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