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  • Updated 01.24.2019
  • Released 02.20.1996
  • Expires For CME 01.24.2022

Brainstem gliomas in adults

Introduction

This article includes discussion of brainstem gliomas in adults, diffuse midline glioma, diffuse midline glioma with histone mutation, H3 K27M mutant glioma, high-grade midline glioma with H3 K27M mutation, medullary glioma, midbrain glioma, pontine glioma, cystic brainstem glioma, diffuse brainstem glioma, exophytic brainstem glioma, and focal brainstem glioma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

In this article, the author discusses every aspect of brainstem gliomas, including pathology, molecular biology, clinical presentation, neuroimaging and diagnostic evaluation, differential diagnosis, prognosis and complications, surgical considerations, radiotherapy, and chemotherapy. In addition, a detailed list of references is included that contains over 100 articles. Although the article focuses on adult patients, relevant information from the pediatric literature is also included. In this latest update, the author summarizes several new reports on the molecular biology of brainstem gliomas as well as new approaches to therapy.

Key points

• Brainstem gliomas are much less common in adults than in children.

• Brainstem gliomas are a very heterogeneous group of tumors, but are often quite aggressive and difficult to treat, with relatively short survival times.

• A subgroup of brainstem gliomas harbor mutations in genes encoding components of the histone complex.

• Surgical resection of brainstem gliomas can be performed in selective focal tumors. For more diffuse tumors, resection is impossible, and a biopsy is preferred. In many cases of diffuse pontine tumors, MRI can be diagnostic.

• Radiotherapy is always necessary for high-grade tumors, but may not be needed for fully resected low-grade tumors. In general, radiotherapy is required for most brainstem gliomas.

• Chemotherapy has minimal activity against most high-grade brainstem gliomas, but it may be helpful in less aggressive tumor types.

Historical note and terminology

The earliest descriptions of brainstem tumors in the literature were made in the 1930s (34; 70; 85; 15). In a review of 1737 verified brain tumors operated on by Harvey Cushing, Buckley found 25 located within the pons, the majority of which were astrocytomas (34). Gibbs noted that brainstem tumors were most common in children and estimated that the comparative incidence in adults was only 10% (70). A review by Hare and Woolf of 432 pediatric brain tumors seen at the Neurological Institute of New York included only 7 tumors of the brainstem (85). In a study evaluating diffuse pontine gliomas in children, Bailey was 1 of the first authors to draw attention to the high frequency of misdiagnosis in this population (15). Guillain and colleagues and Globus and colleagues were the first to document in detail brainstem tumors affecting the course of the aqueduct of Sylvius (79; 74). In a later review, Guillain was 1 of the first to document the behavior of exophytic brainstem tumors, in a review of 250 intracranial neoplasms (78). The first author to study a population of adult patients with brainstem gliomas was White, who evaluated a series of 44 patients accumulated over 31 years at the Neurological Institute in New York (233). The mean age of the patients was 42 years (range 17 to 68 years); most of those studied pathologically had astrocytic tumors of various grades. The clinical presentation and course of patients in this adult brainstem glioma series was similar to a comparable series of pediatric patients studied earlier at the Neurological Institute.

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