Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients
Background Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients. Methods We retrospectively analyzed the clinical, neuro-radiological, histologica...
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Veröffentlicht in: | Journal of neuro-oncology 2022-02, Vol.156 (3), p.615-623 |
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creator | Grimaldi, Stéphan Harlay, Vincent Appay, Romain Bequet, Céline Petrirena, Grégorio Campello, Chantal Barrié, Maryline Autran, Didier Boissonneau, Sébastien Graillon, Thomas Figarella-Branger, Dominique Nanni, Isabelle Chinot, Olivier Tabouret, Emeline |
description | Background
Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients.
Methods
We retrospectively analyzed the clinical, neuro-radiological, histological, and molecular characteristics of all cases of adult thalamic glioma in our regional center.
Results
We included 38 adult patients. Median age at diagnosis was 56.5 years old (range, 24–80). Median KPS at diagnosis was 70%. Two-thirds of patients presented with tumor necrosis on MRI. Bithalamic lesions were present in four patients. The median volume of enhancement associated with lesions was relatively small (14 mm
3
). Two patients had undergone partial surgical resection. All other patients underwent biopsy. Median PFS was 7.1 months (95% CI [3.7–10.5]) and median OS was 15.6 months (95% CI [11.7–19.6]). Among 20 patients with available tumor samples for molecular analyses, only 4 (20%) presented with
H3K27M
mutation. Patients with
H3K27M
mutation had longer survival compared to those without. Finally, we identified a long-term survivor population characterized by a younger age, no cognitive impairment, low steroid dose treatment and the presence of
H3K27M
mutation.
Conclusion
Thalamic adult glioma differs from bithalamic glioma in children with regards to its clinical, radiological and molecular profiles. Long-term survival is observed in young patients with limited symptoms and
H3K27M
mutation. A larger prospective cohort is needed to validate these findings. |
doi_str_mv | 10.1007/s11060-022-03943-7 |
format | Article |
fullrecord | <record><control><sourceid>proquest_hal_p</sourceid><recordid>TN_cdi_hal_primary_oai_HAL_hal_03976537v1</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2618235582</sourcerecordid><originalsourceid>FETCH-LOGICAL-c409t-5e89ea515233d32364066d95ceabea6e4d45776241d80e06a5faef155b1b3de33</originalsourceid><addsrcrecordid>eNp9kUtPGzEUha2qVQlp_0AXlaVuYDHF9vUjXkYICGoQGyp153rGN-mgeaRjDxL_HochVOqCjW1df-fcax9CvnD2nTNmziLnTLOCCVEwsBIK847MuDL5AAbekxnj2hTKyl9H5DjGe8aYNMA_kiOQ1kqrYUZ-L8PYJLqCH8Lc0HZMPmGg6Y9vfFtXdNvUfevpzqcauxRpqOOu8Y_U0xJTwoHuhn7b9bGOe01Hx-5gcZB8Ih82von4-WWfk5-XF3fnq2J9e3V9vlwXlWQ2FQoXFr3iSgAEEKAl0zpYVaEv0WuUQSpjtJA8LBgy7dXG44YrVfISAgLMyenkm0d3u6Fu_fDoel-71XLt9rX8RUYrMA88sycTm6f_O2JMrq1jhU3jO-zH6ITmCwFK5WVOvv2H3vfj0OWXZAo4LJg1--Zioqqhj3HAzesEnLl9Vm7KyuWs3HNWzmTR1xfrsWwxvEoO4WQAJiDmq26Lw7_eb9g-AZs4ncQ</addsrcrecordid><sourcetype>Open Access Repository</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2631380973</pqid></control><display><type>article</type><title>Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients</title><source>MEDLINE</source><source>SpringerNature Journals</source><creator>Grimaldi, Stéphan ; Harlay, Vincent ; Appay, Romain ; Bequet, Céline ; Petrirena, Grégorio ; Campello, Chantal ; Barrié, Maryline ; Autran, Didier ; Boissonneau, Sébastien ; Graillon, Thomas ; Figarella-Branger, Dominique ; Nanni, Isabelle ; Chinot, Olivier ; Tabouret, Emeline</creator><creatorcontrib>Grimaldi, Stéphan ; Harlay, Vincent ; Appay, Romain ; Bequet, Céline ; Petrirena, Grégorio ; Campello, Chantal ; Barrié, Maryline ; Autran, Didier ; Boissonneau, Sébastien ; Graillon, Thomas ; Figarella-Branger, Dominique ; Nanni, Isabelle ; Chinot, Olivier ; Tabouret, Emeline</creatorcontrib><description>Background
Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients.
Methods
We retrospectively analyzed the clinical, neuro-radiological, histological, and molecular characteristics of all cases of adult thalamic glioma in our regional center.
Results
We included 38 adult patients. Median age at diagnosis was 56.5 years old (range, 24–80). Median KPS at diagnosis was 70%. Two-thirds of patients presented with tumor necrosis on MRI. Bithalamic lesions were present in four patients. The median volume of enhancement associated with lesions was relatively small (14 mm
3
). Two patients had undergone partial surgical resection. All other patients underwent biopsy. Median PFS was 7.1 months (95% CI [3.7–10.5]) and median OS was 15.6 months (95% CI [11.7–19.6]). Among 20 patients with available tumor samples for molecular analyses, only 4 (20%) presented with
H3K27M
mutation. Patients with
H3K27M
mutation had longer survival compared to those without. Finally, we identified a long-term survivor population characterized by a younger age, no cognitive impairment, low steroid dose treatment and the presence of
H3K27M
mutation.
Conclusion
Thalamic adult glioma differs from bithalamic glioma in children with regards to its clinical, radiological and molecular profiles. Long-term survival is observed in young patients with limited symptoms and
H3K27M
mutation. A larger prospective cohort is needed to validate these findings.</description><identifier>ISSN: 0167-594X</identifier><identifier>EISSN: 1573-7373</identifier><identifier>DOI: 10.1007/s11060-022-03943-7</identifier><identifier>PMID: 34994963</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Adult ; Biopsy ; Brain Neoplasms - genetics ; Brain Neoplasms - therapy ; Brain tumors ; Clinical Study ; Cognitive ability ; Diagnosis ; Glioma ; Glioma - genetics ; Glioma - therapy ; Histones - genetics ; Humans ; Life Sciences ; Magnetic resonance imaging ; Medical prognosis ; Medicine ; Medicine & Public Health ; Mutation ; Neurology ; Oncology ; Patients ; Prognosis ; Retrospective Studies ; Survival ; Thalamus ; Thalamus - pathology</subject><ispartof>Journal of neuro-oncology, 2022-02, Vol.156 (3), p.615-623</ispartof><rights>The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022</rights><rights>2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.</rights><rights>The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2022.</rights><rights>Distributed under a Creative Commons Attribution 4.0 International License</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c409t-5e89ea515233d32364066d95ceabea6e4d45776241d80e06a5faef155b1b3de33</citedby><cites>FETCH-LOGICAL-c409t-5e89ea515233d32364066d95ceabea6e4d45776241d80e06a5faef155b1b3de33</cites><orcidid>0000-0002-4031-2178</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s11060-022-03943-7$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s11060-022-03943-7$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>230,314,780,784,885,27924,27925,41488,42557,51319</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/34994963$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://hal.science/hal-03976537$$DView record in HAL$$Hfree_for_read</backlink></links><search><creatorcontrib>Grimaldi, Stéphan</creatorcontrib><creatorcontrib>Harlay, Vincent</creatorcontrib><creatorcontrib>Appay, Romain</creatorcontrib><creatorcontrib>Bequet, Céline</creatorcontrib><creatorcontrib>Petrirena, Grégorio</creatorcontrib><creatorcontrib>Campello, Chantal</creatorcontrib><creatorcontrib>Barrié, Maryline</creatorcontrib><creatorcontrib>Autran, Didier</creatorcontrib><creatorcontrib>Boissonneau, Sébastien</creatorcontrib><creatorcontrib>Graillon, Thomas</creatorcontrib><creatorcontrib>Figarella-Branger, Dominique</creatorcontrib><creatorcontrib>Nanni, Isabelle</creatorcontrib><creatorcontrib>Chinot, Olivier</creatorcontrib><creatorcontrib>Tabouret, Emeline</creatorcontrib><title>Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients</title><title>Journal of neuro-oncology</title><addtitle>J Neurooncol</addtitle><addtitle>J Neurooncol</addtitle><description>Background
Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients.
Methods
We retrospectively analyzed the clinical, neuro-radiological, histological, and molecular characteristics of all cases of adult thalamic glioma in our regional center.
Results
We included 38 adult patients. Median age at diagnosis was 56.5 years old (range, 24–80). Median KPS at diagnosis was 70%. Two-thirds of patients presented with tumor necrosis on MRI. Bithalamic lesions were present in four patients. The median volume of enhancement associated with lesions was relatively small (14 mm
3
). Two patients had undergone partial surgical resection. All other patients underwent biopsy. Median PFS was 7.1 months (95% CI [3.7–10.5]) and median OS was 15.6 months (95% CI [11.7–19.6]). Among 20 patients with available tumor samples for molecular analyses, only 4 (20%) presented with
H3K27M
mutation. Patients with
H3K27M
mutation had longer survival compared to those without. Finally, we identified a long-term survivor population characterized by a younger age, no cognitive impairment, low steroid dose treatment and the presence of
H3K27M
mutation.
Conclusion
Thalamic adult glioma differs from bithalamic glioma in children with regards to its clinical, radiological and molecular profiles. Long-term survival is observed in young patients with limited symptoms and
H3K27M
mutation. A larger prospective cohort is needed to validate these findings.</description><subject>Adult</subject><subject>Biopsy</subject><subject>Brain Neoplasms - genetics</subject><subject>Brain Neoplasms - therapy</subject><subject>Brain tumors</subject><subject>Clinical Study</subject><subject>Cognitive ability</subject><subject>Diagnosis</subject><subject>Glioma</subject><subject>Glioma - genetics</subject><subject>Glioma - therapy</subject><subject>Histones - genetics</subject><subject>Humans</subject><subject>Life Sciences</subject><subject>Magnetic resonance imaging</subject><subject>Medical prognosis</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Mutation</subject><subject>Neurology</subject><subject>Oncology</subject><subject>Patients</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><subject>Survival</subject><subject>Thalamus</subject><subject>Thalamus - pathology</subject><issn>0167-594X</issn><issn>1573-7373</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNp9kUtPGzEUha2qVQlp_0AXlaVuYDHF9vUjXkYICGoQGyp153rGN-mgeaRjDxL_HochVOqCjW1df-fcax9CvnD2nTNmziLnTLOCCVEwsBIK847MuDL5AAbekxnj2hTKyl9H5DjGe8aYNMA_kiOQ1kqrYUZ-L8PYJLqCH8Lc0HZMPmGg6Y9vfFtXdNvUfevpzqcauxRpqOOu8Y_U0xJTwoHuhn7b9bGOe01Hx-5gcZB8Ih82von4-WWfk5-XF3fnq2J9e3V9vlwXlWQ2FQoXFr3iSgAEEKAl0zpYVaEv0WuUQSpjtJA8LBgy7dXG44YrVfISAgLMyenkm0d3u6Fu_fDoel-71XLt9rX8RUYrMA88sycTm6f_O2JMrq1jhU3jO-zH6ITmCwFK5WVOvv2H3vfj0OWXZAo4LJg1--Zioqqhj3HAzesEnLl9Vm7KyuWs3HNWzmTR1xfrsWwxvEoO4WQAJiDmq26Lw7_eb9g-AZs4ncQ</recordid><startdate>20220201</startdate><enddate>20220201</enddate><creator>Grimaldi, Stéphan</creator><creator>Harlay, Vincent</creator><creator>Appay, Romain</creator><creator>Bequet, Céline</creator><creator>Petrirena, Grégorio</creator><creator>Campello, Chantal</creator><creator>Barrié, Maryline</creator><creator>Autran, Didier</creator><creator>Boissonneau, Sébastien</creator><creator>Graillon, Thomas</creator><creator>Figarella-Branger, Dominique</creator><creator>Nanni, Isabelle</creator><creator>Chinot, Olivier</creator><creator>Tabouret, Emeline</creator><general>Springer US</general><general>Springer Nature B.V</general><general>Springer Verlag</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7TK</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><scope>1XC</scope><orcidid>https://orcid.org/0000-0002-4031-2178</orcidid></search><sort><creationdate>20220201</creationdate><title>Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients</title><author>Grimaldi, Stéphan ; Harlay, Vincent ; Appay, Romain ; Bequet, Céline ; Petrirena, Grégorio ; Campello, Chantal ; Barrié, Maryline ; Autran, Didier ; Boissonneau, Sébastien ; Graillon, Thomas ; Figarella-Branger, Dominique ; Nanni, Isabelle ; Chinot, Olivier ; Tabouret, Emeline</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c409t-5e89ea515233d32364066d95ceabea6e4d45776241d80e06a5faef155b1b3de33</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Adult</topic><topic>Biopsy</topic><topic>Brain Neoplasms - genetics</topic><topic>Brain Neoplasms - therapy</topic><topic>Brain tumors</topic><topic>Clinical Study</topic><topic>Cognitive ability</topic><topic>Diagnosis</topic><topic>Glioma</topic><topic>Glioma - genetics</topic><topic>Glioma - therapy</topic><topic>Histones - genetics</topic><topic>Humans</topic><topic>Life Sciences</topic><topic>Magnetic resonance imaging</topic><topic>Medical prognosis</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Mutation</topic><topic>Neurology</topic><topic>Oncology</topic><topic>Patients</topic><topic>Prognosis</topic><topic>Retrospective Studies</topic><topic>Survival</topic><topic>Thalamus</topic><topic>Thalamus - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Grimaldi, Stéphan</creatorcontrib><creatorcontrib>Harlay, Vincent</creatorcontrib><creatorcontrib>Appay, Romain</creatorcontrib><creatorcontrib>Bequet, Céline</creatorcontrib><creatorcontrib>Petrirena, Grégorio</creatorcontrib><creatorcontrib>Campello, Chantal</creatorcontrib><creatorcontrib>Barrié, Maryline</creatorcontrib><creatorcontrib>Autran, Didier</creatorcontrib><creatorcontrib>Boissonneau, Sébastien</creatorcontrib><creatorcontrib>Graillon, Thomas</creatorcontrib><creatorcontrib>Figarella-Branger, Dominique</creatorcontrib><creatorcontrib>Nanni, Isabelle</creatorcontrib><creatorcontrib>Chinot, Olivier</creatorcontrib><creatorcontrib>Tabouret, Emeline</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><collection>Hyper Article en Ligne (HAL)</collection><jtitle>Journal of neuro-oncology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Grimaldi, Stéphan</au><au>Harlay, Vincent</au><au>Appay, Romain</au><au>Bequet, Céline</au><au>Petrirena, Grégorio</au><au>Campello, Chantal</au><au>Barrié, Maryline</au><au>Autran, Didier</au><au>Boissonneau, Sébastien</au><au>Graillon, Thomas</au><au>Figarella-Branger, Dominique</au><au>Nanni, Isabelle</au><au>Chinot, Olivier</au><au>Tabouret, Emeline</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients</atitle><jtitle>Journal of neuro-oncology</jtitle><stitle>J Neurooncol</stitle><addtitle>J Neurooncol</addtitle><date>2022-02-01</date><risdate>2022</risdate><volume>156</volume><issue>3</issue><spage>615</spage><epage>623</epage><pages>615-623</pages><issn>0167-594X</issn><eissn>1573-7373</eissn><abstract>Background
Adult thalamic gliomas are a rare entity whose management is challenging for physicians. The aim of this study is to describe the characteristics and prognostic factors of thalamic gliomas in adult patients.
Methods
We retrospectively analyzed the clinical, neuro-radiological, histological, and molecular characteristics of all cases of adult thalamic glioma in our regional center.
Results
We included 38 adult patients. Median age at diagnosis was 56.5 years old (range, 24–80). Median KPS at diagnosis was 70%. Two-thirds of patients presented with tumor necrosis on MRI. Bithalamic lesions were present in four patients. The median volume of enhancement associated with lesions was relatively small (14 mm
3
). Two patients had undergone partial surgical resection. All other patients underwent biopsy. Median PFS was 7.1 months (95% CI [3.7–10.5]) and median OS was 15.6 months (95% CI [11.7–19.6]). Among 20 patients with available tumor samples for molecular analyses, only 4 (20%) presented with
H3K27M
mutation. Patients with
H3K27M
mutation had longer survival compared to those without. Finally, we identified a long-term survivor population characterized by a younger age, no cognitive impairment, low steroid dose treatment and the presence of
H3K27M
mutation.
Conclusion
Thalamic adult glioma differs from bithalamic glioma in children with regards to its clinical, radiological and molecular profiles. Long-term survival is observed in young patients with limited symptoms and
H3K27M
mutation. A larger prospective cohort is needed to validate these findings.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>34994963</pmid><doi>10.1007/s11060-022-03943-7</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0002-4031-2178</orcidid></addata></record> |
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subjects | Adult Biopsy Brain Neoplasms - genetics Brain Neoplasms - therapy Brain tumors Clinical Study Cognitive ability Diagnosis Glioma Glioma - genetics Glioma - therapy Histones - genetics Humans Life Sciences Magnetic resonance imaging Medical prognosis Medicine Medicine & Public Health Mutation Neurology Oncology Patients Prognosis Retrospective Studies Survival Thalamus Thalamus - pathology |
title | Adult H3K27M mutated thalamic glioma patients display a better prognosis than unmutated patients |
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