Childhood rhabdomyosarcoma with meningeal extension: results of combined therapy including central nervous system prophylaxis.

Results achieved in two consecutive series of children with rhabdomyosarcoma (RMSA) of the head and neck area, presenting with meningeal involvement, are compared. The first series consisted of 12 children treated with local radiotherapy and with (mostly) polidrug systemic chemotherapy from 1969 to 1977. The second series consisted of 11 children who, besides local radiotherapy and polichemotherapy, received brain radiotherapy (30 Gy) and intrathecal (I.T.) methotrexate (MTX) as central nervous system (CNS) prophylaxis from 1978 to 1980. In the first group, 50% of patients achieved a complete remission and only one child (8%) is surviving relapse-free. In the second group, 91% of patients achieved a complete remission and seven (64%) are surviving relapse-free after a median follow-up of 32 months. The most important reason for failure in the first series was represented by the inability to achieve a persistent local control of the primary tumor. This observation and the higher doses of radiotherapy delivered to the primary tumor in the second series prevent the conclusion that the improved results are attributable entirely to the addition of CNS prophylaxis.