Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy.

12 patients (10 previously untreated, 2 previously treated) with desmoplastic small round-cell tumor (DSRCT), median age 14 years, male-to-female ratio 11:1.

P6 protocol (7 courses of chemotherapy including cyclophosphamide, doxorubicin, vincristine, ifosfamide, etoposide), followed by attempted tumor resection, and optional radiotherapy and myeloablative thiotepa/carboplatin with stem-cell rescue.

Clinical response (complete or partial remission) and progression-free survivalhard clinical