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CIC-Rearranged Sarcoma by Naohiro Makise; Akihiko Yoshida is a Medicine article available to read on EtoBox.

What is CIC-Rearranged Sarcoma about?

## C IC-rearranged sarcoma is a rare type of small round cell sarcoma. The tumors often affect the deep soft tissues of patients in a wide age range. They are highly aggressive, respond poorly to chemotherapy, and have a worse outcome than Ewing sarcoma. CIC-rearranged sarcoma has characteristic and recognizable histology, including lobulated growth, focal myxoid changes, round to epithelioid cells, and minimal variation of nuclear size and shape. Nuclear ETV4 and WT1 expression are useful immunohistochemical findings. CIC fusion can be demonstrated using various methods; however, even next-generation sequencing suffers from imperfect sensitivity, especially for CIC::DUX4. ## Key points CIC-rearranged sarcoma is a distinct entity newly recognized by the fifth edition of the WHO classification. Histologic findings have been well characterized and are recognizable. Nuclear ETV4 and WT1 positivity are useful immunohistochemical findings. Molecular testing may fail to detect CIC fusion genes. CIC-rearranged sarcoma has a worse prognosis than Ewing sarcoma.

Who reads CIC-Rearranged Sarcoma?

Author

Naohiro Makise; Akihiko Yoshida

Publisher

Elsevier BV

Published

2024

Language

EN

Field

Medicine (Health Sciences)