Mesenchymal Tumors–Sarcoma: A New AEC Work Group

Publication date: Available online 6 November 2018Source: Cirugía Española (English Edition)Author(s): Vicente Artigas Raventós
Source: Cirugia Espanola - Category: Surgery Source Type: research

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We report the case of a 47-year-old man who was diagnosed with severe right ventricular outflow tract (RVOT) stenosis due to a space-occupying lesion; the diagnosis was made using computed tomography. He underwent mass reduction, pulmonary valve replacement, and RVOT reconstruction with a bovine pericardial patch. The pathological diagnosis was undifferentiated pleomorphic sarcoma originating from the myocardium. As the mass resection was incomplete, he received heavy particle therapy. He did not want to receive adjuvant chemotherapy. Four months later, severe RVOT stenosis recurred because the residual mass had invaded th...
Source: Journal of Cardiology Cases - Category: Cardiology Source Type: research
We report a rare case of primary yolk sac tumour of the prostate with extensive local and liver metastasis, the latter of which exhibited sheets of small blue cells expressing CD99 and focal sall4 on biopsy. Positivity for CD99 and gata3 in the initial biopsy raised the differential diagnosis of Ewing sarcoma and poorly differentiated carcinoma. The primary tumour demonstrated an admixture of solid and glandular growth patterns and occasional Schiller-Duval bodies. A panel of immunohistochemical stains showing positivity for AE1/3, sall4, cdx2, and focal alpha-fetoprotein, and negativity for oct-4, facilitated the diagnosi...
Source: Current Oncology - Category: Cancer & Oncology Tags: Curr Oncol Source Type: research
ATF1, CREB1, and CREM constitute the CREB family of transcription factors. The genes encoding these factors are involved in gene fusion events in human tumors. EWSR1-ATF1 and EWSR1-CREB1 are the 2 most characterized fusions, whereas EWSR1-CREM has been less studied. To better understand the phenotypic spectrum of mesenchymal tumors associated with the EWSR1-CREM fusion, we investigated archival cases using fluorescence in situ hybridization and/or RNA sequencing. Among 33 clear cell sarcomas of soft tissue tested, we found 1 specimen, a hand tumor bearing the rearrangements of EWSR1 and CREM, with classic histology and imm...
Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research
The entity “pericytoma with t(7;12)” was described as a rare, distinct perivascular myoid neoplasm provisionally classified within the family of myopericytic tumors that demonstrates t(7;12)(p22;q13) translocation with resultant ACTB-GLI1 fusion and biologically was felt to behave in an indolent fashion. However, a recent study showed that tumors with this and similar translocations may have variable morphology and immunohistochemical phenotype with inconsistent myopericytic characteristics and a propensity for metastasis, raising questions regarding the most appropriate classification of these neoplasms. Herei...
Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research
Publication date: Available online 11 November 2019Source: Pathology - Research and PracticeAuthor(s): Heejin Bang, Soomin Ahn, Eun Ji Kim, Seung Tae Kim, Ha Young Park, Jeeyun Lee, Kyoung-Mee KimABSTRACTRapamycin-insensitive companion of mTOR (RICTOR) is a key component of mammalian target of rapamycin (mTOR) complex 2 (mTORC2), and promotes cellular proliferation and survival through the activation of downstream AGC kinase family members. The amplification of RICTOR has been proposed as a therapeutically relevant genomic alteration. However, other than next-generation sequencing, precise diagnostic methods to detect RICT...
Source: Pathology Research and Practice - Category: Pathology Source Type: research
Authors: Endo G, Nagata N Abstract We herein report a case of gastrointestinal (GI) Kaposi's sarcoma (KS) without cutaneous involvement in a 73-year-old man who had received immunosuppressive drugs for granulomatosis with polyangiitis. After one year of prednisolone use, he presented with tarry stool and severe anemia. Endoscopic and pathological examinations revealed bright-reddish protruding lesions with proliferating spindle cells positive for D2-40, CD34, and HHV-8, which are definitively diagnostic of GI-KS. Drug-induced KS without HIV infection or transplantation is extremely rare, and its clinical features r...
Source: Internal Medicine - Category: Internal Medicine Tags: Intern Med Source Type: research
Conclusion.Regorafenib at a lower dose on a continuous schedule might be an alternative treatment in patients with GISTs after failure of imatinib and sunitinib. Clinical trial identification number. NCT02889328Implications for Practice.Regorafenib at the standard intermittent dosing schedule proved effective in the GRID trial for refractory gastrointestinal stromal tumors (GISTs). However, this dosing schedule requires frequent dose reduction, and the progression of GISTs or tumor‐related symptoms during the off‐treatment period has been noted in some patients. This study was to evaluate the efficacy and safety of reg...
Source: The Oncologist - Category: Cancer & Oncology Authors: Tags: Sarcomas Source Type: research
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Source: Journal of Maternal-Fetal and Neonatal Medicine - Category: Perinatology & Neonatology Authors: Source Type: research
Publication date: Available online 10 November 2019Source: Seminars in Cancer BiologyAuthor(s): Jessica Burns, Christopher P Wilding, Robin L Jones, Paul H HuangAbstractSarcomas are a rare group of mesenchymal cancers comprising over 70 different histological subtypes. For the majority of these diseases, the molecular understanding of the basis of their initiation and progression remains unclear. As such, limited clinical progress in prognosis or therapeutic regimens have been made over the past few decades. Proteomics techniques are being increasingly utilised in the field of sarcoma research. Proteomic research efforts h...
Source: Seminars in Cancer Biology - Category: Cancer & Oncology Source Type: research
This study aimed to analyze AS prognostic factors in a large nationwide cohort of histologically confirmed cases, established through linkage of clinical data from the Netherlands Cancer Registry and pathology data from the Dutch pathology registry (PALGA). All cases were reviewed by an expert pathologist, showing a 16% discordance rate. Multivariable Cox regression survival analysis among 479 confirmed AS patients revealed remarkably poorer overall survival (OS) for primary AS compared to secondary AS (7 vs 21 months, Hazard ratio (HR) = 1.5; 95% confidence interval (CI) = 1.2–1.9). Age above 65 years, male gend...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Article Source Type: research
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