Ewing sarcoma and the new emerging Ewing-like sarcomas: (CIC and BCOR-rearranged-sarcomas). A systematic review.

Abstract:

:Ewing-like sarcomas (ELS) are a heterogenous group of tumors that frequently affect pediatric and young adult patients. Accurate classification and distinction from the Ewing sarcoma family of tumor (ESFT) is decisive in patient management. ELS share a significant morphologic, immunohistochemical and clinical overlap with ESFT, thus the differential diagnosis is challenging, especially with atypical ESFT and tumors with unusual immunoprofiles or uncommon clinicoradiological findings. A subset of ELS harboring the CIC-DUX4 or BCOR-CCNB3 fusions has been described recently. The spectrum of ELS is now expanding, and additional gene fusion partners besides DUX4 or CCNB3 have been detected, and the terms CIC or BCOR-rearranged sarcomas have recently been proposed. We review the clinical, histological, phenotypic and molecular findings of ESFT and these new emerging ELS.

journal_name

Histol Histopathol

authors

Machado I,Navarro S,Llombart-Bosch A

doi

10.14670/HH-11-792

subject

Has Abstract

pub_date

2016-11-01 00:00:00

pages

1169-81

issue

11

eissn

0213-3911

issn

1699-5848

pii

HH-11-792

journal_volume

31

pub_type

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