Parameningeal rhabdomyosarcoma in the young.

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 and 16 were also treated with adjuvant chemotherapy. The overall five year 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%) and the 30% five year survival rate for this group was no different than that for patients without initial meningeal involvement (41%). At first or second relapse, meningeal involvement was present in six of 37 patients and in two patients this occurred as an isolated event with distant meningeal seeding; in four 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%). It is considered that primary tumor radiation treatment parameters may be critical in determining the incidence of subsequent meningeal relapse.