SURVIVAL AND LATE EFFECTS IN MEDULLOBLASTOMA PATIENTS TREATED WITH CRANIOSPINAL IRRADIATION UNDER 3 YEARS OLD

Conventional treatment of medulloblastoma has involved surgery to the primary tumour and radiotherapy to the primary site and craniospinal a xis. However CNS irradiation in a young child may result in significan t side effects. Thus new treatment strategies have emerged which inclu de chemotherapy, given in order to delay radiotherapy, to enable radia tion dose reduction to the primary site and craniospinal axis, or even to eliminate radiotherapy completely. Such treatments have not yet be en adequately evaluated in terms of survival and late effects. We repo rt a retrospective study of 37 patients under the age of 36 months tre ated with postoperative craniospinal irradiation, in which the radiati on dose to the neuroaxis was below conventional dosage. The overall ac tuarial 10-year survival rate was 44% and the actuarial 10-year relaps e free survival rate was 54%. Both radiotherapy and chemotherapy contr ibuted to morbidity and mortality. Four of 16 patients who survived lo nger than 10 years had no hard neurological signs; all but one patient have required extra support at school. Of nine patients available for work, two have obtained employment but only one has maintained this. No young adults have married. Despite lower doses of radiation, all bu t 1 survivor has significant spine shortening, and ail who reached fin al height were short. Further work is needed to complete the profile o f late effects in this group, which should include the survivors own p erceptions of quality of life. It is hoped that multimodality treatmen t and supportive care can sustain acceptable survival rates but reduce the burden of late effects. (C) 1997 Wiley-Liss, Inc.