Expanding the differential of superficial tumors with round-cell morphology: Report of three cases of CIC-rearranged sarcoma, a potentially under-recognized entity Journal Article


Authors: Maloney, N.; Smith, S. M.; Peters, S. B.; Batistatou, A.; Evangelou, Z.; Harms, P. W.; Chan, M. P.; Antonescu, C. R.; Linos, K.
Article Title: Expanding the differential of superficial tumors with round-cell morphology: Report of three cases of CIC-rearranged sarcoma, a potentially under-recognized entity
Abstract: Among sarcomas with a round-cell morphology that lack rearrangement of the EWSR1 gene, rearrangements involving the CIC gene are the most common. In comparison with Ewing Sarcoma, CIC-rearranged sarcomas present at an older average age, arise almost exclusively in soft tissues, are clinically more aggressive, and are more likely to be resistant to the chemotherapy regimens used for Ewing sarcoma. CIC-rearranged sarcomas present more commonly in a deep location, and we suspect that superficial presentations may be under-recognized. In this case series, we report three of such cases. Overall, the morphology is similar to CIC-rearranged sarcomas of deeper locations. We hope to raise awareness among the dermatopathology community by expanding the differential of superficial tumors with round cell morphology. © 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Keywords: case report; cic-dux4; cic; cic-dux; cic-rearranged; round-cell sarcoma
Journal Title: Journal of Cutaneous Pathology
Volume: 47
Issue: 6
ISSN: 0303-6987
Publisher: John Wiley & Sons  
Date Published: 2020-06-01
Start Page: 535
End Page: 540
Language: English
DOI: 10.1111/cup.13639
PUBMED: 31886887
PROVIDER: scopus
PMCID: PMC8353993
DOI/URL:
Notes: Article -- Source: Scopus
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  1. Cristina R Antonescu
    895 Antonescu