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CIC-DUX4 Rearranged Sarcoma Presenting in the Skin: Case Report.

ABSTRACT:

Introduction

Capicua transcriptional repressor (CIC)-DUX4 rearranged sarcoma is a subtype of CIC-rearranged sarcomas composed of undifferentiated Wilms' tumor 1 (WT1)⁺, CD99⁺ round cells with recurrent CIC gene rearrangement. The diagnosis of CIC-rearranged sarcoma remains challenging, and the prognosis of CIC-rearranged sarcomas is poor.

Case presentation

In this report, we described a case of CIC-DUX4 rearranged sarcoma presenting in the skin, expressing WT1 and CD99 in a dot-like pattern. In addition, the assessment of genomic alterations using genome panel testing was useful to confirm the accurate diagnosis.

Conclusion

Our present case suggests that widespread use of genomic panel testing in the future may lead to early treatment and improve the prognosis of CIC-rearranged sarcomas.

SUBMITTER: Amagai R

PROVIDER: S-EPMC11249449 | biostudies-literature | 2024 Jan-Dec

REPOSITORIES: biostudies-literature

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CIC-DUX4 Rearranged Sarcoma Presenting in the Skin: Case Report.

Amagai Ryo R Fujimura Taku T Hashimoto Akira A Yoshida Shinichirou S Asano Yoshihide Y

Case reports in oncology 20240101 1

<h4>Introduction</h4>Capicua transcriptional repressor (CIC)-DUX4 rearranged sarcoma is a subtype of CIC-rearranged sarcomas composed of undifferentiated Wilms' tumor 1 (WT1)<sup>+</sup>, CD99<sup>+</sup> round cells with recurrent CIC gene rearrangement. The diagnosis of CIC-rearranged sarcoma remains challenging, and the prognosis of CIC-rearranged sarcomas is poor.<h4>Case presentation</h4>In this report, we described a case of CIC-DUX4 rearranged sarcoma presenting in the skin, expressing WT ...[more]

PMID: 39015640

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