title: Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.
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 (>or=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 4(th) 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 4(th) ventricle indicates a poor prognosis.
authors.affiliation: Department of Neurology, Hertie Institute for Clinical Brain Research University of Tübingen Medical School, Hoppe-Seyler-Str. 3, 72076 Tübingen, Germany. ulrich.herrlinger@uni-tuebingen.de
authors.forename: Ulrich
authors.initials: U
authors.lastname: Herrlinger
authors.forename: A
authors.initials: A
authors.lastname: Steinbrecher
authors.forename: J
authors.initials: J
authors.lastname: Rieger
authors.forename: P
authors.initials: P
authors.lastname: Hau
authors.forename: R-D
authors.initials: RD
authors.lastname: Kortmann
authors.forename: R
authors.initials: R
authors.lastname: Meyermann
authors.forename: M
authors.initials: M
authors.lastname: Schabet
authors.forename: M
authors.initials: M
authors.lastname: Bamberg
authors.forename: J
authors.initials: J
authors.lastname: Dichgans
authors.forename: U
authors.initials: U
authors.lastname: Bogdahn
authors.forename: M
authors.initials: M
authors.lastname: Weller
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000704:Analysis of Variance; D002528:Cerebellar Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy*; D003131:Combined Modality Therapy; D003710:Demography; D018450:Disease Progression; D018572:Disease-Free Survival; D004307:Dose-Response Relationship, Radiation; D004358:Drug Therapy / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology / Q000628:therapy*; D008875:Middle Aged; D011882:Radiotherapy, High-Energy / Q000379:methods; D012008:Recurrence; D012044:Regression Analysis; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome