Orbital rhabdomyosarcoma: a twenty-year survey in Turkey.

Orbital rhabdomyosarcoma is the most frequently encountered primary malignant orbital tumor in children. Between 1970 and 1991, 68 primary orbital rhabdomyosarcoma cases were diagnosed and treated in our clinic. Mean age at the time of diagnosis was 8.8 years. Seventy-two percent of the cases were of the embryonal type while 14% had alveolar and 14% had botryoid tumors. Following tissue diagnosis by anterior orbitotomy, radiotherapy and multimodal chemotherapy were instituted promptly. Subtotal exenteration was used in massive orbital involvement and total exenteration in recurrences. Using the Kaplan-Meier method of analysis, the survival rate was 84% at three years and 80% at five years. While morbidity due to the disease itself and treatment complications may still be a problem, the survival rates achieved in the long term are promising.