Clinicopathologic Features and Clinical Outcomes of Desmoplastic Small Round Cell Tumors in Abdomen

ABSTRACT

Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor that occurs mainly in the abdominal cavity and has a tendency to occur in young males. But it occurs in other primary sites including the central nerve system, lung, kidney and soft tissue like bone. Major symptoms include abdominal palpable mass, abdominal pain and distention. Useful studies for diagnosis are radiographic study including computerized tomogram and percutaneous pathologic study. DSRCT is an aggressive malignant neoplasm and its prognosis is poor. The overall survival at three years is about 30%. This report describes 5 cases of DSRCT since 2002. The median age of patients was 35 years. All patients are men that presented abdominal pain and abdominal mass. 3 patients underwent surgical resection of tumor and 4 patients received chemotherapy. The survival time of 2 expired patients was 39 and 28 months, individually. And the follow-up period of survival patients is 23~31 months.