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Adult cerebellar glioblastomas (cGBM) are rare and their characteristics remain to be fully described. We analyzed the characteristics of 17 adult patients with cGBM and compared them to a series of 103 patients presenting a supra-tentorial glioblastoma (stGBM). The mean age at GBMc diagnosis was 53.4 years (range 28–77). A history of neurofibromatosis type I was noted in 3 patients. cGBM were hemispheric in 10 patients (58.8%), only vermian in 4 patients (23.5%), and both vermian and hemispheric in 3 patients (17.7%). A H3 K27M mutation was identified in 3/14 patients, a TERT promoter mutation in 3/14 patients and a methylated MGMT promoter in 3/14 patients. None of the patients (0/14) harbored an EGFR amplification, an IDH or a BRAF mutation. Association with neurofibromatosis type I and H3K27M mutations were mutually exclusive. Compared with stGBM, cGBM occurred in younger patients (53.4 vs. 63.2, p = 0.02), were more frequently associated with neurofibromatosis type I (18 vs. 1%, p = 0.009) and with a H3 K27M mutation (21 vs. 3%, p = 0.02). They also tended to have a more frequent multifocal presentation at diagnosis (21 vs. 4.3%, p = 0.06), more frequently resulted in leptomeningeal or intra-axial metastasis (44.5 vs. 5%, p = 0.002) and were associated with a shorter median overall survival (5.9 vs. 14.2 months, p = 0.004). The present study suggests that adult cGBM differ from their supra-tentorial counterpart and constitute a heterogeneous group of IDH wild-type gliomas with at least two subgroups, one associated with H3K27M mutations and the other with neurofibromatosis type I. [ABSTRACT FROM AUTHOR]

Primary cerebellar glioblastoma (CGB) comprises only 0.4-3.4% of all intracranial glioblastoma. The impact of surgical resection on survival and the efficacy of adjuvant therapies are uncertain as CGB is underrepresented in most studies. To elucidate prognostic factors we performed a single-institutional review of the largest series to date of CGB. The University of Texas MD Anderson Cancer Center database was reviewed from 1990 to 2010. Twenty-one consecutive patients met criteria for inclusion. The Kaplan-Meier product limit method was used to estimate overall survival (OS) and progression-free survival (PFS); groups were compared using the log-rank statistic. The multivariate Cox proportional hazards models were fitted to examine the association of resection with OS and PFS adjusted for other clinical variables. The median age was 39.9 years, and Karnofsky performance status (KPS) was ≥80 in 61.5% of patients. The mean extent of resection for contrast enhancement (EOR-CE) was 93.8% (SD = 10.4%; median = 100%), and the median follow-up was 18.4 months (range 1.5-116.1 months). There was no significant association of EOR with OS or PFS. On univariate analysis the presence of leptomeningeal disease (LMD) was associated with a worse OS (6.1 vs. 24.1 months; P = 0.0001) and PFS (3.3 vs. 9 months; P = 0.019). Patients who had adjuvant chemotherapy (CT) had extended PFS (10.1 vs. 2.8 months; P < 0.0001). Adjustment for the presence of leptomeningeal disease (LMD) tended toward an increased risk of progression (HR = 3.46; 95% confidence interval [CI], 0.83-14.5; P = 0.09) and was associated with a significantly increased risk of death (HR = 15.2; 95% CI, 1.3-180; P = 0.03). Having received adjuvant chemotherapy was associated with a decreased risk of progression (HR = 0.02; 95% CI, 0-0.26; P = 0.003). The presence of LMD is a critical factor in the clinical behavior of CGB resulting in markedly decreased OS and PFS. Adjuvant CT resulted in increased PFS but did not significantly affect OS. This was due to a lack of a sizable cohort who did not receive chemotherapy. Furthermore, three of the CT-naïve patients received CT at first progression. In the context of the high EOR in this study, an OS of 18.4 months was achieved. [ABSTRACT FROM AUTHOR]

A 70-year-old woman is reported who had glioblastoma multiforme of the cerebellum 52 years after radiation therapy to a midline cerebellar tumor. Seven similar reported cases are reviewed. Dedifferentiation of astrocytoma to glioblastoma and the role of radiation therapy are discussed. [ABSTRACT FROM AUTHOR]

Cerebellar glioblastoma multiforme in childhood is extremely rare. Only ten such cases have been reported to date. Although treatment results are reported to be extremely poor, complete tumor clearance was achieved in a patient treated with superfractionated radiotherapy. She received high dose postoperative radiotherapy using a three times per day technique and is disease-free for more than three years posttherapy. This suggests that improved tumor response can be obtained by high dose postoperative radiotherapy using a modified fractionation technique. [ABSTRACT FROM AUTHOR]