Ewing sarcoma and the new emerging Ewing-like sarcomas: (CIC and BCOR-rearranged-sarcomas). A systematic review
Isidro Machado1, Samuel Navarro2 and Antonio Llombart-Bosch2
1Pathology Department, Instituto Valenciano de Oncología and 2Pathology Department, University of Valencia, Valencia, Spain
Offprint requests to: Isidro Machado, MD, PhD. Pathology Department. Instituto Valenciano de Oncología, C/Gregorio Gea 31, Valencia, 46009, Spain. e-mail: isidro.machado@uv.es
Summary. 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 immuno-profiles 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. Histol Histopathol 31, 1169-1181 (2016)
Key words: Ewing sarcoma, Ewing-like sarcomas, CIC-rearranged sarcomas, BCOR-rearranged sarcomas
DOI: 10.14670/HH-11-792