Desmoplastic small round cell tumor: A case report and literature review

Desmoplastic small round cell tumors (DSRCTs) are tumors with a poor prognosis that are disseminated in the peritoneum. We report the case of a 21-year-old female who has survived disease-free for 10 years after her initial diagnosis of DSRCT. At the age of 10, the patient developed DSRCT with diffused peritoneal infiltration of the greater omentum, peritoneal metastasis centered on the pelvic peritoneum, and a large amount of cancerous ascites. Cytoreduction surgery of the primary lesion (125 g) that had metastasized to the greater omentum (1095 g) was performed. Adjuvant chemotherapy was administered followed by myeloablative mega-therapy with autologous peripheral blood stem-cell transplantation. Approximately 7 months later, a laparoscopic resection of the localized retroperitoneal recurrence (2.5×2.0 cm) was performed. Two additional cycles of postoperative chemotherapy were performed. At the age of 13, she was diagnosed with follicular adenocarcinoma of the thyroid gland and underwent a right lobectomy. Now at the age of 21, exactly 129 months after the first onset of DSRCT, she is in complete remission. There are 25 cases of DSRCT reported in Japan; however, the outcome for most cases was poor. This case has shown that careful follow-up after multidisciplinary therapy and early detection and treatment of recurrent tumors can lead to long-term tumor free survival.