Brainstem Low-Grade Gliomas in Children-Excellent Outcomes With Multimodality Therapy

J Child Neurol. 2017 Feb;32(2):194-203. doi: 10.1177/0883073816675547. Epub 2016 Nov 3.

Abstract

Safe maximal surgical resection is the initial treatment of choice for pediatric brainstem low-grade gliomas. Optimal therapy for incompletely resected tumors or that progress after surgery is uncertain. We reviewed the clinical characteristics, therapy, and outcomes of all children with nontectal brainstem low-grade gliomas treated at the University of Michigan between 1993 and 2013. Median age at diagnosis was 6 years; histology was confirmed in 23 of 25 tumors, 64% were pilocytic astrocytoma. Nineteen patients underwent initial tumor resection; 14/19 received no upfront adjuvant therapy. Eight patients in the study had progressive disease; 5 initially resected tumors received chemotherapy at tumor relapse, all with partial or complete radiographic responses. Ten-year progression-free survival is 71% and overall survival, 100%. This single-institution retrospective study demonstrates excellent survival rates for children with brainstem low-grade gliomas. The efficacy of the well-tolerated chemotherapy in this series supports its role in the treatment of unresectable or progressive brainstem low-grade gliomas.

Keywords: brainstem tumors; chemotherapy; low-grade glioma; overall survival; progressive tumors.

MeSH terms

  • Adolescent
  • Brain Stem Neoplasms / diagnostic imaging
  • Brain Stem Neoplasms / genetics
  • Brain Stem Neoplasms / therapy*
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Glioma / diagnostic imaging
  • Glioma / genetics
  • Glioma / therapy*
  • Humans
  • Infant
  • Male
  • Proto-Oncogene Proteins B-raf / genetics
  • Retrospective Studies
  • Treatment Outcome

Substances

  • BRAF protein, human
  • Proto-Oncogene Proteins B-raf