Characteristics of cerebellar glioblastomas in adults

• Published in: Journal of neuro-oncology 2018-02, Vol.136 (3), p.555-563
• Main Authors: Picart, Thiébaud, Barritault, Marc, Berthillier, Julien, Meyronet, David, Vasiljevic, Alexandre, Frappaz, Didier, Honnorat, Jérôme, Jouanneau, Emmanuel, Poncet, Delphine, Ducray, François, Guyotat, Jacques
• Format: Article
• Language: English
• Subjects: Cerebellum; Clinical Study; Diagnosis; Epidermal growth factor receptors; Genetic disorders; Glioblastoma; Medicine; Medicine & Public Health; Meninges; Metastases; Mutation; Neurofibromatosis; Neurological disorders; Neurology; Oncology; Tumors
• ISSN: 0167-594X; 1573-7373
• DOI: 10.1007/s11060-017-2682-7

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.