Soft tissue sarcoma of the head and neck in childhood: nonorbital and nonparameningeal sites. A report of the Intergroup Rhabdomyosarcoma Study (IRS)-I.

Seventy-two patients with soft tissue sarcoma arising in the oral cavity, oropharynx, larynx, parotid region, cheek, scalp, and neck, were entered on the Intergroup Rhabdomyosarcoma Study (IRS)-I, and could be analyzed for survival. Sixty-three (88%) attained complete remission (CR), of whom 13 subsequently relapsed (78% actuarial 5-year retained CR rate). The patients with primary tumor arising in sites other than the neck had a retained CR rate exceeding 90%. In contrast, 11 relapses occurred in the 26 patients with neck primaries (54% retained CR rate). Females, and infants younger than age 24 months were more likely to relapse. Prognostic factors with little or no influence on relapse included tumor size, histology, regional lymph node status, clinical group, and treatment arm. Five of the 6 patients with failure at the primary site had either no radiotherapy (2 patients) or an insufficient dose (less than 3000 rad). No patient required major organ sacrifice such as laryngectomy or pharyngectomy. Isolated failure in regional nodes did not occur. Children with nonorbital, nonparameningeal head and neck soft tissue sarcoma treated in accordance with the IRS protocol have an excellent rate of local control and survival. Primary tumors arising in the neck are more likely to relapse locally or distantly.