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Expanding the differential of superficial tumors with round‐cell morphology: Report of three cases of CIC ‐rearranged sarcoma, a potentially under‐recognized entity
Author(s) -
Maloney Nolan,
Smith Stephen M.,
Peters Sara B.,
Batistatou Anna,
Evangelou Zoi,
Harms Paul W.,
Chan May P.,
Antonescu Cristina R.,
Linos Konstantinos
Publication year - 2020
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/cup.13639
Subject(s) - dermatopathology , sarcoma , pathology , differential diagnosis , medicine , gene rearrangement , biology , gene , genetics
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.