[The Cooperative Ewing Sarcoma Study CESS 81 of the German Pediatric Oncology Society--analysis after 4 years]

In 1981 the cooperative Ewing's sarcoma study CESS 81 was initiated with initial 18-weeks-chemotherapy consisting of vincristine, actinomycin D, cyclophosphamide and adriamycin (VACA) followed by local therapy consisting of either radical surgery with complete resection of the involved bone or incomplete resection followed by radiation with 36 gy or radiotherapy only. Patients with radiation only for local therapy and extremity tumor sites are randomised for 46 gy vs 60 gy tumor dose. Following local therapy chemotherapy is continued for an additional 18 weeks. The actuarial results of 83 consecutive patients entered from 51 participating institutions from January 1, 1981, until November 15, 1984 are presented. 68/83 patients were off therapy and under observation for longer than one year following diagnosis. The longest follow-up was 41 months. On November 15, 1984, 39/68 (57%) patients were disease free. According to the site of the primary tumor patients with distal extremity lesions had a more favourable prognosis as compared to proximal extremity and central lesions. According to local therapy patients with radical surgery had a better prognosis as compared to those with resection followed by radiation and those with radiation only for local control. Analysis according to tumor volume revealed the strong interaction between tumor site, local therapy and tumor volume. According to life-table-analysis the disease free survival for patients with a tumor volume less than 100 ml was 75% after 41 months compared to 10% for patients with a tumor volume greater than or equal to 100 ml. The consequences of this analysis for a stratified treatment regimen for patients with primary Ewing's sarcoma of bone are discussed.