Sarcomas with CIC-rearrangements are a distinct pathologic entity with aggressive outcome: a clinicopathologic and molecular study of 115 cases

CR Antonescu, AA Owosho, L Zhang… - The American journal …, 2017 - journals.lww.com
CIC-DUX4 gene fusion, resulting from either at (4; 19) or t (10; 19) translocation, is the most
common genetic abnormality detected in EWSR1-negative small blue round cell tumors.
Following their discovery it was debated if these tumors should be classified as variants of
Ewing sarcoma (ie, atypical Ewing sarcoma) or as a stand-alone pathologic entity. As such
the WHO classification temporarily grouped the CIC-rearranged tumors under
undifferentiated sarcomas with round cell phenotype, until further clinical evidence was …