Ewing's sarcoma of the proximal femur

The cases of 16 patients with Ewing's sarcoma of the proximal femur treated in the era of multiagent chemotherapy were reviewed, with emphasis on the mechanical problem of tumor involvement in this structurally demanding site. Fourteen patients received chemotherapy and local radiotherapy as the initial primary treatment. One patient had chemotherapy and radiotherapy, followed by wide local resection. One patient had amputation, followed by chemotherapy, for pathologic fracture and extensive soft tissue involvement at presentation. Two local recurrences occurred. Excluding the 2 patients whose femurs were fixed prophylactically, the pathologic fracture rate was 79%. In addition, by excluding the 2 patients who died before fracture, the pathologic fracture rate was 92%. Nonunion occurred in 5 (71%) of the 7 pathologic fractures not treated by resection and required as many as 5 additional surgical procedures to obtain union. At latest followup evaluation (average, 6.3 years), 10 patients had no evidence of disease, 1 was alive with disease, and 5 had died of their disease. Options for management should include primary resection and reconstruction or prophylactic internal fixation after completion of chemotherapy plus or minus radiotherapy.