Nonmetastatic pelvic Ewing sarcoma: Report of the French Society of Pediatric Oncology

Background. Since January, 1984, 59 children with histologically confirmed Ewing sarcoma of the pelvic bone have been treated with three successive ch emotherapy protocols recommended by the French Society of Pediatric Oncolog y. The purpose of the current study was to evaluate the role of surgery and /or radiotherapy in local progression-free, disease-free, and overall survi vals (LPFS, DFS, and OS, respectively). Procedure, We retrospectively exami ned 59 children treated for nonmetastatic, pelvic Ewing sarcoma over the la st 12 years. All were first treated with chemotherapy according to the curr ent French protocol. Six patients developed progressive disease before loca l treatment and were excluded for local control and survival analysis. Loca l treatment was surgery alone in 17 cases, radiation therapy in 27 cases, a nd surgery plus radiation therapy in 9 cases. Results. With a median of fol low-up of 6.5 years, no significant differences in local control or surviva l were observed with the three chemotherapeutic protocols. Of the 53 patien ts evaluable for local control, 6 relapsed locally only, 8 had local and di stant relapses, and 9 had distant metastases only. The 5-year OS rate wa wo rst for patients with radiotherapy alone compared to those with surgery or combined modality treatment (44% vs. 72%, P = 0.043). The 5-year LPFS and D FS rates were worst in the radiotherapy-alone group but not significantly ( 63% vs. 79%, P = 0.22 and 42% vs 71%, P = 0.07, respectively). The importan ce of surgery to OS and DFS was confirmed by multivariate analysis (P = 0.0 26 and P = 0.048, respectively). One surviving patient was diagnosed with i n-field fibrosarcoma, which was presumably radiation induced. Conclusions. Despite intensive, multiagent chemotherapy, survival from pelvic: Ewing sar coma has not improved over the past decade; however, the survival rate does not seem to be worse than that from Ewing sarcoma at other locations, inso far as at least 50% of the patients were cured. Surgery or a combination of surgery and radiation therapy are the best local treatment; exclusive radi ation therapy should be reserved for patients with inoperable lesions or pa rtially or nonchemosensitive tumors or when surgery would be an amputation. (C) 1999 Wiley-Liss, Inc.