A pilot study of preirradiation chemotherapy and 1800 cGy craniospinal irradiation in young children with medulloblastoma

Purpose Craniospinal irradiation (CSI) is necessary in the treatment of medulloblastoma, although it results in significant long-term sequelae, particularly in young children. We prospectively evaluated the feasibility of giving preirradiation chemotherapy followed by 1800 cGy CSI to young children with localized medulloblastoma. Methods and materials Between January 1993 and July 1997, 7 consecutive patients (age, 20-64 months) with M0 medulloblastoma were enrolled. After surgical resection, patients received 4 months of multiagent chemotherapy followed by 1800 cGy CSI and 5400 cGy to the posterior fossa. Results Median follow-up is 8.9 years. No patient developed progressive disease during chemotherapy. One patient developed widespread metastatic recurrence 2 months after completing radiation therapy and died. Two additional patients developed isolated frontal horn relapses 32 and 36 months after initial diagnosis and received further irradiation and chemotherapy. Both of these patients remain alive 7.1 and 3.6 years from the time of recurrence. Four of the six survivors have endocrine deficits. All of the survivors require special assistance in school. Conclusions Craniospinal irradiation doses of 1800 cGy may not be adequate to prevent exoprimary recurrences. Despite the CSI dose reduction, neuroendocrine and neurocognitive sequelae are substantial.