Rare glial tumors

Handb Clin Neurol. 2016:134:399-415. doi: 10.1016/B978-0-12-802997-8.00024-4.


This chapter describes the epidemiology, pathology, molecular characteristics, clinical and neuroimaging features, treatment, outcome, and prognostic factors of the rare glial tumors. This category includes subependymal giant cell astrocytoma, pleomorphic xanthoastrocytoma, astroblastoma, chordoid glioma of the third ventricle, angiocentric glioma, ganglioglioma, desmoplastic infantile astrocytoma and ganglioma, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, and rosette-forming glioneuronal tumor of the fourth ventricle. Many of these tumors, in particular glioneuronal tumors, prevail in children and young adults, are characterized by pharmacoresistant seizures, and have an indolent course, and long survival following surgical resection. Radiotherapy and chemotherapy are reserved for recurrent and/or aggressive forms. New molecular alterations are increasingly recognized.

Keywords: diagnosis; epidemiology; glial tumors; glioneuronal tumors; molecular biology; outcome; treatment.

Publication types

  • Review

MeSH terms

  • Brain Neoplasms* / diagnosis
  • Brain Neoplasms* / epidemiology
  • Brain Neoplasms* / therapy
  • Glioma* / epidemiology
  • Glioma* / genetics
  • Glioma* / therapy
  • Humans
  • Rare Diseases* / diagnosis
  • Rare Diseases* / epidemiology
  • Rare Diseases* / therapy
  • Treatment Outcome*