Desmoplastic small round cell tumor: A case report

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.