DESMOPLASTIC SMALL ROUND-CELL TUMORS - PROGNOSTIC INDICATORS AND RESULTS OF SURGICAL-MANAGEMENT

Background: Desmoplastic small round cell tumors (DSRCT or DSCT) are r are aggressive cancers of adolescence and early adulthood. There are f ew reported series to guide clinical therapy. This study correlates su rvival with treatment variables, including aggressive surgical debulki ng. Methods: Thirty-two patients with documented DSRCT received treatm ent at our institution. Demographic, clinical, and treatment variables were correlated with progression-free survival using log-rank statist ics. Results: Thirty patients were male (96%), and two were female (4% ), with a median age at diagnosis of 22 years. The primary site of dis ease in 97% of cases was the abdomen or pelvis. Twenty-nine patients ( 91%) had extensive disease involving peritoneal surfaces, lymph nodes, or discontinuous organs. All 32 patients received systemic chemothera py. Fifteen (47%) underwent tumor debulking greater than 90% at diagno sis or during therapy. A complete or very good response to therapy occ urred in 13 patients, and depended on surgical removal of bulk disease in all. Thirteen patients remained progression-free, but three of the se patients died from treatment toxicity. Improved survival was correl ated with a complete or very good partial response to multimodality th erapy, surgical debulking of more than 90% either before or after chem otherapy, and use of the P6 protocol. Conclusions: DSRCT is an aggress ive cancer that occurs predominantly in young males. Improved survival is correlated with intense chemotherapy and aggressive resection.