Abstract
Adult medulloblastoma is a rare tumor with few retrospective studies published so far. The role of adjuvant chemotherapy or chemotherapy at relapse is unclear. This study reports therapy and outcome in all adult (≥ 16 years old) medulloblastoma (n = 34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n = 2) treated in 2 neuro–oncological centers between 1976 and 2002. The median age was 24.5 years (range 16–76). After resection, 16 patients were treated with craniospinal radiotherapy alone, 20 patients also received adjuvant chemotherapy (8 vincristine, CCNU, cisplatin; 7 methotrexate alone or methotrexate/vincristine–based polychemotherapy; 5 other protocols). Median survival in the whole cohort was 126 months (2+ – 200+ months). Five–year and 10–year survival rates were 79 % and 56%. Adjuvant chemotherapy was associated with a non–significant trend to prolonged survival (relative risk (RR) 1.89; p = 0.068). The median progression–free survival (PFS) after primary therapy was 83 months. At relapse, 10 of 12 evaluable patients achieved a complete response upon second–line therapy. The median survival times from first (n = 17) and second relapse (n = 9) were 21 months (0–67+ months; 5/17 without second relapse) and 20 months (1–29 months). Cox regression analysis revealed the infiltration of the floor of the 4th ventricle at diagnosis as the only therapy–independent prognostic factor (RR 0.48; p = 0.03). In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients. Moreover, second–line therapy may be beneficial for these patients. As in pediatric medulloblastoma patients, primary infiltration of the floor of the 4th ventricle indicates a poor prognosis.
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This publication is dedicated to the late Joachim Kühl, MD, whose life was dedicated to improve therapy for medulloblastoma patients.
*Drs. Herrlinger and Steinbrecher contributed equally to this work.
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Herrlinger*, U., Steinbrecher*, A., Rieger, J. et al. Adult medulloblastoma. J Neurol 252, 291–299 (2005). https://doi.org/10.1007/s00415-005-0560-2
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DOI: https://doi.org/10.1007/s00415-005-0560-2