title: Clinical characteristics and pathogenesis of cerebellar glioblastoma.
abstract: Cerebellar glioblastomas (GBMs) are rare, with neither their pathogenesis nor prognosis being completely understood. The present study aimed to clarify the clinical characteristics of cerebellar GBMs by comparison with supratentorial GBMs, focusing particularly on the pathogenesis. The clinical factors between cerebellar (n=10) and supratentorial (n=216) GBMs were compared. Additionally, p53 and epidermal growth factor receptor (EGFR) levels were investigated in six patients by immunostaining as well as the isocitrate dehydrogenase 1 (IDH1) status of five patients by direct sequencing. Eight males and two females participated in the present study, the mean age at diagnosis was 56.6 years and the range 37-75 years. Four patients presented with hydrocephalus and one with brainstem involvement, and two patients were diagnosed with neurofibromatosis type 1. Two patients had previously received radiotherapy, eight patients received postoperative radiotherapy and seven chemotherapy. The mean Karnofsky performance status (KPS) score was lower in patients with cerebellar GBMs compared to those with supratentorial GBM; however, the survival times did not differ between the two groups. All of the cases of six cerebellar GBMs were p53‑positive and EGFR‑negative, as detected by immunostaining, consistent with secondary GBM. However, no IDH1 mutations were detected in any of the five cases of cerebellar GBMs analyzed, indicating that these tumors were not of the secondary type. The KPS score with cerebellar GBMs may be lower due to hydrocephalus, which was ameliorated by surgery but may have impacted the survival rate. It was confirmed that cerebellar GBMs were identical to supratentorial GBMs with respect to its clinical features, with the possible exception of the KPS score. The present study's genetic analyses indicated that cerebellar GBMs may develop via a pathway different from that of either primary or secondary GBM.
authors.affiliation: Department of Neurosurgery, Graduate School of Medical Science, Kumamoto University, Kumamoto 860-8556, Japan.
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Takahashi
authors.affiliation: Department of Neurosurgery, Graduate School of Medical Science, Kumamoto University, Kumamoto 860-8556, Japan.
authors.forename: Keishi
authors.initials: K
authors.lastname: Makino
authors.affiliation: Department of Neurosurgery, Graduate School of Medical Science, Kumamoto University, Kumamoto 860-8556, Japan.
authors.forename: Hideo
authors.initials: H
authors.lastname: Nakamura
authors.affiliation: Department of Neurosurgery, Graduate School of Medical Science, Kumamoto University, Kumamoto 860-8556, Japan.
authors.forename: Takuichiro
authors.initials: T
authors.lastname: Hide
authors.affiliation: Department of Neurosurgery, Graduate School of Medical Science, Kumamoto University, Kumamoto 860-8556, Japan.
authors.forename: Shigetoshi
authors.initials: S
authors.lastname: Yano
authors.affiliation: Department of Neurosurgery, Hokuto Hospital, Obihiro, Hokkaido 080-0039, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Kamada
authors.affiliation: Department of Neurosurgery, Graduate School of Medical Science, Kumamoto University, Kumamoto 860-8556, Japan.
authors.forename: Jun-Ichi
authors.initials: J
authors.lastname: Kuratsu
mesh_terms: D063646:Carcinogenesis; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D004252:DNA Mutational Analysis; D005260:Female; D005909:Glioblastoma / Q000235:genetics / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007521:Isocitrate Dehydrogenase / Q000235:genetics; D017567:Karnofsky Performance Status; D008297:Male; D008875:Middle Aged; D009154:Mutation; D016019:Survival Analysis
chemical_list: D007521:Isocitrate Dehydrogenase; C543588:IDH1 protein, human