Dataset Information

Characteristics of H3 K27M-mutant gliomas in adults.

ABSTRACT:

Background

Diffuse H3 K27M-mutant gliomas occur primarily in children but can also be encountered in adults. The aim of this study was to describe the characteristics of H3 K27M-mutant gliomas in adults.

Methods

We analyzed the characteristics of 21 adult H3 K27M-mutant gliomas and compared them with those of 135 adult diffuse gliomas without histone H3 and without isocitrate dehydrogenase (IDH) mutation (IDH/H3 wild type).

Results

The median age at diagnosis in H3 K27M-mutant gliomas was 32 years (range: 18-82 y). All tumors had a midline location (spinal cord n = 6, thalamus n = 5, brainstem n = 5, cerebellum n = 3, hypothalamus n = 1, and pineal region n = 1) and were IDH and BRAF-V600E wild type. The identification of an H3 K27M mutation significantly impacted the diagnosis in 3 patients (14%) for whom the histological aspect initially suggested a diffuse low-grade glioma and in 7 patients (33%) for whom pathological analysis hesitated between a diffuse glioma, ganglioglioma, or pilocytic astrocytoma. Compared with IDH/H3 wild-type gliomas, H3 K27M-mutant gliomas were diagnosed at an earlier age (32 vs 64 y, P < .001), always had a midline location (21/21 vs 21/130, P < .001), less frequently had a methylated MGMT promoter (1/21 vs 52/129, P = .002), and lacked EGFR amplification (0/21 vs 26/128, P = .02). The median survival was 19.6 months in H3 K27M-mutant gliomas and 17 months in IDH/H3 wild-type gliomas (P = .3).

Conclusion

In adults, as in children, H3 K27M mutations define a distinct subgroup of IDH wild-type gliomas characterized by a constant midline location, low rate of MGMT promoter methylation, and poor prognosis.

SUBMITTER: Meyronet D

PROVIDER: S-EPMC5570304 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature

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Publications

Characteristics of H3 K27M-mutant gliomas in adults.

Meyronet David D Esteban-Mader Maud M Bonnet Charlotte C Joly Marie-Odile MO Uro-Coste Emmanuelle E Amiel-Benouaich Alexandra A Forest Fabien F Rousselot-Denis Cécilia C Burel-Vandenbos Fanny F Bourg Véronique V Guyotat Jacques J Fenouil Tanguy T Jouvet Anne A Honnorat Jérôme J Ducray François F

Neuro-oncology 20170801 8

<h4>Background</h4>Diffuse H3 K27M-mutant gliomas occur primarily in children but can also be encountered in adults. The aim of this study was to describe the characteristics of H3 K27M-mutant gliomas in adults.<h4>Methods</h4>We analyzed the characteristics of 21 adult H3 K27M-mutant gliomas and compared them with those of 135 adult diffuse gliomas without histone H3 and without isocitrate dehydrogenase (IDH) mutation (IDH/H3 wild type).<h4>Results</h4>The median age at diagnosis in H3 K27M-mut ...[more]

PMID: 28201752

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Dataset Information

Characteristics of H3 K27M-mutant gliomas in adults.

Background

Methods

Results

Conclusion

Publications

Characteristics of H3 K27M-mutant gliomas in adults.

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