Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Jan;38(1):4-13.
doi: 10.1007/s10014-020-00378-8. Epub 2020 Sep 29.

H3 G34-mutant high-grade glioma

Ka Young Lim  1 , Jae Kyung Won  1   2 , Chul-Kee Park  3   2 , Seung-Ki Kim  3   2 , Seung Hong Choi  4   2 , Taemin Kim  5   2 , Hongseok Yun  2 , Sung-Hye Park  6   7   8
Affiliations

H3 G34-mutant high-grade glioma

Ka Young Lim et al. Brain Tumor Pathol. 2021 Jan.

Abstract

H3F3A G34 (H3.3 G34)-mutant high-grade gliomas (HGG) are rare, and newly recognized infiltrating gliomas of the cerebral hemisphere. Here, we report the clinicopathological and molecular characteristics of four H3.3 G34-mutant gliomas in terms of its biological behavior compared to those of glioblastomas (GBMs) and H3 K27M-mutant diffuse midline gliomas (DMGs) of our hospital. The median age of the four patients with H3.3 G34 HGG was 44.5 years (14-66 years). Three patients had tumors in the cerebral hemisphere, whereas one patient had synchronous double tumors in the cerebral hemisphere and posterior fossa. All these tumors were high-grade glioma, but neither microvascular proliferation nor necrosis. They displayed uniform genetic and epigenetic signatures; ATRX-mutant, MGMT promoter-methylated, Olig2-negative, but IDH- and TERT promoter-wildtype. The median survival rate of H3.3 G34-mutant HGGs, IDH-was 23.5 months. In conclusion, H3.3 G34-mutant gliomas were unique HGGs with uniform genetic and epigenetic abnormalities, which suggested a single phylogenic origin. The median survival of H3.3 G34-mutant HGGs was better than those of IDH-wildtype GBMs and H3 K27M-mutant DMGs, but worse than that of IDH-mutant GBM. The tumor-infiltrating area and resectability may be the crucial parameters for the prognosis of the patients.

Keywords: Diffuse glioma; Glioblastoma; H3.3 G34; High grade glioma; Survival.

PubMed Disclaimer

References

    1. Behjati S, Tarpey PS, Presneau N, Scheipl S, Pillay N, Van Loo P, Wedge DC, Cooke SL, Gundem G, Davies H et al (2013) Distinct H3F3A and H3F3B driver mutations define chondroblastoma and giant cell tumor of bone. Nat Genet 45:1479–1482 - DOI
    1. Bjerke L, Mackay A, Nandhabalan M, Burford A, Jury A, Popov S, Bax DA, Carvalho D, Taylor KR, Vinci M et al (2013) Histone H3.3. mutations drive pediatric glioblastoma through upregulation of MYCN. Cancer Discov 3:512–519 - DOI
    1. Brennan CW, Verhaak RG, McKenna A, Campos B, Noushmehr H, Salama SR, Zheng S, Chakravarty D, Sanborn JZ, Berman SH et al (2013) The somatic genomic landscape of glioblastoma. Cell 155:462–477 - DOI
    1. Fang J, Huang Y, Mao G, Yang S, Rennert G, Gu L, Li H, Li GM (2018) Cancer-driving H3G34V/R/D mutations block H3K36 methylation and H3K36me3–MutSalpha interaction. Proc Natl Acad Sci USA 115:9598–9603 - DOI
    1. Gielen GH, Gessi M, Hammes J, Kramm CM, Waha A, Pietsch T (2013) H3F3A K27M mutation in pediatric CNS tumors: a marker for diffuse high-grade astrocytomas. Am J Clin Pathol 139:345–349 - DOI

LinkOut - more resources