Embryonal rhabdomyosarcoma of the limbs and limb-girdles. A clinicopathologic study of 61 cases.

Abstract

Of 235 embryonal rhabdomyosarcomas, 67 affected the limbs and limb-girdles. Of 61 patients studied in detail, 21 had tumors with an alveolar pattern, and 40 had tumors with a solid pattern. Forty-two tumors involved the lower extremities and pelvic girdle, 19 the upper extremities and shoulder girdle. Forty-six patients died of their tumors. Thirteen of 27 exhibited generalized metastasis and 14 exhibited evidence of pulmonary lesions. None of 16 who had regional lymph node metastasis at the time of initial treatment survived more than 24 months. Nine of the 61 patients are known to be alive and free of disease from 3 1/2 to 13 years after treatment, but only 1 patient with a tumor of alveolar pattern has lived more than 5 years. Aggressive surgical ablation of the tumor, accompanied by simultaneous intravenous chemotherapy, offers the best chance for a cure. In selected cases, supplemental aggressive radiation therapy may be of value.