation, and cell death. Cx43 is the most broadly expressed Cx in humans and is attributed an important role in skin tumor development. Its role in cutaneous vascular neoplasms is yet unknown.

METHODS: Fifteen cases each of cutaneous angiosarcoma (cAS), Kaposi sarcoma (KS), and cherry hemangioma (CH) were assessed by immunohistochemistry for expression of Cx43. Expression pattern, intensity, and percentage of positively stained cells were analyzed. Solid basal cell carcinomas served as positive and healthy skin as negative controls.

RESULTS: Most cases of cAS presented with a strong Cx43 staining of almost all tumor cells, whereas endothelia of KS showed medium expression and CH showed mostly weak expression. In comparison with KS or cAS, the staining intensity of CH was significantly lower (P ≤ 0.001). All tissue sections of both cAS and KS were characterized by a mostly diffuse, cytoplasmic staining pattern of the vascular endothelia. None of those showed nuclear staining.

CONCLUSION: The high-to-intermediate expression of Cx43 observed in all cases of cAS and KS suggests that this Cx may play a role in the development of malignant vascular neoplasms and serve as a helpful diagnostic marker.

PMID:34021619 | DOI:10.1111/cup.14066

Permalink for 'Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Epidemiology, Diagnosis, Staging, and Histology-Specific Management Considerations'

Breast Sarcomas, Phyllodes Tumors, and Desmoid Tumors: Epidemiology, Diagnosis, Staging, and Histology-Specific Management Considerations

Fetched: May 20th, 2021, 5:00am GMT by George Z Li

Am Soc Clin Oncol Educ Book. 2021 Mar;41:390-404. doi: 10.1200/EDBK_321341.

ABSTRACT

Breast sarcomas arise from connective tissues of the breast and account for fewer than 1% of all breast malignancies. They can be subclassified as primary breast sarcomas, which arise de novo and are histologically diverse, and secondary breast sarcomas, which arise as a result of radiation or lymphedema and are most commonly angiosarcomas. Two other connective tissue neoplasms that occur within the breast include phyllodes tumors and desmoid tumors, which exhibit a spectrum of behaviors. Malignant phyllodes tumors are biologically similar to primary breast sarcomas, whereas desmoid tumors are technically benign but often locally aggressive. Patients with breast sarcomas often present with a rapidly growing mass or, in cases of radiation-associated angiosarcoma, violaceous cutaneous lesions. Core needle biopsy is generally required to confirm the diagnosis of sarcomas. Staging workup includes MRI and chest imaging, although these are not required in the case of benign phyllodes or desmoid tumors. In general, localized breast sarcomas should be resected, with the extent of resection tailored to histologic subtype. Radiation and chemotherapy can be used in the neoadjuvant or adjuvant setting, but data are limited, so treatment decisions should be made on an individualized basis. Systemic therapy options for metastatic disease and refractory breast desmoids mimic those used for the same histologies when present in other sites. Given the rarity and heterogeneity of breast sarcoma, as well as limited literature describing these entities, expert multidisciplinary evaluation is crucial for optimal decision making.

PMID:34010054 | DOI:10.1200/EDBK_321341

Hemoptysis as the first sign of angiosarcoma: an extremely aggressive cardiac tumor

Fetched: May 19th, 2021, 5:00am GMT by Aleksandra Ciepłucha

Kardiol Pol. 2021 May 18. doi: 10.33963/KP.15920. Online ahead of print.

NO ABSTRACT

PMID:34002848 | DOI:10.33963/KP.15920

A Novel Entity Among Vascular Liver Tumors: The First Reported Liver Transplantation. Is It Feasible?

Fetched: May 19th, 2021, 5:00am GMT by Chiara Di Renzo

Transplant Proc. 2021 May 14:S0041-1345(21)00229-3. doi: 10.1016/j.transproceed.2021.02.027. Online ahead of print.

ABSTRACT

Liver transplant could be considered for certain tumors even if there is still dubious indication, as in the case of hepatic small vessels neoplasms that pose a difficult differential diagnosis with liver angiosarcoma. Liver transplant could be the best choice for patients with stable or slow-progressing tumors, for young patients with impaired quality of life, and when it would use organs that would be otherwise discarded but are capable of affording a good function. Vascular tumors are very heterogeneous cancers and our case represents the first description of a new histologic lesion that cannot be included in any of the pre-existing diagnostic categories. In our paper we want to present our decision to transplant a patient in whom the diagnosis was hepatic small vessels neoplasms (but was still in doubt for angiosarcoma as suggested by pathologists form other institutions). Furthermore, we want to highlight that after liver transplant, a new lesion never described before resulted from the specimen analysis that does not fit any of the pathologic diagnostic categories. This finding could open a scenario for additional improvement in molecular analysis in order to differentiate liver vascular tumors or even single lesion histotypes. This article emphasizes how little we know about the real behavior of liver vascular tumors and their clinical and therapeutic outcomes; we want to raise a question about the possibility for liver transplant in the setting of tumors not yet fully known and in which, in cases of diagnostic doubts, this option has been traditionally excluded, possibly bypassing ethical implications by using marginal donors.

PMID:34001349 | DOI:10.1016/j.transproceed.2021.02.027

Bone hemangiosarcoma in the calvarium of a dog

Fetched: May 18th, 2021, 5:00am GMT by C J Nye

J Small Anim Pract. 2021 May 17. doi: 10.1111/jsap.13355. Online ahead of print.

NO ABSTRACT

PMID:33999442 | DOI:10.1111/jsap.13355

Permalink for 'Multi-omics approach identifies germline regulatory variants associated with hematopoietic malignancies in retriever dog breeds'

Multi-omics approach identifies germline regulatory variants associated with hematopoietic malignancies in retriever dog breeds

Fetched: May 15th, 2021, 5:00am GMT by Jacquelyn M Evans

PLoS Genet. 2021 May 13;17(5):e1009543. doi: 10.1371/journal.pgen.1009543. eCollection 2021 May.

ABSTRACT

Histiocytic sarcoma is an aggressive hematopoietic malignancy of mature tissue histiocytes with a poorly understood etiology in humans. A histologically and clinically similar counterpart affects flat-coated retrievers (FCRs) at unusually high frequency, with 20% developing the lethal disease. The similar clinical presentation combined with the closed population structure of dogs, leading to high genetic homogeneity, makes dogs an excellent model for genetic studies of cancer susceptibility. To determine the genetic risk factors underlying histiocytic sarcoma in FCRs, we conducted multiple genome-wide association studies (GWASs), identifying two loci that confer significant risk on canine chromosomes (CFA) 5 (Pwald = 4.83x10-9) and 19 (Pwald = 2.25x10-7). We subsequently undertook a multi-omics approach that has been largely unexplored in the canine model to interrogate these regions, generating whole genome, transcriptome, and chromatin immunoprecipitation sequencing. These data highlight the PI3K pathway gene PIK3R6 on CFA5, and proximal candidate regulatory variants that are strongly associated with histiocytic sarcoma and predicted to impact transcription factor binding. The CFA5 association colocalizes with susceptibility loci for two hematopoietic malignancies, hemangiosarcoma and B-cell lymphoma, in the closely related golden retriever breed, revealing the risk contribution this single locus makes to multiple hematological cancers. By comparison, the CFA19 locus is unique to the FCR and harbors risk alleles associated with upregulation of TNFAIP6, which itself affects cell migration and metastasis. Together, these loci explain ~35% of disease risk, an exceptionally high value that demonstrates the advantages of domestic dogs for complex trait mapping and genetic studies of cancer susceptibility.

PMID:33983928 | PMC:PMC8118335 | DOI:10.1371/journal.pgen.1009543

Role of autopsy imaging-computed tomography in the post-mortem study of farm animals

Fetched: May 14th, 2021, 5:00am GMT by Kazutaka Yamada

Vet Rec Open. 2021 Apr 7;8(1):e1. doi: 10.1002/vro2.1. eCollection 2021 Dec.

ABSTRACT

BACKGROUND: Autopsy imaging (Ai) is used to determine the cause of death, providing pre-dissection information. Ai is often used in the field of human forensic medicine but has never been applied on farm animals.

METHODS: Ai-computed tomography (CT) was performed before necropsy for farm animals (one goat, one ox, one cow and three calves) that died or were euthanised.

RESULTS: Ai-CT findings of rib fractures (case 1), urethral calculi (case 2), multiple osteolytic bone lesions (case 3 and 4) and hair balls (case 4) were confirmed by dissection. However, a tentative diagnosis of actinomycosis was made in an ox (case 5) using antemortem radiography and Ai-CT, and the mass was identified as ameloblastic fibro-odontoma on histological examination. A tentative diagnosis of maxillary abscess was made from antemortem radiography in a cow (case 6); however, the lesion was shown to be maxillary neoplasia on Ai-CT. The mass was identified as hemangiosarcoma on histopathological examination.

CONCLUSION: Ai is helpful in pathological examination because the specific findings are known before the dissection, the lesions can be pinpointed in the pathological dissection, facilitating workflow; furthermore, the oversight of lesions can be reduced. In addition, Ai-CT images, including three-dimensional images and a three-dimensional printed model, allowed an easy understanding of pathology among students and farmers. Ai-CT for farm animals represents a novel option for veterinary education.

PMID:33981435 | PMC:PMC8109047 | DOI:10.1002/vro2.1

Primary breast angiosarcoma resembling a common benign tumor: A case report

Fetched: May 14th, 2021, 5:00am GMT by Muhammad Alshaar

Ann Med Surg (Lond). 2021 Apr 10;65:102281. doi: 10.1016/j.amsu.2021.102281. eCollection 2021 May.

ABSTRACT

INTRODUCTION AND IMPORTANCE: The breast angiosarcoma is a rare malignant vascular neoplasm; it represents nearly 0.05% of all breast cancers. Clinically, it could look like a haemangioma or pseudoangiomatous hyperplasia. Here, we report a case of a primary angiosarcoma of the breast (PAB) that mimics a giant fibroadenoma in physical examination and ultrasound features.

CASE PRESENTATION: A 22 year-old woman came to our hospital complaining of a mobile non-tender mass in her right breast. It was monitored for a while until it became an 8 × 7 cm². Past medical and family histories were unremarkable. Ultrasonography showed a well circumscribed hypoechoic lobular mass. The wide excision was performed and the pathological study showed angiosarcoma. The patient was re-operated for total mastectomy, screened for distant Metastases and followed-up closely for one year.

CLINICAL DISCUSSION: Fibroadenoma-like lesions are not uncommon and physicians should pay an attention for any breast mass even in the patient who is young and has no risk factors of the traditional breast cancer. PAB is used to be missed in the clinical practice over the world; it is extremely rare and is proved by Immunohistochemistry. The full management could be totally completed by a mastectomy with/out axillary node dissection.

CONCLUSION: The primary angisarcoma of the breast (PAB) could resemble a fibroadenoma in pre-operative investigations. In many reviews, PAB mimicked many diseases. Thus, the medical literature needs more case reports and series to identify an ideal protocol of diagnosis and management.

PMID:33981422 | PMC:PMC8082197 | DOI:10.1016/j.amsu.2021.102281

Breast Angiosarcoma with a Preoperative Diagnosis of Late Recurrence of Breast Cancer: A Case Report

Fetched: May 13th, 2021, 5:00am GMT by Rika Kouhashi

Case Rep Oncol. 2021 Mar 29;14(1):604-609. doi: 10.1159/000513906. eCollection 2021 Jan-Apr.

ABSTRACT

Angiosarcoma is a malignant mesenchymal tumor characterized by the presence of vascular endothelial cells. Although rare, angiosarcoma developing in the mammary glands has a poor prognosis. We report a case of breast angiosarcoma with a preoperative diagnosis of late recurrence of breast cancer. A 78-year-old woman noticed a tumor in her right breast and visited our hospital. The patient had undergone breast-conserving surgery and axillary lymph node dissection from the right breast 12 years before the visit. The tumor was diagnosed as T4bN0M0, stage IIIB. Anastrozole was administered as postoperative adjuvant therapy for 5 years; the patient also received 50-Gy whole-breast radiation therapy after surgery. Physical examination during her visit revealed an elevated lesion with blue purpura around the nipple in the right breast. We performed breast ultrasound and detected a well-defined 19.6 × 16.4 × 10.7 mm hypoechoic tumor in the left subareolar area. The patient underwent core needle biopsy (CNB). Based on the CNB specimen findings, she was suspected to experience late local recurrence after surgery. Therefore, she underwent total mastectomy after breast-conserving surgery. A dark-red tumor sized 18 × 12 mm was found in a specimen from the nipple. The pathological diagnosis of the specimen revealed short spindle-shaped tumor cells with strong nuclear pleomorphism and a significant interstitial fibrosis. Immunohistochemistry using D2-40 and CD31 antibodies showed irregular luminal proliferation at the anastomosis, infiltration into the surrounding tissue, and massive necrosis, thereby leading to the diagnosis of breast angiosarcoma. We have reported a case of breast angiosarcoma with a preoperative diagnosis of late recurrence of breast cancer.

PMID:33976641 | PMC:PMC8077598 | DOI:10.1159/000513906

Rapidly Progressive Periorbital Oedema: A Case of Cutaneous Angiosarcoma

Fetched: May 13th, 2021, 5:00am GMT by Kuan Yee Chow

Case Rep Oncol. 2021 Mar 22;14(1):531-537. doi: 10.1159/000514304. eCollection 2021 Jan-Apr.

ABSTRACT

Angiosarcoma is a rare form of malignant endothelial cell tumour characterised by rapidly infiltrating anaplastic cells of vascular or lymphatic origin. We report an uncommon case of cutaneous angiosarcoma (cAS) manifesting as rapidly progressive unilateral periorbital oedema. Due to the acute onset of disease, the patient was initially treated with antibiotics for presumed periorbital cellulitis. The lack of response to conservative management raised the suspicion of a more serious condition, which eventually revealed the diagnosis of angiosarcoma through skin biopsy. As suggested by several previous case reports, the subtle manifestation of cAS made it a great mimicker of benign skin conditions. This case report serves as a reminder to the aggressive nature of angiosarcoma which can lead to marked facial swelling within several weeks. As the tumour was not resectable by the time of diagnosis, the patient was offered palliative radiotherapy.

PMID:33976630 | PMC:PMC8077485 | DOI:10.1159/000514304

Deregulation of cell cycle and related microRNA expression induced by vinyl chloride monomer in hepatocytes of rats

Fetched: May 12th, 2021, 5:00am GMT by Weizhe Pan

Toxicol Ind Health. 2021 Jun;37(6):365-376. doi: 10.1177/07482337211015591. Epub 2021 May 11.

ABSTRACT

Vinyl chloride (VC) is a confirmed human carcinogen associated with hepatocellular carcinoma and angiosarcoma. However, the role of microRNAs (miRNAs) in liver cell cycle changes under VC exposure remains unclear, which prevents research on the mechanism of VC-induced carcinogenesis. In this study, male rats were injected intraperitoneally with VC (0, 5, 25, and 125 mg/kg body weight) for 6, 8, and 12 weeks. Cell cycle analysis of liver cells, miRNA-222, miRNA-199a, miRNA-195, and miRNA-125b expression in the liver and serum, and target protein expression were performed at different time points. The results showed a higher percentage of hepatocytes in the G1/G0 and S phases at the end of 6 and 12 weeks of VC exposure, respectively. MiRNA-222 expression decreased initially and then increased, whereas miRNA-199a, miRNA-195, and miRNA-125b expression increased initially and then decreased, which corresponded with changes in cell cycle distribution and related target proteins expression (p27, cyclinA, cyclinD1, and CDK6). The corresponding expression levels of miRNAs in serum did not change. Dynamic changes in miR-222, miR-199a, miR-195, and miR-125b induced by VC can lead to cell cycle deregulation by affecting cell cycle-related proteins, and these miRNAs can serve as early biomarkers for malignant transformation caused by VC.

PMID:33973497 | DOI:10.1177/07482337211015591

Epithelioid Angiosarcoma Causing Spinal Cord Compression

Fetched: May 11th, 2021, 5:00am GMT by Luis F Lemus

Cureus. 2021 Apr 6;13(4):e14325. doi: 10.7759/cureus.14325.

ABSTRACT

Epithelioid angiosarcoma is a rare and very aggressive malignant tumor with high rates of metastasis and recurrence that can present in any part of the body, with the head and neck being the most common regions. Wide-margin surgical resection is the treatment of choice following radiotherapy due to the high rate of recurrence. We present a case of an elderly patient who developed angiosarcoma causing spinal cord compression at the level of C7 vertebrae. We discuss the diagnosis, treatment, histopathology, and outcome.

PMID:33968534 | PMC:PMC8101505 | DOI:10.7759/cureus.14325

Permalink for 'Ultrasound-guided fine needle aspiration cytology of angiosarcoma of head and neck: a review of cytomorphologic features and discussion of diagnostic pitfall of aspiration cytology of vascular lesions'

Ultrasound-guided fine needle aspiration cytology of angiosarcoma of head and neck: a review of cytomorphologic features and discussion of diagnostic pitfall of aspiration cytology of vascular lesions

Fetched: May 9th, 2021, 5:00am GMT by Binny Khandakar

Diagn Cytopathol. 2021 Jul;49(7):902-906. doi: 10.1002/dc.24767. Epub 2021 May 8.

ABSTRACT

Angiosarcoma is a rare malignant tumor of vascular origin (<2% of sarcomas). It represents <1% of all head-neck malignancies. A cytological diagnosis on fine needle aspiration (FNA) is extremely difficult. Most tumors yield a predominantly hemorrhagic aspirate with only rare neoplastic cells, a diagnostic pitfall of aspiration cytology of vascular lesions. An 81-year-old male, 2 years status post resection of a nasal tip angiosarcoma and adjuvant radiation therapy, presented to another institution with an enlarging left neck mass of 2 months duration. Outside FNA diagnosis of cyst was made. Patient was referred to our institution for a pathologist-performed ultrasound-guided FNA. Ultrasound examination revealed a hypoechoic cystic left neck mass. Direct smears showed blood with occassional single malignant spindled to epithelioid cells, with some cells showing "spider-leg" like cytoplasmic processes/projections and "tadpole/fiber" like morphology. Immunohistochemistry performed on cell block sections revealed the tumor cells to be positive for D2-40, CD31 and CD34, supported a diagnosis of regional recurrent angiosarcoma. Left neck dissection showed recurrent high-grade angiosarcoma infiltrating periparotid soft tissues and metastatic angiosarcoma involving multiple lymph nodes. A diagnosis of angiosarcoma on FNA samples can be challenging, as the aspirate may contain only rare tumor cells hidden in a background of abundant blood. Such cases require careful screening to avoid an incorrect diagnosis of inadequate or cyst contents. This short review highlights key cytomorphologic features of angiosarcoma, includes novel cytologic features of angiosarcoma which has not been described previously and also discusses challenges and diagnostic pitfalls of FNA of vascular lesions.

PMID:33964187 | DOI:10.1002/dc.24767

Serum angiopoietin-2 levels in dogs with splenic haemangiosarcoma, haemangioma, and splenitis

Fetched: May 9th, 2021, 5:00am GMT by Supissara Wongsuttawas

Acta Vet Hung. 2021 May 5. doi: 10.1556/004.2021.00014. Online ahead of print.

ABSTRACT

Haemangioma (HA) and haemangiosarcoma (HSA) are among the most common splenic neoplasms in dogs. The survival time in splenic HSA is short, probably due to the lack of proper biological markers allowing early detection. We investigated the serum angiopoietin-2 (Ang-2) concentrations in 9 healthy dogs and 40 dogs with abnormal splenic masses. The Ang-2 concentration differences were further compared in healthy dogs, dogs with splenitis, splenic HA and HSA. The results showed that the Ang-2 level in healthy dogs was significantly lower than in the splenitis and splenic HA cases. Moreover, the Ang-2 level was significantly higher in splenic HA than in splenic HSA. Conversely, no significant differences in Ang-2 level were recorded between healthy and splenic HSA dogs, and between splenitis and splenic neoplasms (HA and HSA). No significant correlations were observed between the Ang-2 level and (i) the clinical stage, (ii) histological growth pattern, and (iii) median survival time of splenic HSA dogs. In conclusion, serum Ang-2 concentration is a potentially useful biological marker for the discrimination of dogs with splenitis and splenic HA, as well as for the differentiation of splenic HA from its malignant form, HSA.

PMID:33964126 | DOI:10.1556/004.2021.00014

Angiosarcoma of the Adrenal Gland

Fetched: May 8th, 2021, 5:00am GMT by Lori A Erickson

Mayo Clin Proc. 2021 May;96(5):1376-1378. doi: 10.1016/j.mayocp.2021.03.032.

NO ABSTRACT

PMID:33958072 | DOI:10.1016/j.mayocp.2021.03.032

Primary angiosarcoma of the spleen, a rare indication for splenectomy: a case report

Fetched: May 8th, 2021, 5:00am GMT by Matthew Wheelwright

Int J Surg Case Rep. 2021 May;82:105929. doi: 10.1016/j.ijscr.2021.105929. Epub 2021 Apr 29.

ABSTRACT

INTRODUCTION AND IMPORTANCE: Primary angiosarcoma of the spleen is a rare condition with a nonspecific clinical presentation and is associated with a poor prognosis. We describe two patients with primary splenic angiosarcoma successfully treated with splenectomy and adjuvant chemotherapy.

CASE PRESENTATIONS: Case 1: A 50-year-old female presented with fatigue and left-sided rib, shoulder, and abdominal pain. A CT scan demonstrated a large splenic mass, and biopsy was diagnostic of angiosarcoma. An open en bloc resection of the spleen was performed, and pathologic examination confirmed high-grade angiosarcoma; the surgical margins were negative. The patient received pegylated liposomal doxorubicin (PLD) and ifosfamide; she demonstrated no evidence of recurrence with four years of follow-up. Case 2: A 70-year-old male presented with acute back pain. A CT scan demonstrated a splenic mass; biopsy was diagnostic of angiosarcoma. The patient underwent open splenectomy, and pathology revealed high-grade angiosarcoma; the surgical margins were positive. The patient received PLD and ifosfamide but presented three years later with metastatic tumor to the spine. The patient had a favorable tumor response to pembrolizumab. The patient's tumor burden remains stable at 5 years following splenectomy.

CLINICAL DISCUSSION: Angiosarcoma of the spleen is a rare clinical entity and is often challenging to diagnose early. Moratality is high, especially in the case of metastasis or spontaneous rupture.

CONCLUSION: Due to the rare nature of this tumor, optimal treatment is not known. Here, we show excellent response in two patients to surgery combined with adjuvant therapy.

PMID:33957408 | PMC:PMC8113851 | DOI:10.1016/j.ijscr.2021.105929

A case of cutaneous epithelioid angiosarcoma with multiple metastases

Fetched: May 6th, 2021, 5:00am GMT by Minsu Kim

Int J Dermatol. 2021 May 5. doi: 10.1111/ijd.15570. Online ahead of print.

NO ABSTRACT

PMID:33951183 | DOI:10.1111/ijd.15570

Retrospective Analysis of the Clinical Presentation, Treatment and Outcome of Angiosarcoma in a Sarcoma Referral Center

Fetched: May 6th, 2021, 5:00am GMT by Patrick Schöffski

Oncol Res Treat. 2021;44(6):322-332. doi: 10.1159/000516000. Epub 2021 May 4.

ABSTRACT

INTRODUCTION: Angiosarcoma (AS) is a rare subtype of soft tissue sarcoma. We performed a retrospective analysis of patient characteristics, treatments and prognostic factors in patients treated in a single sarcoma center.

METHODS: We reviewed records of patients treated between 1987 and 2018, categorized in 7 different subtypes according to tissue of origin and underlying risk factors. The Kaplan-Meier method was used to estimate overall survival (OS); the Cox proportional hazards model was used to study prognostic variables.

RESULTS: Among 134 patients, 30% had radiation-induced, 31% primary soft tissue, 24% cutaneous, 5% breast, 4% bone, 2% lymphedema-associated and 4% unknown primary AS. Key patient/disease characteristics varied between subgroups. The median OS was 22.0 months for the entire cohort, with 28.9% with a 5-year survival. Metastasis at diagnosis was seen in 23% of patients; 38% developed metachronous metastasis. Sixty-six (49%) patients received systemic therapy; common first-line treatments were doxorubicin (48%) and paclitaxel (39%), without a significant difference in OS between agents. Younger age, breast/radiation-induced AS, primary surgery and palliative chemotherapy were associated with better OS. Synchronous metastasis, soft tissue/unknown primary location correlated with poor survival.

CONCLUSION: AS is a very heterogeneous sarcoma subtype, with substantial variability in clinical presentation and survival among patient subsets. Prognosis is poor, and there is no difference in outcome comparing the 2 most frequently used chemotherapy agents in the first line, paclitaxel and doxorubicin.

PMID:33946082 | DOI:10.1159/000516000

Cutaneous metastasis of renal pelvic urothelial carcinoma mimicking cutaneous angiosarcoma

Fetched: May 5th, 2021, 5:00am GMT by Ruriko Endo

Jpn J Clin Oncol. 2021 May 4:hyab067. doi: 10.1093/jjco/hyab067. Online ahead of print.

NO ABSTRACT

PMID:33942089 | DOI:10.1093/jjco/hyab067

Permalink for 'Unclassified mesenchymal sarcoma with NTRK1-KHDRBS1 gene fusion: a case report of long-term tumor-free survival with crizotinib treatment'

Unclassified mesenchymal sarcoma with NTRK1-KHDRBS1 gene fusion: a case report of long-term tumor-free survival with crizotinib treatment

Fetched: May 5th, 2021, 5:00am GMT by Weijie Chen

World J Surg Oncol. 2021 Apr 30;19(1):136. doi: 10.1186/s12957-021-02237-y.

ABSTRACT

BACKGROUND: Mesenchymal sarcomas are tumors that originate from mesenchymal tissue. Most mesenchymal sarcomas can be accurately classified, but some are unclassifiable in clinical practice. Molecular detection methods enable patients to benefit from molecular-targeted therapies for many cancers, including lung, breast, and bowel cancers. Further, even unclassified tumors can have therapeutic targets. NTRK gene fusions are sporadic genetic alterations that occur across tumor entities. If NTRK gene fusions are detected, TRK inhibitors can be used regardless of the tumor entity.

CASE PRESENTATION: This report describes a case with an unclassifiable mesenchymal sarcoma carrying a neurotrophic tyrosine receptor kinase NTRK1-KHDRBS1 gene fusion that was diagnosed and treated at multiple hospitals. Diagnostic work-up included pathological and immunohistochemical analysis, which excluded angiosarcoma, dendritic cell sarcoma, and pseudomyogenic hemangioendothelioma. The patient achieved a long-term survival without tumor relapse after treatment with crizotinib.

CONCLUSIONS: This case will be of significant interest to pathologists because, despite the tumor being unclassified, a molecular target was identified. Although the FDA does not currently approve crizotinib for treatment of patients harboring NTRK gene fusions, this case provides new insights for diagnosis and treatment of mesenchymal sarcomas with NTRK1 gene translocations. Similar to ALKomas, which can be successfully treated using NTRK molecular-targeted therapy, tumors with NTRK gene translocations can be classified as NTRKomas, even when they occur at different organ sites, and with varying histological morphologies, and immunophenotypes.

PMID:33941195 | PMC:PMC8091785 | DOI:10.1186/s12957-021-02237-y

A new-onset pulmonary artery stenosis in a young man: case report

Fetched: May 4th, 2021, 5:00am GMT by Francesco Vanni

Eur Heart J Case Rep. 2021 Apr 24;5(4):ytab118. doi: 10.1093/ehjcr/ytab118. eCollection 2021 Apr.

ABSTRACT

BACKGROUND : Poorly differentiated and undifferentiated sarcomas are the most common primary tumours of the pulmonary arteries. They usually affect large-calibre vessels and present with predominantly intraluminal growth. Dyspnoea, cough, chest pain, and haemoptysis are the most common presenting symptoms. Clinical and imaging manifestations can mimic pulmonary embolisms and correct diagnosis may require multimodal imaging. The overall prognosis is poor; however, early diagnosis and complete surgical resection seem to improve the prognosis.

CASE SUMMARY : A 31-year-old male was admitted to our department after a pre-syncopal episode associated with dyspnoea of recent onset. Echocardiography showed a mass with irregular borders attached to the pulmonary artery trunk, almost obliterating its lumen and determining a flow acceleration with a peak velocity and gradient, respectively, of 3.8 m/s and 60 mmHg. At cardiac magnetic resonance imaging and positron emission tomography-computed tomography scan, the mass had inhomogeneous contrast impregnation and an intense 18-fluorodeoxyglucose uptake, both findings are highly suggestive of an angiosarcoma of the pulmonary artery. Biopsy specimens were taken through bronchoscopy but the material was insufficient for diagnosis. The patient decided to continue treatment in another hospital, where he died a few months later.

DISCUSSION : The presence of a unique mass involving the main trunk of the pulmonary artery or proximal branches associated with rapidly progressive dyspnoea in a patient at low risk for pulmonary embolism should raise the suspicion of primary sarcoma of the pulmonary artery. There are no guidelines for the treatment. Surgery and neo/adjuvant chemotherapy are reported in literature but burdened by bias and concerning a small number of cases.

PMID:33937621 | PMC:PMC8068665 | DOI:10.1093/ehjcr/ytab118

Cancer-associated POT1 mutations lead to telomere elongation without induction of a DNA damage response

Fetched: May 4th, 2021, 5:00am GMT by Won-Tae Kim

EMBO J. 2021 Jun 15;40(12):e107346. doi: 10.15252/embj.2020107346. Epub 2021 May 2.

ABSTRACT

Mutations in the shelterin protein POT1 are associated with chronic lymphocytic leukemia (CLL), Hodgkin lymphoma, angiosarcoma, melanoma, and other cancers. These cancer-associated POT1 (caPOT1) mutations are generally heterozygous, missense, or nonsense mutations occurring throughout the POT1 reading frame. Cancers with caPOT1 mutations have elongated telomeres and show increased genomic instability, but which of the two phenotypes promotes tumorigenesis is unclear. We tested the effects of CAS9-engineered caPOT1 mutations in human embryonic and hematopoietic stem cells (hESCs and HSCs, respectively). HSCs with caPOT1 mutations did not show overt telomere damage. In vitro and in vivo competition experiments showed the caPOT1 mutations did not confer a selective disadvantage. Since DNA damage signaling is known to affect the fitness of HSCs, the data argue that caPOT1 mutations do not cause significant telomere damage. Furthermore, hESC lines with caPOT1 mutations showed no detectable telomere damage response while showing consistent telomere elongation. Thus, caPOT1 mutations are likely selected for during cancer progression because of their ability to elongate telomeres and extend the proliferative capacity of the incipient cancer cells.

PMID:33934394 | DOI:10.15252/embj.2020107346

Competing risk analysis of cutaneous angiosarcoma: implications for surveillance

Fetched: May 4th, 2021, 5:00am GMT by Kevin A Nguyen

Int J Dermatol. 2021 May 2. doi: 10.1111/ijd.15637. Online ahead of print.

NO ABSTRACT

PMID:33934332 | DOI:10.1111/ijd.15637

Combined VEGFR and MAPK pathway inhibition in angiosarcoma

Fetched: May 2nd, 2021, 5:00am GMT by Michael J Wagner

Sci Rep. 2021 Apr 30;11(1):9362. doi: 10.1038/s41598-021-88703-9.

ABSTRACT

Angiosarcoma is an aggressive malignancy of endothelial cells that carries a high mortality rate. Cytotoxic chemotherapy can elicit clinical responses, but the duration of response is limited. Sequencing reveals multiple mutations in angiogenesis pathways in angiosarcomas, particularly in vascular endothelial growth factor (VEGFR) and mitogen-activated protein kinase (MAPK) signaling. We aimed to determine the biological relevance of these pathways in angiosarcoma. Tissue microarray consisting of clinical formalin-fixed paraffin embedded tissue archival samples were stained for phospho- extracellular signal-regulated kinase (p-ERK) with immunohistochemistry. Angiosarcoma cell lines were treated with the mitogen-activated protein kinase kinase (MEK) inhibitor trametinib, pan-VEGFR inhibitor cediranib, or combined trametinib and cediranib and viability was assessed. Reverse phase protein array (RPPA) was performed to assess multiple oncogenic protein pathways. SVR angiosarcoma cells were grown in vivo and gene expression effects of treatment were assessed with whole exome RNA sequencing. MAPK signaling was found active in over half of clinical angiosarcoma samples. Inhibition of MAPK signaling with the MEK inhibitor trametinib decreased the viability of angiosarcoma cells. Combined inhibition of the VEGF and MAPK pathways with cediranib and trametinib had an additive effect in in vitro models, and a combinatorial effect in an in vivo model. Combined treatment led to smaller tumors than treatment with either agent alone. RNA-seq demonstrated distinct expression signatures between the trametinib treated tumors and those treated with both trametinib and cediranib. These results indicate a clinical study of combined VEGFR and MEK inhibition in angiosarcoma is warranted.

PMID:33931674 | PMC:PMC8087824 | DOI:10.1038/s41598-021-88703-9

Next-generation sequencing for the management of sarcomas with no known driver mutations

Fetched: May 1st, 2021, 5:00am GMT by Simon Vyse

Curr Opin Oncol. 2021 Jul 1;33(4):315-322. doi: 10.1097/CCO.0000000000000741.

ABSTRACT

PURPOSE OF REVIEW: Next-generation sequencing (NGS) has enabled fast, high-throughput nucleotide sequencing and has begun to be implemented into clinical practice for genomic-guided precision medicine in various cancer types. This review will discuss recent evidence that highlights opportunities for NGS to improve outcomes in sarcomas that have complex genomic profiles with no known driver mutations.

RECENT FINDINGS: Global genomic signatures detectable by NGS including tumour mutational burden and microsatellite instability have potential as biomarkers for response to immunotherapy in certain sarcoma subtypes including angiosarcomas. Identification of hallmarks associated with 'BRCAness' and homologous recombination repair defects in leiomyosarcomas and osteosarcomas may predict sensitivity to poly(adenosine diphosphate-ribose) polymerase (PARP) inhibitors. Lastly, the use of NGS for evaluating cancer predisposition in sarcomas may be useful for early detection, screening and surveillance.

SUMMARY: Currently, the implementation of NGS for every sarcoma patient is not practical or useful. However, adopting NGS as a complementary approach in sarcomas with complex genomics and those with limited treatment options has the potential to deliver precision medicine to a subgroup of patients, with novel therapies such as immune checkpoint and PARP inhibitors. Moving forward, molecular tumour boards incorporating multidisciplinary teams of pathologists, oncologists and genomic specialists to interpret NGS data will complement existing tools in diagnosis and treatment decision making in sarcoma patients.

PMID:33927108 | DOI:10.1097/CCO.0000000000000741

Permalink for 'Correction: da Costa et al. Establishment of a Temperature-Sensitive Model of Oncogene-Induced Senescence in Angiosarcoma Cells. Cancers 2020, 12, 395'

Correction: da Costa et al. Establishment of a Temperature-Sensitive Model of Oncogene-Induced Senescence in Angiosarcoma Cells. Cancers 2020, 12, 395

Fetched: May 1st, 2021, 5:00am GMT by Adilson da Costa

Cancers (Basel). 2021 Apr 22;13(9):2015. doi: 10.3390/cancers13092015.

ABSTRACT

The authors wish to make the following corrections to this paper [...].

PMID:33922400 | PMC:PMC8122306 | DOI:10.3390/cancers13092015

Angiosarcoma of the Urinary Bladder Following Radiotherapy: Report of a Case and Review of the Literature

Fetched: May 1st, 2021, 5:00am GMT by Gianmartin Cito

Medicina (Kaunas). 2021 Apr 1;57(4):329. doi: 10.3390/medicina57040329.

ABSTRACT

Background and objectives: Angiosarcomas are uncommon and extremely aggressive malignancies derived from vascular endothelial cells. Although they can occur anywhere in the body and at any age, they are more frequently found in the skin of the head and neck regions and in the elderly. Few cases have been recorded in deep soft tissues and in parenchymal organs. Angiosarcomas of the urinary bladder are exceedingly rare. They usually arise in adult patients with a history of radiation therapy, cigarette smoking, or exposure to chemical agents (e.g., vinyl chloride). Despite multidisciplinary treatment approaches combining surgery, radiotherapy, and chemotherapy, prognosis is dismal. Materials and Methods: We describe a case of a 78-year-old Caucasian man presenting with a vesical mass incidentally discovered with abdominal computerized tomography (CT). He underwent transurethral resection of the bladder (TURB), and histology was compatible with angiosarcoma. Results: The patient had been a heavy smoker and his medical history included therapeutic irradiation for prostate cancer eight years previously. Radical cystoprostatectomy was feasible, and pathologic examination of the surgical specimen confirmed angiosarcoma involving the urinary bladder, prostate, and seminal vesicles. Post-operative peritonitis resulted in progressive multi-organ failure and death. Conclusions: Angiosarcoma primary to the urinary bladder is seldom encountered, however, it should be considered in the differential diagnosis of vesical tumors, especially in elderly men with a history of pelvic radiotherapy.

PMID:33915778 | PMC:PMC8066026 | DOI:10.3390/medicina57040329

Status of microsatellite stability in angiosarcoma: Angiosarcoma is a microsatellite stable tumor

Fetched: April 30th, 2021, 5:00am GMT by Saki Maeda-Otsuka

J Dermatol. 2021 Apr 29. doi: 10.1111/1346-8138.15938. Online ahead of print.

NO ABSTRACT

PMID:33914950 | DOI:10.1111/1346-8138.15938

Far From Luck's Way: A Concurrence of Kaposi Sarcoma and Cutaneous Angiosarcoma in the Setting of Chronic Lymphocytic Leukemia

Fetched: April 30th, 2021, 5:00am GMT by Billur Sezgin

J Cutan Aesthet Surg. 2020 Oct-Dec;13(4):344-348. doi: 10.4103/JCAS.JCAS_127_19.

ABSTRACT

Kaposi sarcoma and cutaneous angiosarcoma are rare forms of skin malignancies that are vascular in nature and are frequently encountered in the immunosuppressed population. Although synchronous angiosarcoma and Kaposi sarcoma have been documented, to our knowledge, the coexistence of these vascular malignancies with underlying chronic lymphocytic leukemia has not been previously reported. A 51-year-old male patient with chronic lymphocytic leukemia presented with Kaposi sarcoma located on his left ankle. Shortly after, the patient presented with de novo lesions located on the plantar region of the right foot, reported as angiosarcoma. Following a multidisciplinary decision, treatment with adjuvant chemotherapy consisting of paclitaxel and carboplatin with consolidation radiotherapy was planned. The patient's Kaposi sarcoma has remained unchanged throughout this period. In such cases, the treatment is advised to be planned around the more aggressive malignancy. These patients should also be followed-up by dermatology due to the higher risk of secondary cutaneous malignancies.

PMID:33911419 | PMC:PMC8061659 | DOI:10.4103/JCAS.JCAS_127_19

Permalink for 'Combination of targeted therapy and immune checkpoint blocker in a patient with xeroderma pigmentosum presenting an aggressive angiosarcoma and a recurrent non-resectable basal cell carcinoma'

Combination of targeted therapy and immune checkpoint blocker in a patient with xeroderma pigmentosum presenting an aggressive angiosarcoma and a recurrent non-resectable basal cell carcinoma

Fetched: April 28th, 2021, 5:00am GMT by Celine Boutros

Eur J Cancer. 2021 Jun;150:130-132. doi: 10.1016/j.ejca.2021.03.022. Epub 2021 Apr 23.

NO ABSTRACT

PMID:33901791 | DOI:10.1016/j.ejca.2021.03.022

Radiologic-Pathologic Correlation of Liver Tumors

Fetched: April 28th, 2021, 5:00am GMT by Eric Lachance

In: Sergi CM, editor. Liver Cancer [Internet]. Brisbane (AU): Exon Publications; 2021 Apr 6. Chapter 5.

ABSTRACT

Radiologic and pathologic features of common and/or critical tumor or tumor-like diagnoses (lesions) of the liver are discussed within. Hepatocellular lesions (focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma, and hepatoblastoma), biliary lesions (mucinous cystic neoplasm and intrahepatic cholangiocarcinoma), vascular mesenchymal lesions (cavernous hemangioma, epithelioid hemangioendothelioma, and hepatic angiosarcoma), and metastatic malignancies are the primary focus, although a more comprehensive list of lesions is also provided. Definitions, distributions, gross appearances and microscopic pathological features are introduced first, followed by radiologic correlation. Multiple imaging modalities are explored with an emphasis on those that provide the greatest value for the lesion under evaluation. A common understanding of the features of both diagnostic specialties will allow for high-quality correlation and subsequent high-quality patient care. Representative images highlighting important features are also presented.

PMID:33905200 | Bookshelf:NBK569803 | DOI:10.36255/exonpublications.livercancer.2021.ch5

Multiple Firm Livid Nodules on the Proximal Lower Leg: A Quiz

Fetched: April 27th, 2021, 5:00am GMT by Kai-Philipp Linse

Acta Derm Venereol. 2021 Apr 26. doi: 10.2340/00015555-3809. Online ahead of print.

NO ABSTRACT

PMID:33899111 | DOI:10.2340/00015555-3809

Clinical outcomes following high-dose-rate surface applicator brachytherapy for angiosarcoma of scalp and face

Fetched: April 27th, 2021, 5:00am GMT by Devarati Mitra

J Contemp Brachytherapy. 2021 Apr;13(2):172-178. doi: 10.5114/jcb.2021.105285. Epub 2021 Apr 14.

ABSTRACT

PURPOSE: Angiosarcoma is a sub-type of soft tissue sarcoma, often presenting as a multifocal or diffuse disease process with poor prognosis. This study presents outcomes of a single institution cohort of patients with angiosarcoma of the scalp and face following treatment with multimodality therapy, including high-dose-rate surface applicator (HDR-SA) brachytherapy, and represents the largest cohort utilizing this therapeutic approach.

MATERIAL AND METHODS: Twenty patients with primary or recurrent angiosarcoma of the face or scalp were treated with HDR-SA brachytherapy between 2003-2018, with clinical characteristics and outcomes collected from medical records and used to identify prognostic features.

RESULTS: Median follow-up was 45 months. Patients treated with HDR-SA brachytherapy had a 4-year local control rate of 63%, a 4-year progression-free survival (PFS) rate of 20%, and a 4-year overall survival rate of 54%. Disease features associated with worse loco-regional control (LRC) included location on the scalp (vs. face, p = 0.04) and tumor size ≥ 5 cm (p = 0.0099). Outcomes after HDR-SA brachytherapy for salvage therapy vs. HDR-SA brachytherapy as a component of an initial treatment approach were also significantly different, with worse LRC (p = 0.0084) and worse overall survival (OS) (p = 0.0019) in a setting of salvage therapy.

CONCLUSIONS: Local control rates following HDR-SA brachytherapy for scalp or face angiosarcoma are moderate and similar to what is described in the literature using a variety of local control treatment modalities. Smaller tumors and those involving the face rather than scalp had better outcomes. PFS rates were poor and there is a pressing need for treatment intensification and novel therapeutic options.

PMID:33897791 | PMC:PMC8060956 | DOI:10.5114/jcb.2021.105285

Intravascular pseudolymphomatous angiosarcoma: a new finding potentially mistaken for intravascular lymphoma

Fetched: April 26th, 2021, 5:00am GMT by Amrita Goyal

J Cutan Pathol. 2021 Apr 25. doi: 10.1111/cup.14032. Online ahead of print.

ABSTRACT

Pseudolymphomatous infiltrates associated with angiosarcoma are a rarely-reported phenomenon. Recognition of this reactive process is critical to making an accurate diagnosis, both in diagnosing the angiosarcoma and in avoiding an incorrect diagnosis of lymphoma. Here we present a novel histopathologic pattern, angiosarcoma with a prominently intravascular atypical lymphoid component, mimicking intravascular T-cell lymphoma. Interestingly, serial biopsies in this case revealed a progressive increase in lymphocyte density and intravascular component over time. Despite prior reports of improved progression-free survival and overall survival of patients with pseudolymphomatous angiosarcoma, this patient demonstrated rapid disease progression. This article is protected by copyright. All rights reserved.

PMID:33896019 | DOI:10.1111/cup.14032

Permalink for 'Computed tomography-guided biopsy of radiologically unclear lesions in advanced skin cancer: A retrospective analysis of 47 cases'

Computed tomography-guided biopsy of radiologically unclear lesions in advanced skin cancer: A retrospective analysis of 47 cases

Fetched: April 26th, 2021, 5:00am GMT by Cindy Franklin

Eur J Cancer. 2021 Jun;150:119-129. doi: 10.1016/j.ejca.2021.03.026. Epub 2021 Apr 23.

ABSTRACT

BACKGROUND: Radiological imaging such as computed tomography (CT) is used frequently for disease staging and therapy monitoring in advanced skin cancer patients. Detected lesions of unclear dignity are a common challenge for treating physicians. The aim of this study was to assess the frequency and outcome of CT-guided biopsy (CTGB) of radiologically unclear, suspicious lesions and to depict its usefulness in different clinical settings.

METHODS: This retrospective monocentric study included advanced skin cancer patients (melanoma, Merkel cell carcinoma, squamous cell carcinoma, angiosarcoma, cutaneous lymphoma) with radiologically unclear lesions who underwent CTGB between 2010 and 2018.

RESULTS: Of 59 skin cancer patients who received CTGB, 47 received CTGB to clarify radiologically suspicious lesions of unclear dignity. 32 patients had no systemic therapy (cohort A), while 15 patients received systemic treatment at CTGB (cohort B). In both cohorts, CTGB revealed skin cancer metastasis in a large proportion of patients (37.5%, 40.0%, respectively), but benign tissue showing inflammation, fibrosis or infection in an equally large percentage (37.5%, 46.7%, respectively). Additionally, a significant number of other cancer entities was found (25.0%, 13.3%, respectively). In patients receiving BRAF/MEK inhibitors, CTGB confirmed suspicious lesions as skin cancer metastasis in 83.3%, leading to treatment change. In immune checkpoint inhibitor-treated patients, skin cancer metastasis was confirmed in 11.1% of patients only, whereas benign tissue changes (inflammation/fibrosis) were found in 77.8%.

CONCLUSIONS: Our results highlight the relevance of clarifying radiologically unclear lesions by CTGB before start or change of an anti-tumour therapy to exclude benign alterations and secondary malignancies.

PMID:33895683 | DOI:10.1016/j.ejca.2021.03.026

Second Malignancies after Radiation Therapy: Update on Pathogenesis and Cross-sectional Imaging Findings

Fetched: April 24th, 2021, 5:00am GMT by Lokesh Khanna

Radiographics. 2021 May-Jun;41(3):876-894. doi: 10.1148/rg.2021200171. Epub 2021 Apr 23.

ABSTRACT

A wide spectrum of second cancers occur as late complications of radiation therapy (RT) used to treat various malignancies. In addition to the type and dose of radiation, lifestyle, environmental, and genetic factors are important to the development of second malignancies in cancer survivors. Typically, RT-induced malignancies (RTIMs) are biologically aggressive cancers with a variable period of 5-10 years for hematologic malignancies and 10-60 years for solid tumors between RT and the development of the second cancer. Although carcinomas and leukemias commonly develop after low-dose RT, sarcomas occur in tissues or organs that receive high-dose RT. Angiosarcomas and unclassified pleomorphic sarcomas are the two most common RT-associated sarcomas; other sarcomas include malignant peripheral nerve sheath tumors, leiomyosarcomas, osteosarcomas, chondrosarcomas, and dedifferentiated or pleomorphic liposarcomas. Select RTIMs show tumor genetic characteristics that allow accurate diagnosis. Nearly all cutaneous angiosarcomas after RT for breast cancer and 90% of RT-associated malignant peripheral nerve sheath tumors are characterized by MYC gene amplifications and loss of H3 K27me3 expression, respectively. Classic papillary thyroid carcinomas that develop after RT frequently harbor RET/PTC rearrangements and have a favorable prognosis, despite their advanced stage at patient presentation. Select RTIMs demonstrate characteristic imaging findings and typically develop in the prior radiation field. Imaging is essential to early diagnosis, characterization, localization, and staging of RTIMs. Familiarity of radiologists with the diverse spectrum of RTIMs is essential for early diagnosis and optimal management. An invited commentary by Shapiro is available online. ^©RSNA, 2021.

PMID:33891523 | DOI:10.1148/rg.2021200171

Primary malignant vascular tumors of the liver in children: Angiosarcoma and epithelioid hemangioendothelioma

Fetched: April 24th, 2021, 5:00am GMT by Sami Bannoura

World J Gastrointest Oncol. 2021 Apr 15;13(4):223-230. doi: 10.4251/wjgo.v13.i4.223.

ABSTRACT

Primary malignant vascular neoplasms of the liver, angiosarcoma and epithelioid hemangioendothelioma, are extremely rare entities in the pediatric population. International Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age group. In this article, we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric population. Hepatic angiosarcoma in children shows a slight female predominance with an average age of 40 mo at diagnosis. The distinct histologic features include whorls of atypical spindled cells and eosinophilic globules, in addition to the general findings of angiosarcoma. Histologic diagnosis of pediatric hepatic angiosarcoma is not always straightforward, and the diagnostic challenges are discussed in the article. Hepatic epithelioid hemangioendothelioma also demonstrates a female predominance, but is more commonly identified in adolescents (median age at diagnosis: 12 years). Histologically, the lesion is characterized by epithelioid cells and occasional intracytoplasmic lumina with a background of fibromyxoid stroma. While WWTR1-CAMTA1 and YAP1-TFE3 fusions have been associated with epithelioid hemangioendothelioma, there are currently no known signature genetic alterations seen in pediatric hepatic angiosarcoma. Advancement in molecular pathology, particularly for pediatric hepatic angiosarcoma, is necessary for a better understanding of the disease biology, diagnosis, and development of targeted therapies.

PMID:33889274 | PMC:PMC8040065 | DOI:10.4251/wjgo.v13.i4.223

Permalink for 'DNA methylation profiling discriminates between malignant pleural mesothelioma and neoplastic or reactive histological mimics'

DNA methylation profiling discriminates between malignant pleural mesothelioma and neoplastic or reactive histological mimics

Fetched: April 24th, 2021, 5:00am GMT by Luca Bertero

J Mol Diagn. 2021 Apr 20:S1525-1578(21)00090-8. doi: 10.1016/j.jmoldx.2021.04.002. Online ahead of print.

ABSTRACT

The diagnosis of malignant pleural mesothelioma (MPM) is challenging because of its potential overlap with other neoplasms or even with reactive conditions. Recently, DNA methylation analysis was reported as effective in the diagnosis of tumors. In the present study, this approach was tested for use in the diagnosis of MPM. The DNA methylation patterns of a discovery cohort and an independent-validation cohort of MPMs were compared to those of 202 cases representing malignant and benign diagnostic mimics (angiosarcoma, desmoid-type fibromatosis, epithelioid sarcoma, leiomyosarcoma, lung adenocarcinoma, lung squamous cell carcinoma, melanoma, nodular fasciitis, reactive mesothelial hyperplasia, sclerosing fibrous pleuritis, solitary fibrous tumor, and synovial sarcoma). By both unsupervised hierarchical clustering and t-distributed stochastic neighbor embedding analysis, MPM samples in the discovery cohort exhibited a DNA methylation profile different from those of other neoplastic and reactive mimics. These results were confirmed in the independent-validation cohort and by in silico analysis of the MPM-The Cancer Genome Atlas data set. Copy number-variation profiles were also inferred to identify molecular hallmarks of MPM, including CDKN2A and NF2 deletions. Methylation profiling was effective in the diagnosis of MPM, although caution is advised in samples with low tumor cell content.

PMID:33887463 | DOI:10.1016/j.jmoldx.2021.04.002

Permalink for 'Radiotherapy-associated angiosarcoma in the breast reconstructed by autologous free-flap and treated with electrochemotherapy'

Radiotherapy-associated angiosarcoma in the breast reconstructed by autologous free-flap and treated with electrochemotherapy

Fetched: April 23rd, 2021, 5:00am GMT by Romi Cencelj-Arnez

Radiol Oncol. 2020 Dec 29;55(1):77-81. doi: 10.2478/raon-2020-0074.

ABSTRACT

BACKGROUND: Radiotherapy-associated angiosarcoma (RAA) of the breast is a rare complication of radiotherapy, which is often difficult to identify and has poor prognosis. It usually presents as violaceous skin, erythema or rapidly growing palpable firm mass that can be confused with other benign skin lesions.

PATIENTS AND METHODS: After reviewing the literature, we found only four cases with RAA after mastectomy and autologous breast reconstruction. The presented case is the first that was treated by electrochemotherapy. The patient presented with secondary angiosarcoma of the breast five years after mastectomy, immediate breast reconstruction with deep inferior epigastric artery perforator free flap and adjuvant radiotherapy.

RESULTS: Electrochemotherapy was feasible, safe and effective in treatment of radiation induced sarcoma. Most of the treated lesions in several consecutive electrochemotherapy sessions responded with complete response, but multiple recurrences occurred in non-treated areas.

CONCLUSIONS: Patients with breast cancer after skin-sparing mastectomy and immediate breast reconstruction, who receive radiotherapy, need regular long-term follow up and low threshold for biopsy of any suspicious lesions is mandatory. Electrochemotherapy proved as one of feasible modalities of treatment for RAA.

PMID:33885245 | PMC:PMC7877258 | DOI:10.2478/raon-2020-0074

Prognostic value of sternal lymphadenopathy on malignancy and survival in dogs undergoing splenectomy

Fetched: April 21st, 2021, 5:01am GMT by Jennifer Kelsey

Vet Comp Oncol. 2021 Apr 20. doi: 10.1111/vco.12700. Online ahead of print.

ABSTRACT

The purpose of this retrospective cohort study was to determine prevalence of sternal lymphadenopathy in a population of dogs undergoing splenectomy, and to evaluate if the prevalence was associated with splenic malignancy. In addition, the study investigated if survival in dogs diagnosed with hemangiosarcoma (HSA) differed between those with or without sternal lymphadenopathy at the time of surgery. Digital radiographs and medical records for dogs who underwent splenectomy from 2013 to 2016 where retrospectively reviewed. One hundred ninety-five dogs underwent splenectomy during the study period. The overall prevalence of sternal lymphadenopathy was 12.8%. The prevalence of sternal lymphadenopathy in dogs with hemangiosarcoma was 16.2% (12/74), other malignancy was 15.8% (3/19), and with a benign process 9.8% (10/102). There was no significant association between sternal lymphadenopathy with hemoperitoneum (p = .20) or between sternal lymphadenopathy and presence of neoplasia (p = .37). There was no significant difference in survival probability in all dogs with or without sternal lymphadenopathy (p = .073). However, sternal lymphadenopathy was associated with lower survival in 74 dogs with HSA (p = .036) and 19 with other splenic malignancies (p = .039). The presence of sternal lymphadenopathy should not be considered a negative prognostic indicator if present in dogs presenting with a hemoperitoneum. Although the presence of sternal lymphadenopathy at the time of initial presentation was not significantly associated with survival time in all dogs with splenic disease, it may have predictive value related to survival of dogs with splenic malignancy.

PMID:33876555 | DOI:10.1111/vco.12700

Clinical and imaging characteristics and prognosis of patients with primary cardiac angiosarcoma

Fetched: April 21st, 2021, 5:01am GMT by Y Y Zhu

Zhonghua Xin Xue Guan Bing Za Zhi. 2021 Apr 24;49(4):374-379. doi: 10.3760/cma.j.cn112148-20200427-00351. Online ahead of print.

ABSTRACT

Objective: To investigate the clinical, cardiac imaging characteristics and prognosis of patients with primary cardiac angiosarcoma. Methods: The clinical data of 14 patients hospitalized with primary cardiac angiosarcoma from January 2001 to December 2017 in Peking Union Medical College Hospital were collected and analyzed. Metastatic cardiac angiosarcoma were not included in this study. Patients were followed up post discharge per telephone call or clinical visit. Results: Of the 14 patients, 8 were males and 6 were females, average age was 48 years. The main clinical symptoms were shortness of breath (8/14), hemoptysis (6/14), fever (5/14), chest pain (4/14) and cough (3/14). Imaging examinations showed that the tumors of 8 patients were located in the right heart and 6 in the pericardial cavity. Tumors in the right heart often infiltrate the atrial wall and cause pericardial effusion (7/8). Tumors in the pericardium were characterized by recurrent bloody pericardial effusion (6/6), prone to progressive constrictive pericarditis (3/6), pericardial fluid cytology was often negative (6/6). MRI showed heterogeneous high signal intensity (cauliflower aspect) on T2-weighted image and heterogeneous enhancement with a"sunray"aspect at the perfusion study. At the time of diagnosis, 8 patients developed lung or adrenal metastasis (8/14). The median survival was only 305 days. Conclusion: Primary cardiac angiosarcoma is a rare disease with non-specific clinical manifestation and poor prognosis. Imaging examinations may help diagnosis. The high invasiveness and the easy-to-metastasis feature of the tumor contribute to the poor prognosis of cardiac angiosarcoma.

PMID:33874688 | DOI:10.3760/cma.j.cn112148-20200427-00351

Primary angiosarcoma of superior vena cava: an unexpected diagnosis after an oncological emergency

Fetched: April 18th, 2021, 5:00am GMT by Fábio Rêgo Salgueiro

BMJ Case Rep. 2021 Apr 16;14(4):e241266. doi: 10.1136/bcr-2020-241266.

ABSTRACT

Angiosarcoma (AS) is a rare malignant tumour representing 1%-2% of all sarcomas. Primary AS of superior vena cava (SVC) was reported in two cases worldwide. We report a 69-year-old woman with neck discomfort, headache and dyspnoea for 3 months. CT angiography showed thrombosis in SVC and brachiocephalic veins resulting in an SVC syndrome. The patient began anticoagulant therapy and underwent balloon angioplasty with clinical improvement. Additionally, a positron emission tomography scan confirmed the presence of a mediastinal mass involving the SVC locally. The tumour was excised and a prosthesis was placed on the SVC. Histology revealed a heterogeneous tumour matrix, either myxoid and composed by fusiform cells with vimentin, homogeneous CD31 and a 30% Ki67 immunoexpression, supporting the diagnosis of an AS. Due to multiple complications, the patient never started chemotherapy, and after tumour recurrence, she died within 5 months after diagnosis.

PMID:33863773 | PMC:PMC8055115 | DOI:10.1136/bcr-2020-241266

Angiosarcoma presenting as a diffuse facial edematous induration: a rare and atypical clinical feature

Fetched: April 17th, 2021, 5:01am GMT by Shogo Wada

J Dtsch Dermatol Ges. 2021 Apr 16. doi: 10.1111/ddg.14352. Online ahead of print.

NO ABSTRACT

PMID:33861890 | DOI:10.1111/ddg.14352

Tumor Biology Impacts Survival in Surgically Managed Primary Hepatic Vascular Malignancies

Fetched: April 16th, 2021, 5:00am GMT by Epameinondas Dogeas

J Surg Res. 2021 Apr 12;264:481-489. doi: 10.1016/j.jss.2021.02.043. Online ahead of print.

ABSTRACT

BACKGROUND: Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database.

MATERIALS AND METHODS: In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Clinicopathologic factors were extracted and compared. Overall survival (OS) was estimated and predictors of survival were identified.

RESULTS: Three hundred ninty patients with AS and 216 with HEHE were identified. Only 16% of AS and 36% of HEHE patients underwent surgery. The median OS for patients who underwent surgical intervention was 97 months, with 5-year OS of 30% for AS versus 69% for HEHE patients (P< 0.001). Tumor biology strongly impacted OS, with AS histology (Hazard Ratio [HR] of 3.61 [1.55-8.42]), moderate/poor tumor differentiation (HR = 3.86 [1.03-14.46]) and tumor size (HR = 1.01 [1.00-1.01]) conferring worse prognosis. The presence of metastatic disease in the surgically managed cohort (HR = 5.22 [2.01-13.57]) and involved surgical margins (HR = 3.87 [1.59-9.42]), were independently associated with worse survival.

CONCLUSIONS: In this national cohort of PHVM, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size, was strongly associated with worse survival after surgery. Additionally, residual tumor burden after resection, in the form of positive surgical margins or the presence of metastasis, was also negatively associated with survival. Long-term clinical outcomes remain poor for patients with the above high-risk features, emphasizing the need to develop effective forms of adjuvant systemic therapies for this group of malignancies.

PMID:33857792 | DOI:10.1016/j.jss.2021.02.043

Malignant transformation of monostotic fibrous dysplasia into angiosarcoma in the proximal tibia

Fetched: April 15th, 2021, 5:00am GMT by Selin Narter

Indian J Pathol Microbiol. 2021 Apr-Jun;64(2):394-397. doi: 10.4103/IJPM.IJPM_897_20.

ABSTRACT

The majority of bone angiosarcomas are primary tumors while secondary angiosarcomas arise after radiation therapy or bone infarctus. This article presents a case of malignant transformation of monostotic fibrous dysplasia into angiosarcoma. An 80-year-old female presented with pain on right cruris. Radiological examination revealed a lesion with lytic areas and destruction of cortical bone on right tibia. Gross and histopathological examination showed two areas with an abrupt transition. The solid component was composed of curved, immature bony trabeculae in a fibroblastic stroma. The other component involved epitheloid cells forming slit-like vascular spaces. The diagnosis of angiosarcoma and fibrous dysplasia was given. Malignant transformation of fibrous dysplasia into angiosarcoma is extremely rare; as this is the sixth case in the existing literature. Prognosis of fibrous dysplasia is generally good and less than 1% of the patients develop a malignant tumor. Therefore, patients with fibrous dysplasia should be offered a life-long follow-up.

PMID:33851644 | DOI:10.4103/IJPM.IJPM_897_20

Mohs Surgery Outside Usual Indications: A Review

Fetched: April 10th, 2021, 5:00am GMT by Pedro Rodríguez-Jiménez

Acta Dermatovenerol Croat. 2020 Dec;28(7):210-214.

ABSTRACT

The body of literature supporting the use of Mohs micrographic surgery (MMS) in tumors outside the main indications (basal cell carcinoma, squamous cell carcinoma, dermatofibrosacroma protuberans, lentigo maligna) is constantly growing, but it is still based on case reports, case series, or at best institutional case series that focus on a single malignancy. Our aim in this review was to assess use of MMS in an array of rare tumors outside the usual indications. A review was performed using the MEDLINE database and the search engine ClinicalKey®. We reviewed the use of MMS on atypical fibroxanthoma (AFX)/malignant fibrous histiocytoma, microcystic adnexal carcinoma, extramammary Paget's disease, Merkel cell carcinoma, pocrine/eccrine carcinoma/porocarcinoma, trichilemmal carcinoma, leiomyosarcoma, and angiosarcoma. Mohs micrographic surgery appears to be scarcely used in these tumors due to their low incidence. It is mainly performed for tumors in the H-zone of the face, and can be performed safely. The overall recurrence rate is lower compared with simple or wide local excision. MMS should be used in a more generalized fashion for these tumors.

PMID:33834992

Permalink for 'Epithelioid angiosarcoma of the popliteal artery- primary or secondary? A rare disease entity difficult to diagnose and easily overlooked Literature research and case report'

Epithelioid angiosarcoma of the popliteal artery- primary or secondary? A rare disease entity difficult to diagnose and easily overlooked Literature research and case report

Fetched: April 9th, 2021, 5:00am GMT by Ursula Elisabeth

Ann Vasc Surg. 2021 Apr 5:S0890-5096(21)00198-9. doi: 10.1016/j.avsg.2021.02.010. Online ahead of print.

ABSTRACT

INTRODUCTION: The popliteal artery is a common site of aneurysm formation, whereas sarcomas of the vascular system are very rare. The diagnosis is very difficult to establish. During our literature research we found only seven reports about angiosarcomas of the popliteal artery. Four of them were associated with aneurysms. Because of the poor prognosis early diagnosis is the key to successful treatment.

REPORT: We present a well-documented case of an 83-year-old patient with an angiosarcoma of the popliteal artery diagnosed as a popliteal artery aneurysm at first.

CONCLUSION: It is important to think of this rare, highly aggressive tumor entity. Especially the aneurysms that need revision surgery should cause suspicion- histological samples from the aneurysm wall should always be taken.

PMID:33831522 | DOI:10.1016/j.avsg.2021.02.010

Permalink for 'Neoplastic lesions in domestic pigs detected at slaughter: literature review and a 20-year review (1998-2018) of carcass inspection in Catalonia'

Neoplastic lesions in domestic pigs detected at slaughter: literature review and a 20-year review (1998-2018) of carcass inspection in Catalonia

Fetched: April 9th, 2021, 5:00am GMT by Antonia Morey-Matamalas

Porcine Health Manag. 2021 Apr 7;7(1):30. doi: 10.1186/s40813-021-00207-0.

ABSTRACT

BACKGROUND: The present paper reviews the occurrence of neoplasms in swine and presents a case series of 56 tumors submitted to the Slaughterhouse Support Network (Servei de Suport a Escorxadors [SESC] IRTA-CReSA]) from slaughtered pigs from 1998 to 2018 (April) in Catalonia (Spain). The aim of the study was to describe the spectrum of spontaneous neoplastic lesions found in slaughtered pigs and to compare the reported tumor cases with previous published data. Lymphoid neoplasms were characterized and classified using the WHO classification adapted for animals.

RESULTS: The most reported neoplasm during this period was lymphoma (28). Within lymphomas, the B-cell type was the most common, being the diffuse large B-cell lymphoma (15/28) the most represented subtype. Other submitted non-lymphoid neoplasms included melanoma (7), nephroblastoma (3), mast cell tumor (2), liposarcoma (2), osteochondromatosis (2), papillary cystadenocarcinoma (1), peripheral nerve sheath tumor (1), lymphoid leukemia (1), fibropapilloma (1), hemangiosarcoma (1), hepatoma (1), histiocytic sarcoma (1), pheochromocytoma (1) and osteosarcoma (1).

CONCLUSIONS: The existence of a well-established Slaughterhouse Support Network allowed the compilation of comprehensive data for further epidemiological and pathological studies, particularly about less commonly reported lesions in livestock such as neoplasms in pigs.

PMID:33827694 | PMC:PMC8025367 | DOI:10.1186/s40813-021-00207-0

Clinical characteristics and surgical treatments of primary hepatic angiosarcoma

Fetched: April 9th, 2021, 5:00am GMT by Lei Jiang

BMC Gastroenterol. 2021 Apr 7;21(1):156. doi: 10.1186/s12876-021-01743-3.

ABSTRACT

PURPOSE: Primary hepatic angiosarcoma is a very rare and highly malignant tumor with poor prognosis. It is difficult to diagnose because of the lack of typical clinical features, and the treatment protocols for PHA are also not clear. Therefore, this study wants to find out the clinical characteristics and surgical treatments of primary hepatic angiosarcoma.

METHODS: Among 8990 patients diagnosed with primary malignant tumor of the liver from January 2000 to December 2019 in our hospital, only four patients were diagnosed with primary hepatic angiosarcoma. The demographics, clinical manifestation, past history, serology test results, MRI features, pathology, treatment modality and prognosis of four patients were collected and analyzed.

RESULTS: Three of four patients had no clinical symptoms, while one patient's symptom was abdominal pain. The levels of tumor markers of all four patients were within the normal reference range and serological tests were negative for hepatitis B and C virus. The MRI imaging findings of all four patients were mixed mass with highly disordered vascular characteristics. All four patients were misdiagnosed preoperatively. One patient who underwent hepatic lobectomy was still alive for about 18 months after surgery. One patient who underwent hepatic lobectomy has survived for only 6 months due to severe pneumonia. The other two patients who received transarterial chemoembolization survived 16 months and 11 months respectively.

CONCLUSION: The clinical symptoms of primary hepatic angiosarcoma are not typical, and primary hepatic angiosarcoma is easily misdiagnosed. The typical imaging manifestations are structural disorder and heterogeneous tumor. Hepatic lobectomy and transarterial chemoembolization may be important surgical treatments to improve the prognosis of patients.

PMID:33827427 | PMC:PMC8028144 | DOI:10.1186/s12876-021-01743-3

An Incidental Finding of Scalp Angiosarcoma: A Case Report

Fetched: April 6th, 2021, 5:00am GMT by Akram F Alwarqi

Cureus. 2021 Feb 28;13(2):e13610. doi: 10.7759/cureus.13610.

ABSTRACT

Scalp angiosarcoma is a malignant tumor of the vascular endothelial cells. We present the case of an elderly male patient with a history of urinary bladder transitional cell cancer and trauma (falling on his head) who came to the emergency department with scalp swelling, which was found on brain imaging to infiltrate into the skull, reaching the dural matter. A biopsy was done, which showed angiosarcoma, which is rare for that area. Further studies are recommended to establish if there is a possible genetic association between both cancers (urinary bladder transitional cell cancer and scalp angiosarcoma) as both arise from endothelial cells.

PMID:33816009 | PMC:PMC8009560 | DOI:10.7759/cureus.13610

$Permalink for 'Primary breast angiosarcoma with disseminated intravascular coagulation is successfully treated with self-subcutaneous unfractionated heparin calcium injection: A case report'$

Primary breast angiosarcoma with disseminated intravascular coagulation is successfully treated with self-subcutaneous unfractionated heparin calcium injection: A case report

Fetched: April 6th, 2021, 5:00am GMT by Toshinari Yagi

Mol Clin Oncol. 2021 May;14(5):104. doi: 10.3892/mco.2021.2266. Epub 2021 Mar 17.

ABSTRACT

Angiosarcoma is a rare sarcoma with a poor prognosis and is prone to disseminated intravascular coagulation (DIC), where DIC often interferes with chemotherapy. Primary angiosarcoma of the breast (PASB) is a type of angiosarcoma that is located in mammary parenchyma and is not associated with radiation exposure. The current study reported a 47-year-old female with DIC associated with PASB. The DIC of the patient relapsed during mono-chemotherapy with paclitaxel (PTX) after first-line anticoagulant therapy using thrombomodulin-α. The second-line danaparoid sodium therapy, followed by self-subcutaneous injection of unfractionated heparin calcium (UFH), resulted in long-term stabilization of DIC. Under this second-line anticoagulant therapy, the patient continued chemotherapy and chemoradiotherapy for >13 months in the outpatient setting without impairment of quality of life. The present case suggested that self-subcutaneous injections of UFH may be a useful therapeutic option for long-term control of DIC associated with PASB. However, further prospective clinical trails are needed to verify the efficacy of self-subcutaneous injection of UFH in similar settings.

PMID:33815793 | PMC:PMC8010513 | DOI:10.3892/mco.2021.2266

Proposal to Eliminate Urethane-Treated Positive Control Dose Groups in 26-Week Tg.rasH2 Carcinogenicity Studies

Fetched: April 6th, 2021, 5:00am GMT by Madhav G Paranjpe

Int J Toxicol. 2021 Apr 5:10915818211003308. doi: 10.1177/10915818211003308. Online ahead of print.

ABSTRACT

Short-term (26 weeks) Tg.rasH2 mouse carcinogenicity studies have been conducted as an alternative model to the conventional 2-year mouse carcinogenicity studies, using urethane as a positive control material. In these studies, urethane was used at a dose of 1,000 mg/kg/dose, administered intraperitoneally on days 1, 3, and 5. Urethane consistently produces lung adenomas and carcinomas and hemangiosarcomas of the spleen, proving validity of the assay. We conducted 3 pilot studies at 3 different sites of Charles River Laboratories using a lower dose of urethane (500 mg/kg/dose), administered on days 1, 3, and 5, followed by a 12-week observation period. Our results demonstrate that a lower dose can be used successfully with fewer number of animals per sex to prove the validity of the assay. However, based on our cumulative experience with this model, we propose to eliminate positive control dose groups in future Tg.rasH2 carcinogenicity studies.

PMID:33813924 | DOI:10.1177/10915818211003308

Correlation between Tumour Associated Macrophage (TAM) Infiltration and Mitotic Activity in Canine Soft Tissue Sarcomas

Fetched: April 4th, 2021, 5:00am GMT by Riccardo Finotello

Animals (Basel). 2021 Mar 4;11(3):684. doi: 10.3390/ani11030684.

ABSTRACT

Tumour-associated macrophages (TAMs) are an important part of the tumour microenvironment but knowledge of their distribution in canine soft tissue sarcomas (STSs) is limited to absent. We analysed 38 STSs retrieved from the veterinary pathology archive; oral and visceral STSs, synovial cell sarcoma, tumours of histiocytic origin, haemangiosarcoma, carcinosarcomas, and undifferentiated tumours were excluded. Iba-1 positive, non-neoplastic tumour infiltrating cells (morphologically indicative of macrophages) were classified as TAMs and were counted in 10 consecutive tumours areas, where no necrosis or other inflammatory cells could be identified. Associations between numbers of TAMs and mitoses, differentiation, and necrosis scores or grade were investigated. TAMs were evident in all STSs and ranged between 6% to 62% of the cells in the microscopic field. The number of TAMs positively correlated with the STSs' histologic grade. When the components of the grade were analysed separately, TAMs were statistically correlated with mitoses, but not with differentiation or necrosis score. The present findings suggest that TAMs are present in higher numbers when STS proliferation is the predominant feature that drives tumour grade. The abundant presence of TAMs in high-grade STSs may also increase the likelihood of a pathologist misdiagnosing STS for histiocytic sarcoma.

PMID:33806407 | PMC:PMC8000832 | DOI:10.3390/ani11030684

CD11c-CD8 Spatial Cross Presentation: A Novel Approach to Link Immune Surveillance and Patient Survival in Soft Tissue Sarcoma

Fetched: April 4th, 2021, 5:00am GMT by Yanhong Su

Cancers (Basel). 2021 Mar 9;13(5):1175. doi: 10.3390/cancers13051175.

ABSTRACT

Checkpoint inhibitors are slowly being introduced in the care of specific sarcoma subtypes such as undifferentiated pleomorphic sarcoma, alveolar soft part sarcoma, and angiosarcoma even though formal indication is lacking. Proper biomarkers to unravel potential immune reactivity in the tumor microenvironment are therefore expected to be highly warranted. In this study, intratumoral spatial cross presentation was investigated as a novel concept where immune cell composition in the tumor microenvironment was suggested to act as a proxy for immune surveillance. Double immunohistochemistry revealed a prognostic role of direct spatial interactions between CD11c+ antigen-presenting cells (APCs) and CD8+ cells in contrast to each marker alone in a soft tissue sarcoma (STS) cohort of 177 patients from the Karolinska University Hospital (MFS p = 0.048, OS p = 0.025). The survival benefit was verified in multivariable analysis (MFS p = 0.012, OS p = 0.004). Transcriptomics performed in the TCGA sarcoma cohort confirmed the prognostic value of combining CD11c with CD8 (259 patients, p = 0.005), irrespective of FOXP3 levels and in a CD274 (PD-LI)-rich tumor microenvironment. Altogether, this study presents a histopathological approach to link immune surveillance and patient survival in STS. Notably, spatial cross presentation as a prognostic marker is distinct from therapy response-predictive biomarkers such as immune checkpoint molecules of the PD-L1/PD1 pathway.

PMID:33803245 | PMC:PMC7967210 | DOI:10.3390/cancers13051175

B-Mode and Contrast Enhanced Ultrasonography Features of Gastric Inflammatory and Neoplastic Diseases in Dogs

Fetched: April 4th, 2021, 5:00am GMT by Francesco Simeoni

Animals (Basel). 2021 Mar 3;11(3):670. doi: 10.3390/ani11030670.

ABSTRACT

Canine gastric disorders are common in veterinary clinical practice and among these neoplasms require rapid identification and characterization. Standard ultrasound (US) is the imaging modality of choice for gastric wall assessment. The aim of this prospective study is to describe the specific B-mode and contrast enhanced US (CEUS) features of normal, inflammatory, and neoplastic gastric wall in dogs. B-mode US and CEUS of the stomach were performed in anesthetized dogs with or without gastric disorders. Gastric wall qualitative and quantitative parameters were evaluated on B-mode US and CEUS examination. A total of 41 dogs were included: 6 healthy (HEA) as the control group; 9 gastritis (INF); 8 adenocarcinoma (AC); 8 alimentary lymphoma (AL); 4 leiomyosarcoma (LEIS); 2 gastrointestinal stromal tumor (GIST); 2 leiomyoma; 1 undifferentiated sarcoma; 1 metastatic gastric hemangiosarcoma. Gastric tumors appear as a marked wall thickness with absent layers definition and possible regional lymphadenopathy (AC and AL) and steatitis (AC) while gastritis generally shows no/mild thickening and no other alterations on B-mode US. On CEUS, neoplasm shows a higher and faster wash in if compared to that of gastritis. B-mode and CEUS assessment may be useful in the evaluation of canine gastric disorders in the distinction between gastritis and gastric neoplasms, even if there are no specific features able to discriminate between the different tumor histotypes.

PMID:33802392 | PMC:PMC7998525 | DOI:10.3390/ani11030670

Permalink for 'Treatment of Angiosarcoma with Pazopanib and Paclitaxel: Results of the EVA (Evaluation of Votrient() in Angiosarcoma) Phase II Trial of the German Interdisciplinary Sarcoma Group (GISG-06)'

Treatment of Angiosarcoma with Pazopanib and Paclitaxel: Results of the EVA (Evaluation of Votrient() in Angiosarcoma) Phase II Trial of the German Interdisciplinary Sarcoma Group (GISG-06)

Fetched: April 4th, 2021, 5:00am GMT by Daniel Pink

Cancers (Basel). 2021 Mar 11;13(6):1223. doi: 10.3390/cancers13061223.

ABSTRACT

We aimed to evaluate the efficacy and toxicity of paclitaxel combined with pazopanib in advanced angiosarcoma (AS). The primary end point was progression-free survival (PFS) rate at six months (PFSR6). Planned accrual was 44 patients in order to detect a PFSR6 of >55%, with an interim futility analysis of the first 14 patients. The study did not meet its predetermined interim target of 6/14 patients progression-free at 6 months. At the time of this finding, 26 patients had been enrolled between July 2014 and April 2016, resulting in an overrunning of 12 patients. After a median follow-up of 9.5 (IQR 7.7-15.4) months, PFSR6 amounted to 46%. Two patients had a complete and seven patients a partial response. Patients with superficial AS had a significantly higher PFSR6 (61% vs. 13%, p = 0.0247) and PFS (11.3 vs. 2.7 months, p < 0.0001) compared to patients with visceral AS. The median overall survival in the entire cohort was 21.6 months. A total of 10 drug-related serious adverse effects were reported in 5 patients, including a fatal hepatic failure. Although our study did not meet its primary endpoint, the median PFS of 11.6 months in patients with superficial AS appears to be promising. Taking recent reports into consideration, future studies should evaluate the safety and efficacy of VEGFR and immune checkpoint inhibitors with or without paclitaxel in a randomized, multiarm setting.

PMID:33799576 | PMC:PMC8000466 | DOI:10.3390/cancers13061223

Surgical and multimodality treatment of cardiac sarcomas: A systematic review and meta-analysis

Fetched: April 3rd, 2021, 5:00am GMT by Saeed Torabi

J Card Surg. 2021 Apr 2. doi: 10.1111/jocs.15538. Online ahead of print.

ABSTRACT

INTRODUCTION: Primary cardiac sarcomas (PCSs) are an extremely rare and aggressive type of malignancies that have been described only by a limited number of observational studies. This study aimed to evaluate the currently existing evidence comparing surgical to multimodality treatment of PCS.

METHODS: We systematically reviewed Embase, MEDLINE, Cochrane Database, and Google Scholar, from inception to December 2020, for original articles about surgical and multimodality treatment of PCS. The outcomes included were mortality at various time points, resection margin status, and mean estimated survival. The pooled treatment effects were calculated using a random-effects model.

RESULTS: Ten studies including a total of 1570 patients met our inclusion criteria. Surgery was associated with significantly lower mortality when compared to conservative treatment at 1, 2, and 3 years, whereas no significant difference was found at 5 years. Furthermore, multimodality treatment showed significantly lower mortality at 1 year when compared to surgery alone, but not at 2 and 5 years. We found no difference in mortality between angiosarcomas and other PCS subtypes.

CONCLUSION: Overall, surgery was found to provide a significant mortality advantage to PCS patients up to 3 years following treatment. Multimodality treatment might be of additional benefit, although only within the first year. Prospective randomized studies are needed to further explore these differences in the treatment of PCS.

PMID:33797789 | DOI:10.1111/jocs.15538

Permalink for 'Metastasis of primary breast angiosarcoma to axillary and supraclavicular lymph nodes: a rare case diagnosed using imaging data'

Metastasis of primary breast angiosarcoma to axillary and supraclavicular lymph nodes: a rare case diagnosed using imaging data

Fetched: April 1st, 2021, 5:00am GMT by Xiaojuan Qin

J Int Med Res. 2021 Mar;49(3):3000605211002337. doi: 10.1177/03000605211002337.

ABSTRACT

Primary breast angiosarcoma (PBA) is a rare malignant tumor. PBA usually undergoes hematogenous metastasis; lymph node metastasis is very rare in such patients, and metastasis of PBA to the supraclavicular lymph nodes has not previously been reported. Here, we describe a rare case of PBA manifested by a diffuse enlargement of the left breast, with metastasis to the left axillary and bilateral supraclavicular lymph nodes. Contrast-enhanced ultrasound and positron emission tomography findings indicated a malignant lesion, whereas magnetic resonance imaging suggested a benign lesion. Core needle biopsy identified the lesion as a lymphangioma, and the histological characteristics suggested a high-grade angiosarcoma. Multimodal imaging and perfusion patterns obtained using various contrast agents can thus help to diagnose PBA.

PMID:33784853 | PMC:PMC8020107 | DOI:10.1177/03000605211002337

CT Findings of Primary Renal Angiosarcoma

Fetched: March 30th, 2021, 5:00am GMT by Hisashi Komoto

Case Rep Oncol. 2021 Mar 1;14(1):212-216. doi: 10.1159/000512015. eCollection 2021 Jan-Apr.

ABSTRACT

Primary angiosarcomas of the kidney are very rare but highly aggressive tumors showing poor prognosis. We present a case of primary renal angiosarcoma occurring in a 60-year-old man with left flank pain. CT images depicted a huge exophytic mass (14 cm in diameter) in the left kidney, exhibiting central extensive hemorrhage or necrosis without contrast enhancement. The mass showed centripetal peripheral nodular enhancement on dynamic contrast-enhanced CT images. We suggest its inclusion in the differential diagnosis of cases of hemorrhagic renal tumors with prominent vasculature.

PMID:33776706 | PMC:PMC7983682 | DOI:10.1159/000512015

Infiltrating Angiosarcoma of the Ascending, Arch and Descending Aorta Manifested as Acute Mesenteric Ischemia

Fetched: March 30th, 2021, 5:00am GMT by Giuseppe Deiana

Vasc Specialist Int. 2021 Mar 31;37(1):46-49. doi: 10.5758/vsi.200083.

ABSTRACT

Primary malignant aortic tumors are rare and aggressive. Most cases are diagnosed at advanced stages or during autopsies with a median overall survival of 8 months from diagnosis. We present the case of a 59-year-old male with angiosarcoma involving all segments of the thoracic aorta and a large floating thrombus causing acute mesenteric ischemia, which was treated successfully with embolectomy. Graft replacement of the aorta should be considered in cases of localized disease and when patients are fit for surgery. The best medical and surgical treatment remains unclear, and further studies are needed.

PMID:33775936 | PMC:PMC8021497 | DOI:10.5758/vsi.200083

Primary Angiosarcomas Of The Bone: An Evaluation Of 4 Cases

Fetched: March 29th, 2021, 5:00am GMT by Selçuk Yılmaz

J Ayub Med Coll Abbottabad. 2021 Jan-Mar;33(1):150-154.

ABSTRACT

Angiosarcoma is a rare mesenchymal neoplasm that may arise from vascular or lymphatic tissue. Angiosarcoma of bone is a rare high-grade malignant vascular tumour, representing less than 1% of all angiosarcomas. The most common locations of unifocal tumour are the long and short tubular bones, followed by the pelvis, and trunk. The literature regarding treatment and outcome of patients with this tumour is limited. We performed a retrospective study to analyse treatment and survival of four patients with angiosarcoma of bone.

PMID:33774973

GATA binding protein 3 (GATA3) as a marker for metaplastic spindle cell carcinoma of the breast

Fetched: March 28th, 2021, 5:00am GMT by Cao Jin

Pathol Res Pract. 2021 Mar 20;221:153413. doi: 10.1016/j.prp.2021.153413. Online ahead of print.

ABSTRACT

Spindle cell lesions of the breast comprise a diverse set of tumors; harboring significant histological and immunohistochemical (IHC) overlap. Accurate diagnosis and classification of spindle cell lesions in the breast remains challenging, especially in core biopsies. In the current study, we evaluated a spectrum of spindle cell lesion of the breast with a panel of IHC antibodies in an effort to differentiate metaplastic spindle cell carcinoma from its benign and malignant mimickers. Our study included 92 patients who underwent breast core biopsies or breast resections at Northwell Health who were diagnosed with benign and malignant tumor/tumor-like spindle cell lesions. Tumors subtypes in this the study included: angiosarcoma, nodular fasciitis, fibromatosis, myofibroblastoma, phyllodes tumors (benign, borderline and malignant), primary sarcomas and metaplastic spindle cell carcinoma. Our biomarker panel included high molecular weight keratin (HMWK), CAM5.2, AE1/AE3, p63, CD34 and GATA3. GATA3 expression was significantly higher in metaplastic carcinomas (88.9 % vs 4.1 %, p < 0.001), when compared to other spindle cell lesions. The sensitivity and specificity for detecting metaplastic carcinomas reached 84.2 % and 97.3 %, respectively. Regarding cytokeratin panels, none of the three individual markers were as sensitive or specific for metaplastic breast carcinoma. GATA3 is the most specific and sensitive marker forfor the identification of metaplastic spindle cell carcinoma of the breast.

PMID:33770555 | DOI:10.1016/j.prp.2021.153413

A rare cardiac tumor presenting with myxoma: Primary cardiac hemangioendothelioma

Fetched: March 27th, 2021, 5:00am GMT by Ertürk Karaağaç

Turk Gogus Kalp Damar Cerrahisi Derg. 2021 Jan 13;29(1):110-113. doi: 10.5606/tgkdc.dergisi.2021.20695. eCollection 2021 Jan.

ABSTRACT

Epithelioid hemangioendothelioma is a rare malignant vascular tumor which originates from the vascular endothelial or dendritic cells and has a moderate malignancy between benign hemangioma and malignant hemangiosarcoma, arising from vascular structures. Radical resection of epithelioid hemangioendothelioma is recommended, due to both its malignant nature and the adverse effects of associated complications on mortality and morbidity. In this article, we present a rare case of primary cardiac epithelioid hemangioendothelioma accompanied by a left atrial myxoma which was treated by resection.

PMID:33768989 | PMC:PMC7970082 | DOI:10.5606/tgkdc.dergisi.2021.20695

Primary central nervous system angiosarcoma with recurrent acute subdural hematoma

Fetched: March 27th, 2021, 5:00am GMT by Marc Valera-Melé

Neurocirugia (Astur). 2021 Mar 22:S1130-1473(21)00027-0. doi: 10.1016/j.neucir.2021.02.002. Online ahead of print.

ABSTRACT

Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.

PMID:33766476 | DOI:10.1016/j.neucir.2021.02.002

PDGFB RNA in situ hybridization for the diagnosis of dermatofibrosarcoma protuberans

Fetched: March 26th, 2021, 5:00am GMT by Jeffrey M Cloutier

Mod Pathol. 2021 Mar 24. doi: 10.1038/s41379-021-00800-2. Online ahead of print.

ABSTRACT

Dermatofibrosarcoma protuberans (DFSP) is a spindle cell neoplasm of the skin and superficial soft tissue with a tendency for locally aggressive behavior; metastatic potential coincides with fibrosarcomatous transformation. The vast majority of DFSPs harbor the t(17;22) translocation resulting in a COL1A1-PDGFB fusion that drives autocrine growth stimulation via PDGFB overexpression. Here, we examined the utility of PDGFB RNA chromogenic in situ hybridization (CISH) for the diagnosis of DFSP. A total of 337 tumors represented in whole tissue sections and tissue microarrays, including 37 cases of DFSP and 300 histologically similar spindle cell tumors, were subjected to PDGFB RNA CISH using commercially available probes. PDGFB overexpression was observed by light microscopy in 24 of 26 conventional DFSPs (92%) and 11 of 11 fibrosarcomatous DFSPs (100%). One of two DFSPs negative for PDGFB by RNA CISH was found to harbor an uncommon alternative rearrangement involving PDGFD. All examined cases of histologic mimics were negative for PDGFB overexpression; limited PDGFB expression, not reaching an empirical threshold of greater than 5 puncta or one aggregate of chromogen in more than 25% of cells, was observed in 7 of 300 mimics (2.3%), including desmoplastic melanoma, malignant peripheral nerve sheath tumor, angiosarcoma, and pleomorphic dermal sarcoma. Vascular PDGFB expression was seen in several tumor types. We conclude that PDGFB RNA CISH, with careful interpretation and the use of appropriate thresholds, may serve as a surrogate marker of PDGFB rearrangement and a useful ancillary tool for the diagnosis of DFSP.

PMID:33762682 | DOI:10.1038/s41379-021-00800-2

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