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Intraluminal EWSR1-CREB1 gene rearranged, low-grade myxoid sarcoma of the pulmonary artery resembling extraskeletal myxoid chondrosarcoma (EMC)


Opitz, Isabelle; Lauk, Olivia; Schneiter, Didier; Ulrich, Silvia; Maisano, Francesco; Weder, Walter; Bode-Lesniewska, Beata (2019). Intraluminal EWSR1-CREB1 gene rearranged, low-grade myxoid sarcoma of the pulmonary artery resembling extraskeletal myxoid chondrosarcoma (EMC). Histopathology, 74(3):526-530.

Abstract

Fusions of the EWSR1 gene with partners from the CREB gene family have been identified in several histologic entities, mostly mesenchymal tumors of variable clinical malignant potential, including angiomatoid fibrous histiocytomas (AFH), clear cell sarcomas (CCS), clear cell sarcoma‐like tumors of the gastrointestinal tract (CCSLTGT), primary pulmonary myxoid sarcoma (PPMS), intracranial myxoid neoplasias (iMN) and rare carcinoma variants. Interestingly, some of EWSR1‐CREB‐family gene fusion associated tumors show a prominent myxoid component and some of such tumors were histologically in the past diagnosed as “consistent with extraskeletal myxoid chondrosarcomas (EMC)”.

Abstract

Fusions of the EWSR1 gene with partners from the CREB gene family have been identified in several histologic entities, mostly mesenchymal tumors of variable clinical malignant potential, including angiomatoid fibrous histiocytomas (AFH), clear cell sarcomas (CCS), clear cell sarcoma‐like tumors of the gastrointestinal tract (CCSLTGT), primary pulmonary myxoid sarcoma (PPMS), intracranial myxoid neoplasias (iMN) and rare carcinoma variants. Interestingly, some of EWSR1‐CREB‐family gene fusion associated tumors show a prominent myxoid component and some of such tumors were histologically in the past diagnosed as “consistent with extraskeletal myxoid chondrosarcomas (EMC)”.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Vascular Surgery
04 Faculty of Medicine > University Hospital Zurich > Institute of Pathology and Molecular Pathology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Pneumology
04 Faculty of Medicine > University Hospital Zurich > Clinic for Thoracic Surgery
Dewey Decimal Classification:610 Medicine & health
Uncontrolled Keywords:Pathology and Forensic Medicine, Histology, General Medicine
Language:English
Date:1 February 2019
Deposited On:01 Nov 2018 07:36
Last Modified:16 Oct 2019 00:00
Publisher:Wiley-Blackwell Publishing, Inc.
ISSN:0309-0167
OA Status:Green
Publisher DOI:https://doi.org/10.1111/his.13773
PubMed ID:30326150

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