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H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis

Background: H3 K27M mutation was originally described in pediatric diffuse intrinsic pontine gliomas (DIPGs), but has been recently recognized to occur also in adult midline diffuse gliomas, as well as midline tumors with other morphologies, including gangliogliomas (GGs), anaplastic GGs, pilocytic...

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Autores principales: Kleinschmidt-DeMasters, Bette K., Mulcahy Levy, Jean M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dustri-Verlag Dr. Karl Feistle 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5822176/
https://www.ncbi.nlm.nih.gov/pubmed/29393845
http://dx.doi.org/10.5414/NP301085
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author Kleinschmidt-DeMasters, Bette K.
Mulcahy Levy, Jean M.
author_facet Kleinschmidt-DeMasters, Bette K.
Mulcahy Levy, Jean M.
author_sort Kleinschmidt-DeMasters, Bette K.
collection PubMed
description Background: H3 K27M mutation was originally described in pediatric diffuse intrinsic pontine gliomas (DIPGs), but has been recently recognized to occur also in adult midline diffuse gliomas, as well as midline tumors with other morphologies, including gangliogliomas (GGs), anaplastic GGs, pilocytic astrocytomas (PAs), and posterior fossa ependymomas. In a few patients with H3 K27M-mutant tumors with these alternate morphologies, longer survival has been reported, making grading difficult for the neuropathologist. Few series compare tumors in adult vs. pediatric cohorts; we report our 4-year experience. Materials and methods: Text Word database searches using “H3 K27M” in reports generated between January 2013 and November 10, 2017 were used to identify patients. Clinical and histological features as well as survival were evaluated for each case. Results: 28 H3 K27M-mutant tumors were identified, with equal numbers of adults (13) vs. children (15). For adults, mean and median age was 52 years (range = 27 – 81 years), 2 decades older than a recently-published adult series. Tumors involved thalamic (adult = 7; pediatric = 7), spinal cord (adult = 4; pediatric = 2), pons (adult = 1; pediatric = 6), and hypothalamic (n = 1) sites. Other morphologies at presentation included pure GG (n = 3, pediatric) and PA (n = 1, adult). One adult and 1 pediatric patient each presented with leptomeningeal dissemination or developed leptomeningeal dissemination within 1 year after diagnosis, with transformation from PA or GG histology to glioblastoma. Mean survival was 9.3 (adults) vs. 8.9 (pediatric) months. Patients with tumors of other morphologies (GG, PA) did not enjoy extended survival. Conclusion: H3 K27M-mutant tumors can affect patients at advanced ages, may show leptomeningeal dissemination at time of presentation, and “pure” GG or PA morphology is not rare. Regardless of patient age or tumor morphology, patients fare equally poorly.
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spelling pubmed-58221762018-03-01 H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis Kleinschmidt-DeMasters, Bette K. Mulcahy Levy, Jean M. Clin Neuropathol Research Article Background: H3 K27M mutation was originally described in pediatric diffuse intrinsic pontine gliomas (DIPGs), but has been recently recognized to occur also in adult midline diffuse gliomas, as well as midline tumors with other morphologies, including gangliogliomas (GGs), anaplastic GGs, pilocytic astrocytomas (PAs), and posterior fossa ependymomas. In a few patients with H3 K27M-mutant tumors with these alternate morphologies, longer survival has been reported, making grading difficult for the neuropathologist. Few series compare tumors in adult vs. pediatric cohorts; we report our 4-year experience. Materials and methods: Text Word database searches using “H3 K27M” in reports generated between January 2013 and November 10, 2017 were used to identify patients. Clinical and histological features as well as survival were evaluated for each case. Results: 28 H3 K27M-mutant tumors were identified, with equal numbers of adults (13) vs. children (15). For adults, mean and median age was 52 years (range = 27 – 81 years), 2 decades older than a recently-published adult series. Tumors involved thalamic (adult = 7; pediatric = 7), spinal cord (adult = 4; pediatric = 2), pons (adult = 1; pediatric = 6), and hypothalamic (n = 1) sites. Other morphologies at presentation included pure GG (n = 3, pediatric) and PA (n = 1, adult). One adult and 1 pediatric patient each presented with leptomeningeal dissemination or developed leptomeningeal dissemination within 1 year after diagnosis, with transformation from PA or GG histology to glioblastoma. Mean survival was 9.3 (adults) vs. 8.9 (pediatric) months. Patients with tumors of other morphologies (GG, PA) did not enjoy extended survival. Conclusion: H3 K27M-mutant tumors can affect patients at advanced ages, may show leptomeningeal dissemination at time of presentation, and “pure” GG or PA morphology is not rare. Regardless of patient age or tumor morphology, patients fare equally poorly. Dustri-Verlag Dr. Karl Feistle 2018 2018-02-02 /pmc/articles/PMC5822176/ /pubmed/29393845 http://dx.doi.org/10.5414/NP301085 Text en © Dustri-Verlag Dr. K. Feistle http://creativecommons.org/licenses/by/2.5/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Kleinschmidt-DeMasters, Bette K.
Mulcahy Levy, Jean M.
H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis
title H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis
title_full H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis
title_fullStr H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis
title_full_unstemmed H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis
title_short H3 K27M-mutant gliomas in adults vs. children share similar histological features and adverse prognosis
title_sort h3 k27m-mutant gliomas in adults vs. children share similar histological features and adverse prognosis
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5822176/
https://www.ncbi.nlm.nih.gov/pubmed/29393845
http://dx.doi.org/10.5414/NP301085
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