A compilation of tumors associated with EWSR1 rearrangement. EWSR1 rearrangements were first identified in Ewing sarcoma, but EWSR1-rearranged neoplasms now includes many tumour subtypes. EWSR1 gene is located in chromosome 22q12. Here is a compilation of tumors associated with EWSR1 rearrangement. This includes latest updates from the WHO 2020 5th edition of soft tissue and bone tumors. TUMORS ASSOCIATED WITH EWSR1-REARRANGEMENT Ewings sarcomaDesmoplastic small round cell tumor (DSRCT)Extraskeletal myxoid chondrosarcoma (EMC)Angiomatoid fibrous histiocytoma (AFH) Clear cell sarcoma (CCS)Primary pulmonary myxoid sarcoma (PPMS) Salivary gland hyalinizing clear cell carcinomasUndifferentiated round cell sarcomas with EWSR-non ETS fusion- NEW ENTITY EWSR1 – SMAD3 fibroblastic tumor PROVISIONAL ENTITY Myxoid liposarcoma (MLPS)Clear cell sarcoma of the gastrointestinal tract (CCSGT)Mesothelioma AN EASY WAY TO REMEMBER 1.Ewings sarcoma Common EWSR1 fusion transcripts associated with Ewing’s sarcoma. t(11;22)(q24;q12)of FLI1-EWSR1 genest(21;22)(q12q12)of ERG-EWSR1 genes 2.Desmoplastic small round cell tumor (DSRCT) Has pathogenic balanced translocation t(11;22)(p13;q12) of the EWSR1 gene to the Wilms tumor (WT1 gene) 3.Extraskeletal myxoid chondrosarcoma (EMC) t(9;22)(q22-31;q11-12): CHN-EWS fusion gene, is consistent marker for extraskeletal myxoid chondrosarcoma 4.Angiomatoid fibrous histIocytoma (AFH) t(12;22)(q13;q12) creating ATF1-EWSR1 genet(2;22)(q33;q12) creating CREB1-EWSR1 gene However, the most common rearrangement in angiomatoid fibrous histoocytoma is t(12;16)(q13;p11) creating ATF1-FUS fusion. 5.Clear cell sarcoma (CCS) t(12;22)(q13;q12): ATF1 and EWSR1 6.Primary pulmonary myxoid sarcoma (PPMS) EWSR1 rearrangement is seen in 85% cases. Fusion partner is usually ATF1 same as AFH and CSS. 7.Salivary gland hyalinizing clear cell carcinomas > 80% of cases have EWSR1-ATF1 fusion 8.Undifferentiated round cell sarcomas with EWSR-non ETS fusion- NEW ENTITY EWSR1-NFATC2EWSR1-PATZ1 Both have distinct morphologic features.- CHECK BELOW 9.EWSR1 – SMAD3 fibroblastic tumor PROVISIONAL ENTITY EWSR1-SMAD1 fusion HERE IS A QUICK SUMMARY 10.Myxoid liposarcoma (MLPS) t(12;22)(q13;p11) – EWSR1-DDIT3 -rare Most common translocation associated with t(12;16)(q13;p11) – FUS-DDIT3 is seen in 90% 11.Clear cell sarcoma of the gastrointestinal tract (CCSGT) EWSR1-CREB fusion 12.Mesothelioma EWSR1-YY1 appears to be uncommon Did we miss out anything? Leave a reply. Ref: WHO 5th edition soft tissue and bone tumors 2020 PARIS SYSTEM FOR REPORTING URINARY CYTOLOGY MCQs by Pathology MCQs 22 Mar 2022 Breast Pathology MCQ 1 by Pathology MCQs 12 Mar 2022 CNS Pathology case based MCQs-2 by Pathology MCQs 23 Feb 2022 Share this:FacebookTelegramWhatsAppMoreLinkedInTwitterLike this:Like Loading...
SOFT TISSUE AND BONE TUMOR PATHOLOGY QUIZ SOFT TISSUE AND BONE TUMORS PATHOLOGY QUIZ. USEFUL FOR NEET-SS/ DM- Oncopathology/ DM HistopathologyOncopathology FellowshipsFRCPath- Histopathology and American Board of anatomic and clinical pathologyVarious other boards. CLICK HERE TO ATTEMPT THE QUIZ Click HERE for pathology quizzes RECENT UPDATES FROM WHO 2020 SOFT TISSUE AND BONE TUMORS EWSR1-SMAD3 fibroblastic tumour Undifferentiated small round cell sarcomas of bone and soft tissues A compilation of tumors associated with EWSR1 rearrangement. OTHER SBT BLOGS Tumors characterized by loss of SMARCB1/INI1 CHENILLE BODIES IN ELASTOFIBROMA ILLUSTRATIONS ON THE TOPIC Share this:FacebookTelegramWhatsAppMoreLinkedInTwitterLike this:Like Loading...
EWSR1-SMAD3 fibroblastic tumour EWSR1-SMAD3 fibroblastic tumour- WHO 2020 UPDATE. EWSR1-SMAD3 fibroblastic tumour represents one of the new entities in which the name is determined by the involved fusion gene. It is a benign neoplasm most often occurring in the hands and feet with a broad age range. Morphologically it is composed of intersecting cellular fascicles of cytologically bland monomorphic spindle cells, alternating with hypocellular, hyalinised areas. Immunohistochemically it is characterized by nuclear expression of ERG. CNS Pathology case based MCQs-1 by Pathology MCQs 22 Feb 2022 Central nervous system pathology MCQ 1 by Pathology MCQs 13 Feb 2022 Characteristics of Epithelial-Mesenchymal transition by Pathology MCQs 7 Dec 2021 Share this:FacebookTelegramWhatsAppMoreLinkedInTwitterLike this:Like Loading...
CHENILLE BODIES IN ELASTOFIBROMA The fragments of elastic fibers seen in elastofibroma are typically arranged linearly, creating a “beads on a string” appearance, and are known as chenille bodies. FOR LATEST BLOGS RB-1 DELETED SOFT TISSUE TUMORS by Pathology MCQs 6 Dec 2021 SALIVARY GLAND PATHOLOGY QUIZ by Pathology MCQs 16 Nov 2021 THYROID PATHOLOGY QUIZ by Pathology MCQs 26 Oct 2021 Share this:FacebookTelegramWhatsAppMoreLinkedInTwitterLike this:Like Loading...