Extending the Neuroanatomic Territory of Diffuse Midline Glioma, K27M Mutant: Pineal Region Origin

Andrea R. Gilbert, Wafik Zaky, Murat Gokden, Christine E. Fuller, Eylem Ocal, Norman E. Leeds, Gregory N. Fuller

Research output: Contribution to journalArticlepeer-review

15 Scopus citations

Abstract

Diffuse midline glioma, H3-K27M mutant (DMG-K27M) is a newly described, molecularly distinct infiltrative glioma that almost exclusively arises in midline CNS structures, including the brain stem, especially the pons, as well as the thalamus and spinal cord with rare examples seen in the cerebellum, third ventricle, and hypothalamus. To our knowledge, only 1 case of a molecularly confirmed DMG-K27M arising in the pineal region has been previously reported. We present the second occurrence of a tissue-confirmed DMG-K27M of the pineal region, which, to our knowledge, is the first case reported in a child and the first case with documented preoperative MRI. This case, in addition to a prior report described in an adult, defines the lower end of a broad age range of DMG-K27M onset (12-65 years) and establishes the pineal gland as a bona fide site of origin for this newly codified midline glioma.

Original languageEnglish (US)
Pages (from-to)59-63
Number of pages5
JournalPediatric Neurosurgery
Volume53
Issue number1
DOIs
StatePublished - Dec 1 2017

Keywords

  • Diffuse midline glioma
  • H3-K27M mutant
  • Pineal tumor

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Clinical Neurology

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