Prognosis of orbital rhabdomyosarcoma in children in Japan.

Retrospective studies were made of patients with orbital rhabdomyosarcoma (RMS) who were registered in the Japan Children's Cancer Registry and the National Registry of Ocular Tumors in Japan during 1974-1989. Thirty-five children under 15 years of age were registered as orbital RMS patients. The median age of onset was 5 years and 2 months; lid swelling was the most frequent initial symptom and finding. Histologically, 79% were classified as the embryonal type. Orbital exenteration was the standard treatment until the mid-1970's, but all the 5 patients who had only surgical therapy experienced relapses. Since the mid-1970's, a combined regimen of surgery, radiation and chemotherapy has been used. Chemotherapy has recently been considered to play the most important role in this combined therapy. Among the 35 patients studied, three patients with only biopsy as the surgical procedure have had no relapses up to the present time. Recurrent tumors developed in 15 of the remaining 32 patients: 7 of those were local, 8 were distant. All relapses (10 of the 14 patients in the 1970's, and 5 of the 18 in the 1980's) occurred within 1 year and 11 months after initial treatment. The 3-year survival rate of these 32 patients, estimated by the Kaplan-Meier method, was 70% (52% in the 1970's, 86% in the 1980's).