Twenty-nine patients with Ewing's sarcoma of the lower extremity who survived for two or more years following therapy (5000 rad locally and systemic chemotherapy) were studied to assess functional status of the affected leg. Twenty-two of twenty-nine were alive and were reexamined; the deceased patients were evaluated by record review. Twenty-two of the twenty-nine had serial radiographs, which were reviewed to assess growth change induced by radiation. The living patients were divided on the basis of clinical examination into four functional groups with Group I comprising patients with minor functional limitations and leg length discrepancy 1.5 cm or less. Group II patients had moderate functional limitations with 2.5-cm leg-length discrepancy or less. Group III patients had severe functional limitations with up to 4-cm leg length discrepancy. Group IV patients had severe complications, sufficient enough to warrant amputation. Thirteen of twenty-two patients were classified as functional Group I, five as Group II, three as Group III, and one as Group IV. Radiographic changes in growing bone did not correlate with functional results. Although a femoral fracture and an age less than 16 years at diagnosis were found to be less favorable prognostic factors for the functional treatment result, these results show that neither femoral location nor young age justify primary amputation for Ewing's sarcoma of the lower leg extremity.

Since 1964, 66 consecutive patients with Ewing's sarcoma have been treated at the National Cancer Institute with local irradiation of the primary site combined with adjuvant regimens of progressively more intensive systemic chemotherapy. Actuarial survival rates for the total series show a 56% 2‐year and 35% 5‐year survival. The 43 patients without clinically detectable metastases at diagnosis have 64% 2‐year and 52% 5‐year survival rates. The current protocol, alternating high‐dose pulses of adriamycin and cyclophosphamide‐vincristine, is providing improved disease‐free survival as compared to previous protocols, and indicates further progress toward the ultimate goal of complete tumor eradication. In addition to the problems of diagnostic accuracy in evaluating treatment results, other major factors influencing prognosis include initial metastatic disease, site of the primary tumor, age at diagnosis, and presence of systemic symptoms. At least these, and probably others, must be taken into account in developing randomized prospective trials for determination of optimal adjuvant therapy.

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An autopsy study of 26 cases of Ewing's sarcoma treated with radiation to the primary site plus adjuvant chemotherapy has shown metastatic tumor in 23 cases. Metastases were found typically in lungs, pleura, bones and regional lymph nodes. In three cases no tumor could be found at autopsy, and death was due to complications of treatment. Tumor was found in the irradiated primary site in 13 of the 20 cases in which the primary site was examined at autopsy. Histologically, the tumor at autopsy frequently had increased pleomorphism and increased numbers of bizarre giant cells; however, these changes did not affect the presence of glycogen in tumor cells, thus reaffirming the importance of intracytoplasmic glycogen in the diagnosis of Ewing's sarcoma.