Primary cerebellar glioblastomas in children: clinical presentation and management
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ORIGINAL ARTICLE
Primary cerebellar glioblastomas in children: clinical presentation and management Qiguang Wang 1 & Jian Cheng 1 & Zhang Si 1 & Wenke Liu 1 & Xuhui Hui 1 & Qiang Li 1 & Yan Ju 1 Received: 10 March 2020 / Revised: 7 August 2020 / Accepted: 18 August 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Pediatric cerebellar glioblastomas (pcGBMs) are rare and their characteristics remain ill-defined. We conducted a retrospective analysis of pediatric cerebellar glioblastomas who underwent surgery from 2008 to 2019 in our department. Besides, we performed a literature review of the literature data on pcGBMs. Ten children with mean age of 9.4 years were included. During the follow-up, six patients died with mean survival time of 11.7 months, four patients survived with mean follow-up of 28 months. Seven patients underwent molecular analysis, no patients detected IDH1 mutations, four patients (57.1%) had H3K27M mutations, and two patients (28.6%) had MGMT promoter methylation. The literature review identified 38 pcGBMs cases (including ours), with mean age of 8.84 ± 4.20 years (range, 1–16 years). Increased ICP was the commonest sign. Eighteen (47.4%) patients underwent GTR and fifteen (45.5%) patients received STR. Postoperative radiation (RT) was conducted in 28 patients (75.7%) and 23 patients (65.7%) received chemotherapy. During the follow-up, 25 patients died with mean survival time of 12.21 months and 11 patients survived with average follow-up of 29.3 months. Kaplan-Meier survival depicted chemotherapy (P < 0.001) or radiation (P < 0.001) had positive impact on overall survival. Multivariate analysis revealed chemotherapy was a significant predictor of survival with a hazard ratio of 3.264 (P = 0.038). Our study found mean overall survival time for pcGBMs patients was 12.21 months. PcGBMs may have distinct molecular features, with higher incidence of H3K27M mutation and were always IDH1 wild-type. We recommend the routine postoperative radiotherapy and chemotherapy in pcGBMs. Keywords Primary cerebellar glioblastomas . Treatment . Outcome
Introduction Brain tumors are the most common solid tumors in children, (40–50%) of which are constituted by astrocytomas [8]. Pediatric glioblastomas (GBMs) are rare, with the incidence of approximately 0.85 per 100,000 [5]. Pediatric GBM tumors found in the cerebellum are even more rare, with only 28 cases have been reported and the
Qiguang Wang and Jian Cheng contributed equally to this work. Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10143-020-01373-5) contains supplementary material, which is available to authorized users. * Yan Ju [email protected]; [email protected] 1
Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China
literature reports are limited to case reports and small case series [2, 4, 7, 19, 22, 26, 30, 31, 33, 34, 36, 38]. Therefore, neither clinical features nor the optimum treatment of which has been fully discussed. It is rep
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