Important Recently Characterized Non-Ewing Small Round Cell Tumors - 31/01/19

Doi : 10.1016/j.path.2018.10.008

Cody S. Carter, MD ^a, Rajiv M. Patel, MD ^b,^c,^⁎
^a Department of Pathology, Michigan Medicine, University of Michigan, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109, USA
^b Department of Pathology, Sections of Dermatopathology and Bone and Soft Tissue Pathology, Michigan Medicine, University of Michigan, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109, USA
^c Department of Dermatology, Michigan Medicine, University of Michigan, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA

^∗Corresponding author. Department of Pathology, Michigan Medicine, University of Michigan, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109.Department of PathologyMichigan MedicineUniversity of Michigan2800 Plymouth RoadBuilding 35Ann ArborMI48109

Abstract

Round cell sarcomas morphologically similar to Ewing sarcoma, but lacking the classic immunohistochemical features, EWSR-ETS family fusions, and other signs of differentiation, are classified as Ewing-like sarcomas. Recent molecular advances led to the discovery and characterization of two recurrent oncogenic fusion rearrangements, CIC-DUX4 and BCOR-CCNB3, in a significant subset of Ewing-like sarcomas. Uncovered alternate fusion partners broadened the proposed classification of these tumors to CIC-rearranged sarcomas and BCOR-rearranged sarcomas. This article summarizes the clinicopathologic and molecular features of these entities, with particular attention paid to those features that overlap with and distinguish these sarcomas from other round cell sarcomas.

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Keywords : Round cell sarcoma, Non-Ewing sarcoma, BCOR, CIC, Atypical Ewing sarcoma, Ewing-like sarcoma, Undifferentiated sarcoma, Sarcoma