Parameningeal rhabdomyosarcoma in the young

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Abstract
Forty cases of young patients with embryonal rhabdomyosarcoma or undifferentiated sarcoma in parameningeal sites of the head and neck were reviewed. All 40 were treated with radiation therapy in conjunction with surgery; 16 were also treated with adjuvant chemotherapy [actinomycin D, vincristine, cyclophosphamide, adriamycin]. The overall 5 yr survival rate was 35%. A primary tumor dose of at least 5000 rad resulted in a significantly greater survival rate compared with lesser doses. Meningeal involvement at diagnosis was present in 20 patients (50%); the 30% 5 yr survival rate for this group was no different than that for patients without initial meningeal involvement (41%). At 1st or 2nd relapse, meningeal involvement was present in 6 of 37 patients; in 2 patients this occurred as an isolated event with distant meningeal seeding. In 4 other patients, meningeal involvement was a manifestation of local recurrence. As of the time of the last follow-up examination, control of the primary tumor had been achieved in 22 of the 40 (55%). Primary tumor radiation treatment parameters may be critical in determining the incidence of subsequent meningeal relapse.