IDH-mutant brainstem gliomas in adolescent and young adult patients: Report of three cases and review of the literature

Ellen K Chang; Matthew A Smith-Cohn; Benita Tamrazi; Jianling Ji; Mark Krieger; Matthias Holdhoff; Charles G Eberhart; Ashley S Margol; Jennifer A Cotter

doi:10.1111/bpa.12959

IDH-mutant brainstem gliomas in adolescent and young adult patients: Report of three cases and review of the literature

Brain Pathol. 2021 Jul;31(4):e12959. doi: 10.1111/bpa.12959. Epub 2021 May 7.

Authors

Ellen K Chang¹, Matthew A Smith-Cohn^{2

3}, Benita Tamrazi⁴, Jianling Ji⁵, Mark Krieger⁶, Matthias Holdhoff⁷, Charles G Eberhart⁸, Ashley S Margol¹, Jennifer A Cotter⁵

Affiliations

¹ Division of Hematology, Oncology and Blood & Marrow Transplantation, Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, Los Angeles, CA, USA.
² Department of Neurology, Johns Hopkins Hospital, Baltimore, MD, USA.
³ Neuro-Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.
⁴ Department of Radiology, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁵ Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁶ Division of Neurosurgery, Children's Hospital Los Angeles, Los Angeles, CA, USA.
⁷ Department of Oncology, Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD, USA.
⁸ Department of Pathology, Johns Hopkins Hospital, Baltimore, MD, USA.

Abstract

Isocitrate dehydrogenase (IDH) mutations are rare in pediatric and adolescent gliomas. We recently identified three adolescent/young adult (AYA) patients with IDH-mutant low grade gliomas of the brainstem with several key clinicopathologic and molecular features in common. We discuss these three cases and review the current literature.

Keywords: IDH; brain biopsy; brainstem glioma; isocitrate dehydrogenase; low grade glioma; young adult.

Publication types

Case Reports
Letter
Research Support, Non-U.S. Gov't
Review

MeSH terms

Adult
Brain Neoplasms / genetics*
Brain Neoplasms / pathology*
Brain Stem / pathology
Brain Stem Neoplasms / diagnosis
Brain Stem Neoplasms / genetics
Brain Stem Neoplasms / pathology
Glioma / diagnosis
Glioma / genetics*
Glioma / pathology*
Humans
Isocitrate Dehydrogenase / genetics*
Male
Mutation / genetics
Young Adult

Substances

Isocitrate Dehydrogenase