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
. 2025 Jun 23;17(13):2107.
doi: 10.3390/cancers17132107.

Prognostic Features of Recurrent Midline and H3 K27M-Mutant Glioma

Stephen J Bagley  1 , Yoshie Umemura  2 , Joe S Mendez  3 , Isabel Arrillaga-Romany  4 , Kevin J Bielamowicz  5 , Nick Butowski  6 , Kelley Hutchins  7 , Xiao-Tang Kong  8 , Yazmin Odia  9 , Akanksha Sharma  10 , Lauren Weintraub  11 , Carl Koschmann  12 , Patrick Y Wen  13 , Amanda M Saratsis  14   15 , Tom Brundage  16 , Samuel C Ramage  16 , Rohinton S Tarapore  16 , Truman Knowles  6 , Dewen Yang  16 , Joshua E Allen  16 , Timothy Cloughesy  17
Affiliations

Prognostic Features of Recurrent Midline and H3 K27M-Mutant Glioma

Stephen J Bagley et al. Cancers (Basel). .

Abstract

High-grade glial tumors represent the most morbid form of brain cancer [...].

Keywords: H3 K27M; diffuse glioma; diffuse midline glioma.

PubMed Disclaimer

Conflict of interest statement

Patrick Wen, Isabel Arrillaga-Romany, Nick Butowski, Yazmin Odia, Akanksha Sharma, Yoshie Umemura, Lauren Weintraub, Timothy Cloughesy, and Stephen J. Bagley have received research and/or clinical trial support from Chimerix, Inc., a subsidiary of Jazz Pharmaceuticals. Amanda M. Saratsis is a paid consultant for Chimerix, Inc. Patrick Wen has served on an advisory board for Chimerix, Inc. Dewen Yang was an employee of Chimerix, Inc. at the time of the conduct of this study. Tom Brundage, Samuel C. Ramage, Rohinton Tarapore, and Joshua E. Allen are employees of and have stock ownership in Chimerix, Inc. Joshua E. Allen has royalties and licenses associated with ONC201 and related products, and has patents for ONC201 and related products. Truman Knowles, Joe S. Mendez, Kevin J. Bielamowicz, and Xiao-Tang Kong have no relevant conflicts of interest to declare.

Figures

Figure 1
Figure 1
Patient selection flowchart citing inclusion and exclusion criteria and numbers ultimately yielding n = 44 patient records analyzed in the present study.
Figure 2
Figure 2
Overall survival (A) from first recurrence in all patients, (B) from first recurrence in patients excluding DIPG, leptomeningeal spread, CSF dissemination, or primary spinal tumors, (C) from second recurrence, and (D) from third recurrence. In general, OS was identical (5.1 months) from first recurrence in all patients, and in the cohort of patients excluding DIPG, leptomeningeal spread, CSF dissemination, and spinal tumors (A and B, respectively). Survival at second recurrence (5.3 months) was slightly longer than that at third recurrence (4.7 months, C and D, respectively). Shaded areas represent the 95% CI.
Figure 3
Figure 3
Overall survival by (A) H3 K27M mutation status, (B) DIPG versus other patients, (C) primary spinal tumors, (D) infratentorial tumors excluding DIPG, and (E) supratentorial tumors. Shaded areas represent the 95% CI.
See this image and copyright information in PMC

References

    1. Weller M., van den Bent M., Preusser M., Le Rhun E., Tonn J.C., Minniti G., Bendszus M., Balana C., Chinot O., Dirven L., et al. EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood. Nat. Rev. Clin. Oncol. 2020;18:170–186. doi: 10.1038/s41571-020-00447-z. - DOI - PMC - PubMed
    1. Khuong-Quang D.-A., Buczkowicz P., Rakopoulos P., Liu X.-Y., Fontebasso A.M., Bouffet E., Bartels U., Albrecht S., Schwartzentruber J., Letourneau L., et al. K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas. Acta Neuropathol. 2012;124:439–447. doi: 10.1007/s00401-012-0998-0. - DOI - PMC - PubMed
    1. Schwartzentruber J., Korshunov A., Liu X.-Y., Jones D.T.W., Pfaff E., Jacob K., Sturm D., Fontebasso A.M., Khuong-Quang D.-A., Tönjes M., et al. Driver mutations in histone H3.3 and chromatin remodelling genes in paediatric glioblastoma. Nature. 2012;482:226–231. doi: 10.1038/nature10833. Erratum in Nature 2012, 484, 130. - DOI - PubMed
    1. Saratsis A.M., Kambhampati M., Snyder K., Yadavilli S., Devaney J.M., Harmon B., Hall J., Raabe E.H., An P., Weingart M., et al. Comparative multidimensional molecular analyses of pediatric diffuse intrinsic pontine glioma reveals distinct molecular subtypes. Acta Neuropathol. 2013;127:881–895. doi: 10.1007/s00401-013-1218-2. - DOI - PMC - PubMed
    1. Karremann M., Gielen G.H., Hoffmann M., Wiese M., Colditz N., Warmuth-Metz M., Bison B., Claviez A., van Vuurden D.G., O von Bueren A., et al. Diffuse high-grade gliomas with H3 K27M mutations carry a dismal prognosis independent of tumor location. Neuro-Oncology. 2017;20:123–131. doi: 10.1093/neuonc/nox149. - DOI - PMC - PubMed

LinkOut - more resources