Primary cerebellar glioblastomas in children: clinical presentation and management

Neurosurg Rev. 2021 Jun;44(3):1747-1754. doi: 10.1007/s10143-020-01373-5. Epub 2020 Aug 26.

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: Outcome; Primary cerebellar glioblastomas; Treatment.

MeSH terms

  • Adolescent
  • Antineoplastic Agents / administration & dosage
  • Cerebellar Neoplasms / diagnostic imaging*
  • Cerebellar Neoplasms / mortality
  • Cerebellar Neoplasms / therapy*
  • Child
  • Child, Preschool
  • Disease Management*
  • Female
  • Follow-Up Studies
  • Glioblastoma / diagnostic imaging*
  • Glioblastoma / mortality
  • Glioblastoma / therapy*
  • Humans
  • Male
  • Radiosurgery / methods
  • Radiosurgery / mortality
  • Retrospective Studies
  • Survival Rate / trends

Substances

  • Antineoplastic Agents