Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center and Geisel School of Medicine at Dartmouth, One Medical Center Drive, New Hampshire, Lebanon.
Division of Dermatopathology, The Ohio State University Wexner Medical Center, Columbus, Ohio.
J Cutan Pathol. 2020 Jun;47(6):535-540. doi: 10.1111/cup.13639. Epub 2020 Jan 10.
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.
在缺乏 EWSR1 基因重排的圆形细胞形态肉瘤中,涉及 CIC 基因的重排最为常见。与尤因肉瘤相比,CIC 重排肉瘤的发病年龄更大,几乎只发生在软组织中,临床侵袭性更强,并且对用于治疗尤因肉瘤的化疗方案更具耐药性。CIC 重排肉瘤更常见于深部位置,我们怀疑浅表表现可能被低估了。在本病例系列中,我们报告了其中的 3 例。总的来说,形态与深部位置的 CIC 重排肉瘤相似。我们希望通过扩大具有圆形细胞形态的浅表肿瘤的鉴别诊断,提高皮肤病理学界的认识。