TREATMENT RESULTS IN EWINGS-SARCOMA WITH PULMONARY METASTASES AT DIAGNOSIS - A RETROSPECTIVE ANALYSIS OF 41 PATIENTS

41 patients presenting with primary metastatic Ewing's sarcoma or mali gnant peripheral neuroectodermal tumor (PNET) with initial metastases restricted to the lungs and/or pleural space were analysed with respec t to clinical manifestation and treatment results retrospectively. All patients were treated according to the protocols CESS 81 and CESS 86 of the German Society of Pediatric Oncology and Hematology (GPOH). The time since diagnosis ranges from 19 to 137 months, with a median of 7 2 months. Median relapse-free survival time was 21.8 months. 18 patien ts were female, 23 were male. The majority of primary tumors exceeded 100 ml of volume. Preferred sites were the pelvis with 16 cases, the l imbs with 14 cases and the chest wall with 6 cases. The histological s pecification of the tumor was Ewing's sarcoma in 22 and PNET in 11 pat ients, in 8 cases no specific distinction was given. As to local thera py of the primary tumor, 12 patients underwent radiotherapy, 11 surger y, and 18 a combination of both. Patients were allocated to one of the se three options on an individual basis. Cytostatic drug treatment was given according to the GPOH - CESS 81 and CESS 86 protocols. As calcu lated by means of the Kaplan-Meier analysis, relapse-free survival was 30% ten years after diagnosis. Surgery or pulmonary irradiation of 12 -20 Gy was applied to lung metastases. 12 of 27 patients are in contin uous complete remission following this therapeutic approach.