Desmoplastic Small-Round-Cell Tumor: Case Report

Abstract

Introduction: The desmoplastic small round cell tumor is a rare neoplasm that starts and spreads through the peritoneal surface. It was first described in 1989 and in 1991 was recognized as a distinct clinical and pathological entity. Case report: A 34-year-old man presented with abdominal pain and weight loss, progressing to an intestinal obstruction after two months. Laparotomy showed an unresectable abdominopelvic mass. Anatomopathological an immunohistochemistry analysis showed a desmoplastic small-round-cell tumor. Computerized Tomography showed bilateral pleural effusion, peritoneal implants, along with masses in the abdominal and pelvic region. Chemotherapy with carbo/taxol was administered at intervals of 21-days. Later, the chemotherapy was changed to VAC/IE at a 21-day interval, with a partial response, but it was still an unresectable tumor. There was a worsening in patient performance, and he died of an abdominal obstruction on the 15º month of follow-up. Conclusion: Due to its rarity, the desmoplastic small-round-cell tumor, is still a diagnostic and treatment challenge.