Desmoplastic small round cell tumor: Extra abdominal and abdominal presentations and the results of treatment

Abstract

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare malignant neoplasm of adolescent males.Current multimodality treatment prolongs life and rarely achieves cure. AIM: To review the presenting features, histopathology and outcome of 18 patients with DSRCT treated at a single institution. SETTING AND DESIGN:This is a retrospective observational study of patients with DSRCT who presented at the Tata Memorial Hospital between January 1994 to January 2005. MATERIALS AND METHODS: Eighteen patients of DSRCT seen during this period were evaluated for their clinical presentation, response to chemotherapy and other multimodality treatment and overall survival. The cohort of 18 patients included 11 males (61%) and 7 females (39%) with a mean age of 16 years (Range 1½ -30 years). Majority (83%) presented with abdomino-pelvic disease. The others, involving chest wall and extremities. There were 6 patients (33%) with metastatic disease at presentation. RESULTS: The treatment primarily included a multimodality approach using a combination of multiagent chemotherapy with adjuvant surgery and radiotherapy as applicable. A response rate of 39% (CR-1, PR-6), with chemotherapy was observed. The overall response rate after multimodality treatment was 39% (CR-5, PR-2). The overall survival was poor except in patients who had complete excision of the tumor. CONCLUSION: Abdomino-pelvic site was the commonest presentation, the disease can occur at other non-serosal surfaces also. Despite aggressive treatment the outcome was poor. However, complete surgical excision seems to provide a better survival.

Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options

“…However, the impact of cytoreductive surgery on survival remains unclear. Some authors suggest an improvement in survival with surgery with a median survival of 34 months compared to 14 months for operable and inoperable tumors, respectively [1]. Similarly, Lal in its cohort has reported a significant impact of surgery on overall survival with 3 year survival of 58% as compared to 0% in no resectable disease [15], the benefit is greater after complete resection but frequently this act is not feasible due to the extensive involvement as the case of our patient.…”

“…Very rarely, DSRCT can arise from head and neck, base of skull, paratesticular region, ovary, brain and thoracic viscera [1].…”

“…These abnormalities may be associated with ascites, lymph nodes, diffuse nodular peritoneal thickening or with distant metastasis. The lier, lung and bone marrow are common sites of metastases [1,11].…”

“…Regarding the aggressiveness of the disease, treatment is based on multimodal therapy including an extensive surgery when it is feasible, systemic chemotherapy with or without abdominal radiotherapy [12]. It has been reported that the combination of three modalities has shown best results leading to an overall response rate of 39% and a 3-year survival rate of approximately 50% [1,12] as compared to each modality used separately. However, the impact of cytoreductive surgery on survival remains unclear.…”

“…Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive sarcoma occurring mainly in children and young adults [1]. The first case was described in 1989 by Gerald and Rosai [2].…”