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CASE REPORT
Desmoplastic small round cell tumor: A case report
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1
Department of Obstetrics and Gynecology, Iwaki City Medical Center, Iwaki, Fukushima,
Japan
2
Department of Pathology, Iwaki City Medical Center, Iwaki, Fukushima,
Japan
Tumor Discovery, 7104 https://doi.org/10.36922/td.7104
Submitted: 7 December 2024 | Revised: 2 February 2025 | Accepted: 18 February 2025 | Published: 4 March 2025
© 2025 by the Author(s). This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution 4.0 International License ( https://creativecommons.org/licenses/by/4.0/ )
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. I n 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
Funding
None.
Conflict of interest
The authors declare no conflicts of interest.
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