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J Neurooncol. 2019 Mar 12. doi: 10.1007/s11060-019-03134-x. [Epub ahead of print]

Incidence and clinicopathologic features of H3 K27M mutations in adults with radiographically-determined midline gliomas.

Author information

1
Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, 201 North Broadway, Viragh Building, 9th floor, Post Box 3, Baltimore, MD, 21287, USA.
2
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
3
Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
4
Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
5
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
6
Department of Pathology, University of Maryland School of Medicine, Baltimore, MD, USA.
7
Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins, 201 North Broadway, Viragh Building, 9th floor, Post Box 3, Baltimore, MD, 21287, USA. mholdho1@jhmi.edu.
8
Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. mholdho1@jhmi.edu.

Abstract

PURPOSE:

H3 K27 mutations, most commonly in H3F3A, are common in diffuse midline glioma. The exact frequency of these mutations in adults with gliomas in the midline location is unknown. This study was conducted to define the incidence of H3 K27M mutations in this location and to compare clinicopathological features with those of patients who do not harbor this mutation.

METHODS:

Consecutive glioma cases from 2007 to 2017 were screened for gliomas in the midline location. Immunohistochemistry was performed on all available tissue for mutations of H3 K27M, IDH1, and ARTX.

RESULTS:

Of 850 gliomas screened, 163 cases had midline glioma on MRI. Sufficient FFPE tissue was available for 123 cases (75%). H3 K27M mutation was identified in 18 of 123 cases (15%). All except one H3 K27M-mutant tumors were WHO grade III or IV on histology, while non-mutant tumors encompassed all four grades. The most common midline locations for H3 K27M-mutated tumors were midbrain (2/3; 67%), pons (4/11; 36%), and cerebellum (6/24; 25%). As compared to H3 K27M-wildtype tumors, there were no differences in age at diagnosis, sex, tumor grade, contrast enhancement on MRI, extent of resection, or treatment received. In this cohort, median survival was longer for patients with H3 K27M-mutated tumors (n = 18; 17.6 months) compared with high-grade wildtype tumors (n = 74; 7.7 months, p = 0.03).

CONCLUSIONS:

H3 K27M mutations are common in midline gliomas in adults and can present in all midline locations. Survival comparison between H3 K27M-mutant and wildtype midline gliomas suggests that survival may be similar or possibly improved if the mutation is present.

KEYWORDS:

Diffuse midline glioma; Glioma; H3 K27M; IDH1

PMID:
30864101
DOI:
10.1007/s11060-019-03134-x

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