Treatment of high-risk sarcomas in children and young adults: analysis of local control using intensive combined modality therapy.

Although combination chemotherapy and local irradiation are quite effective treatment for some children and young adults with small round cell sarcomas, high-risk patient groups, including patients with localized disease of the trunk and proximal extremity and those who present with metastases, continue to fare poorly with standard combined modality therapy. In an attempt to improve the local and systemic response of these tumors, an intensive treatment protocol was designed that integrates five cycles of chemotherapy with vincristine, doxorubicin, and cyclophosphamide (VADRIAC) plus radiation therapy to the primary tumor (55-60 Gy), bone, and soft tissue metastases (45-50 Gy). Patients achieving complete response to this induction therapy receive intensification treatment with total body irradiation (8.0 Gy), a cycle of VADRIAC, and autologous bone marrow transplantation. All treatment is completed within 6-7 months. From January 1983 to February 1986, 76 consecutive, previously untreated patients were entered in this study; 75 patients are evaluable. Twenty-five patients were diagnosed with rhabdomyosarcoma, 23 with Ewing's sarcoma, 15 with primitive neuroepithelioma, 12 with primitive sarcoma, and 1 patient with metastatic neuroblastoma. Forty-three patients (57%) had metastases at presentation. Overall, 68 of 75 patients (91%) achieved complete response. Fifty-eight of 61 patients with measurable soft tissue masses at the primary site had greater than or equal to 50% tumor reduction with two cycles of chemotherapy prior to local irradiation. Seven patients failed to have complete response at the primary site following five cycles of chemotherapy and local external beam irradiation, although 3 were subsequently rendered locally disease free by intraoperative radiotherapy (2 patients) or surgery (1 patient).(ABSTRACT TRUNCATED AT 250 WORDS)