Primary chemotherapy and tumor resection in Ewing's sarcoma of the ribs. Report of the French society of paediatric oncology

Publisher Website
Google Scholar
Abstract
Fifteen patients with primary Ewing's sarcoma of the ribs were entered into the same protocol: thirteen of them had localized disease while two had metastatic disease. The protocol consisted of (1) initial chemotherapy according to size of tumor: patients with a small tumor were given rwo courses of VAC (vincristine, actinomycine D. cyclophosphamide); patients with a large tumor, pleuraj effusion or metastatic disease were given alternating courses of VAC and VAd (vincristine. adriamycine) until maximum regression of the tumor: (2) local radiotherapy: (3) maintenance chemotherapy with VAC/VAd: (4) complete surgical excision at diagnosis in 4 patients, after primary chemotherapy in 6 patients, after chemotherapy and radiotherapy in 2 patients. Six of the 9 evaluable patients had tumor regression higher than 50% after primary chemotherapy. Fourteen patients achieved complete remission. Six patients with localized disease remained disease-free for a median duration of 50 months. These 6 patients were treated by chemotherapy, complete surgical excision and radiotherapy. The results suggest that aggressive treatment with chemotherapy and surgery improve disease-free survival in patients with Ewing's sarcoma of the ribs. Chemotherapy, Ewing's Sarcoma, Rib, Surgery