Desmoplastic small round cell tumor: A case report

Abstract

Desmoplastic small round cell tumors are extremely rare, occurring primarily in young males. This disease is characterized by the reciprocal translocation of the EWS-WT1 fusion gene. Treatment for this tumor is multidisciplinary, including surgery, chemotherapy, and radiation therapy. The disease progresses rapidly and even with complete resection, local recurrence, and distant metastasis is likely to occur and the prognosis is poor. This report describes a case of primary ovarian cancer in a 33-year-old woman. The pre-operative test showed elevated CA125, CA19-9 and carcinoembryonic antigen, and hypercalcemia. Staging laparotomy was performed without residual tumor. The clinical stage was IIB, and adjuvant chemotherapy was performed. Immediately after the end of chemotherapy, multiple lymph node metastases, and the patient subsequently experienced repeated recurrences and died 10 months after surgery. In the future, it is desirable to establish standard treatment for desmoplastic small round cell tumors by analyzing more cases.

Keywords

Case report / Desmoplastic small round cell tumor / Ovarian cancer / Chemotherapy / Multikinase inhibitor / Immune checkpoint inhibitor

Acknowledgments

We would like to thank Editage (www.editage.com) for the English language editing.

Funding

None.

Conflict of interest

The authors declare no conflicts of interest.

Author contributions

Conceptualization: Masaru Kanasugi

Investigation: Masaru Kanasugi

Writing -original draft: Masaru Kanasugi

Writing -review & editing: Tsuyoshi Honda, Shigeyuki Asano

Ethics approval and consent to participate

The patient and her family were given an oral explanation and written consent was obtained.

Consent for publication

The patient and her family gave consent to publish their data.

Availability of data

Not applicable.

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