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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...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Dustri-Verlag Dr. Karl Feistle
2018
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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. |
format | Online Article Text |
id | pubmed-5822176 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Dustri-Verlag Dr. Karl Feistle |
record_format | MEDLINE/PubMed |
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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