Page 113 - TD-4-2

Tumor Discovery

CASE REPORT
Desmoplastic small round cell tumor: A

case report

1
1
Masaru Kanasugi * , Tsuyoshi Honda , and Shigeyuki Asano 2
1 Department of Obstetrics and Gynecology, Iwaki City Medical Center, Iwaki, Fukushima, Japan
2 Department of Pathology, Iwaki City Medical Center, Iwaki, Fukushima, Japan

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.
*Corresponding author:
Masaru Kanasugi
(kanasugi@outlook.com)
Keywords: Case report; Desmoplastic small round cell tumor; Ovarian cancer;
Citation: Kanasugi M, Honda T, Chemotherapy; Multikinase inhibitor; Immune checkpoint inhibitor
Asano S. Desmoplastic small round
cell tumor: A case report. Tumor
Discov. 2025;4(2):105-111.
doi: 10.36922/td.7104
Received: December 7, 2024 1. Introduction
Revised: February 2, 2025 Desmoplastic small round cell tumor (DSRCT) is an extremely rare malignant tumor.
DSRCT is more prevalent in men than in women, with a male-to-female ratio of 4:1.
Accepted: February 18, 2025
DSRCT rarely occurs in the ovaries; it mainly occurs in adolescents and young adults,
Published online: March 4, 2025 and tends to cause peritoneal dissemination, lymph-node metastasis, and distant
Copyright: © 2025 Author(s). metastasis to the liver and lungs. A molecular biological feature of DSRCT is the
This is an Open-Access article
distributed under the terms of the reciprocal translocation of the EWS-WT1 fusion gene. Treatment for DSRCT includes
Creative Commons Attribution multidisciplinary treatments such as surgery, chemotherapy, and radiation therapy,
License, permitting distribution, and may include radiofrequency ablation, gamma knife, cryoablation, and vascular
and reproduction in any medium,
provided the original work is embolization in cases of recurrence. In the present case, despite complete resection,
properly cited. lymph node and distant organ metastases occurred early. Recently, molecular-targeted
Publisher’s Note: AccScience drug treatment has also been expected, and there have been reports of long-term growth
Publishing remains neutral with inhibitory effects of the multikinase inhibitor sunitinib. However, in the case reported
1
regard to jurisdictional claims in
published maps and institutional herein, no response was obtained despite treatment with a protocol that included
affiliations. lenvatinib.

Volume 4 Issue 2 (2025) 105 doi: 10.36922/td.7104