Background: Less than 200 cases have been reported in the world literature since desmoplastic small round cell tumor (DSRCT) was first described in 1989. To date, chemotherapy, radiation therapy, and surgery have resulted in a poor survival of 30% to 55%. We used hyperthermic intraperitoneal chemotherapy (HIPEC) at the time of complete tumor resection as an adjunct to treatment of pediatric and adolescent patients with DSRCT.
Purpose: The aim of this study was to assess survival as a function of disease burden and response to HIPEC in patients with DSRCT.
Methods: Twenty-four patients with DSRCT from 1995 to 2008 were evaluated. Eight patients undergoing cytoreductive surgery and HIPEC were compared with 16 historical controls that had chemotherapy +/- radiation therapy or surgery alone.
Results: Median age was 12 years in 8 patients who underwent HIPEC. Significant morbidity after HIPEC included renal insufficiency and gastroparesis. There were no operative mortalities. The estimated median overall 3-year survival for patients not undergoing surgery or HIPEC was 26% compared with 71% in patients who underwent HIPEC. Extraabdominal metastasis correlated with poor survival (P = .021).
Conclusion: Hyperthermic intraperitoneal chemotherapy is safe in children with DSRCT. It may prolong disease-free survival in selected cases of DSRCT. It may have a limited role as an adjunct to local control in patients with DSRCT.
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