Purpose of the study: We wanted to assess long-term outcome after treatment for Ewing tumor of the pelvis.

Material and method: We reviewed 62 patients aged 5 to 28 years treated from 1983 through 1993. There were 35 males and 27 males. Sixteen patients had pulmonary metastases at diagnosis. Patients were given chemotherapy using three protocols (Ew 84, Ew 88, Ew 93) proposed by the French Society of Pediatric Oncology. Fourteen patients were give high-dose chemotherapy with a bone marrow graft. The local treatment was not randomized. Radiotherapy was used alone in 25 patients and 15 underwent surgery and radiotherapy. Eighteen underwent surgery without complementary radiotherapy. For patients were not given local treatment. Outcome at last follow-up was assessed retrospectively.

Results: Mean follow-up was 6.6 years (3 months to 18 years); 29 patients were in remission, 6 had progressive disease, and 27 had died. Two patients who had bone marrow grafts developed a second tumor in the radiated territory. The overall chances of survival were 55±6% at five years and 53±7% at ten years. There was no significant difference by type of chemotherapy. In the group of operated patients, the five year survival was 68% versus 43% in the group of non-operated patients (p=0.007). In patients with initial metastases, chances of survival at ten years were 19.7±10% versus 65.9±7% in patients without metastasis. Only two patients who presented metastases initially were in remission at last follow-up. Five patients developed local recurrence after surgery and none had been radiated despite incomplete response to chemotherapy or presence of contaminated resections.

Discussion: Rigorous comparison between operated and non-operated patients is hindered due to the different indications. Results of treatment of Ewing tumors of the pelvis without metastasis are comparable to those obtained for tumors in other localizations. The fact that a second tumor can develop in the radiated territory is a particularly important factor in patients given high-dose chemotherapy with a bone marrow graft.

Conclusion: Surgical treatment appears to improve local control of Ewing tumors of the pelvis. If initial metastasis is not present, the prognosis appears to be similar to other localizations. Radiotherapy remains and indispensable adjuvant in the event of surgical resection or incomplete response to chemotherapy.