Successful dose-intensive treatment of desmoplastic small round cell tumorin three children

Desmoplastic small round cell tumor (DSRCT) is a rare soft tissue tumor of primitive origin occurring primarily in children and young adults. Based on published reports in the literature, the response to conventional chemothe rapy is poor. We report three pediatric patients successfully treated with dose-intensive, multimodal therapy. Between August 1994 and March 1998, we evaluated three consecutive patients with DSRCT at Children's Hospital and Regional Medical Center, Seattle, Washington. We established the diagnosis based on clinical presentation, radiologic staging, and pathologic review w ith immunohistochemical staining. All patients received a combined modality protocol including dose-intensive chemotherapy (two of them with periphera l blood stem cell [PBSC] support), second look surgery, and consolidative l ocal irradiation. The patients remain in continuous remission at 66, 42, an d 26 months after diagnosis, respectively. Two of our patients were younger than any previously reported patient, extending the age group for which DS RCT should be considered on diagnosis of small round cell tumors. The unifo rm survival achieved in our series indicates potential benefit for the comb ination of dose-intensive multiagent chemotherapy, local irradiation, and a ggressive surgical approach in this disease.