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The role of adjuvant chemotherapy in patients with H3K27 altered diffuse midline gliomas: a multicentric retrospective study

Purpose Adult Diffuse midline glioma (DMG) is a very rare disease. DMGs are currently treated with radiotherapy and chemotherapy even if only a few retrospective studies assessed the impact on overall survival (OS) of these approaches. Methods We carried out an Italian multicentric retrospective stu...

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Published in:Journal of neuro-oncology 2024-03, Vol.167 (1), p.145-154
Main Authors: Di Nunno, Vincenzo, Lombardi, Giuseppe, Simonelli, Matteo, Minniti, Giuseppe, Mastronuzzi, Angela, Di Ruscio, Valentina, Corrà, Martina, Padovan, Marta, Maccari, Marta, Caccese, Mario, Simonetti, Giorgia, Berlendis, Arianna, Farinotti, Mariangela, Pollo, Bianca, Antonelli, Manila, Di Muzio, Antonio, Dipasquale, Angelo, Asioli, Sofia, De Biase, Dario, Tosoni, Alicia, Silvani, Antonio, Franceschi, Enrico
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container_title Journal of neuro-oncology
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creator Di Nunno, Vincenzo
Lombardi, Giuseppe
Simonelli, Matteo
Minniti, Giuseppe
Mastronuzzi, Angela
Di Ruscio, Valentina
Corrà, Martina
Padovan, Marta
Maccari, Marta
Caccese, Mario
Simonetti, Giorgia
Berlendis, Arianna
Farinotti, Mariangela
Pollo, Bianca
Antonelli, Manila
Di Muzio, Antonio
Dipasquale, Angelo
Asioli, Sofia
De Biase, Dario
Tosoni, Alicia
Silvani, Antonio
Franceschi, Enrico
description Purpose Adult Diffuse midline glioma (DMG) is a very rare disease. DMGs are currently treated with radiotherapy and chemotherapy even if only a few retrospective studies assessed the impact on overall survival (OS) of these approaches. Methods We carried out an Italian multicentric retrospective study of adult patients with H3K27-altered DMG to assess the effective role of systemic therapy in the treatment landscape of this rare tumor type. Results We evaluated 49 patients from 6 Institutions. The median age was 37.3 years (range 20.1–68.3). Most patients received biopsy as primary approach ( n  = 30, 61.2%) and radiation therapy after surgery ( n  = 39, 79.6%). 25 (51.0%) of patients received concurrent chemotherapy and 26 (53.1%) patients received adjuvant temozolomide. In univariate analysis, concurrent chemotherapy did not result in OS improvement while adjuvant temozolomide was associated with longer OS (21.2 vs. 9.0 months, HR 0.14, 0.05–0.41, p  
doi_str_mv 10.1007/s11060-024-04589-3
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DMGs are currently treated with radiotherapy and chemotherapy even if only a few retrospective studies assessed the impact on overall survival (OS) of these approaches. Methods We carried out an Italian multicentric retrospective study of adult patients with H3K27-altered DMG to assess the effective role of systemic therapy in the treatment landscape of this rare tumor type. Results We evaluated 49 patients from 6 Institutions. The median age was 37.3 years (range 20.1–68.3). Most patients received biopsy as primary approach ( n  = 30, 61.2%) and radiation therapy after surgery ( n  = 39, 79.6%). 25 (51.0%) of patients received concurrent chemotherapy and 26 (53.1%) patients received adjuvant temozolomide. In univariate analysis, concurrent chemotherapy did not result in OS improvement while adjuvant temozolomide was associated with longer OS (21.2 vs. 9.0 months, HR 0.14, 0.05–0.41, p  &lt; 0.001). Multivariate analysis confirmed the role of adjuvant chemotherapy (HR 0.1, 95%CI: 0.03–0.34, p  = 0.003). In patients who progressed after radiation and/or chemotherapy the administration of a second-line systemic treatment had a significantly favorable impact on survival (8.0 vs. 3.2 months, HR 0.2, 95%CI 0.1–0.65, p  = 0.004). Conclusion In our series, adjuvant treatment after radiotherapy can be useful in improving OS of patients with H3K27-altered DMG. When feasible another systemic treatment after treatment progression could be proposed.</description><identifier>ISSN: 0167-594X</identifier><identifier>EISSN: 1573-7373</identifier><identifier>DOI: 10.1007/s11060-024-04589-3</identifier><identifier>PMID: 38457090</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Biopsy ; Chemotherapy ; Glioma ; Medicine ; Medicine &amp; Public Health ; Multivariate analysis ; Neurology ; Oncology ; Patients ; Radiation therapy ; Survival ; Temozolomide</subject><ispartof>Journal of neuro-oncology, 2024-03, Vol.167 (1), p.145-154</ispartof><rights>The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2024. 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The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c326t-74c226afe0822f859579177d81de04bd51f4ba2c9847a85a144071d4238b54c73</cites><orcidid>0000-0003-4441-9834</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27922,27923</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38457090$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Di Nunno, Vincenzo</creatorcontrib><creatorcontrib>Lombardi, Giuseppe</creatorcontrib><creatorcontrib>Simonelli, Matteo</creatorcontrib><creatorcontrib>Minniti, Giuseppe</creatorcontrib><creatorcontrib>Mastronuzzi, Angela</creatorcontrib><creatorcontrib>Di Ruscio, Valentina</creatorcontrib><creatorcontrib>Corrà, Martina</creatorcontrib><creatorcontrib>Padovan, Marta</creatorcontrib><creatorcontrib>Maccari, Marta</creatorcontrib><creatorcontrib>Caccese, Mario</creatorcontrib><creatorcontrib>Simonetti, Giorgia</creatorcontrib><creatorcontrib>Berlendis, Arianna</creatorcontrib><creatorcontrib>Farinotti, Mariangela</creatorcontrib><creatorcontrib>Pollo, Bianca</creatorcontrib><creatorcontrib>Antonelli, Manila</creatorcontrib><creatorcontrib>Di Muzio, Antonio</creatorcontrib><creatorcontrib>Dipasquale, Angelo</creatorcontrib><creatorcontrib>Asioli, Sofia</creatorcontrib><creatorcontrib>De Biase, Dario</creatorcontrib><creatorcontrib>Tosoni, Alicia</creatorcontrib><creatorcontrib>Silvani, Antonio</creatorcontrib><creatorcontrib>Franceschi, Enrico</creatorcontrib><title>The role of adjuvant chemotherapy in patients with H3K27 altered diffuse midline gliomas: a multicentric retrospective study</title><title>Journal of neuro-oncology</title><addtitle>J Neurooncol</addtitle><addtitle>J Neurooncol</addtitle><description>Purpose Adult Diffuse midline glioma (DMG) is a very rare disease. DMGs are currently treated with radiotherapy and chemotherapy even if only a few retrospective studies assessed the impact on overall survival (OS) of these approaches. Methods We carried out an Italian multicentric retrospective study of adult patients with H3K27-altered DMG to assess the effective role of systemic therapy in the treatment landscape of this rare tumor type. Results We evaluated 49 patients from 6 Institutions. The median age was 37.3 years (range 20.1–68.3). Most patients received biopsy as primary approach ( n  = 30, 61.2%) and radiation therapy after surgery ( n  = 39, 79.6%). 25 (51.0%) of patients received concurrent chemotherapy and 26 (53.1%) patients received adjuvant temozolomide. In univariate analysis, concurrent chemotherapy did not result in OS improvement while adjuvant temozolomide was associated with longer OS (21.2 vs. 9.0 months, HR 0.14, 0.05–0.41, p  &lt; 0.001). 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DMGs are currently treated with radiotherapy and chemotherapy even if only a few retrospective studies assessed the impact on overall survival (OS) of these approaches. Methods We carried out an Italian multicentric retrospective study of adult patients with H3K27-altered DMG to assess the effective role of systemic therapy in the treatment landscape of this rare tumor type. Results We evaluated 49 patients from 6 Institutions. The median age was 37.3 years (range 20.1–68.3). Most patients received biopsy as primary approach ( n  = 30, 61.2%) and radiation therapy after surgery ( n  = 39, 79.6%). 25 (51.0%) of patients received concurrent chemotherapy and 26 (53.1%) patients received adjuvant temozolomide. In univariate analysis, concurrent chemotherapy did not result in OS improvement while adjuvant temozolomide was associated with longer OS (21.2 vs. 9.0 months, HR 0.14, 0.05–0.41, p  &lt; 0.001). Multivariate analysis confirmed the role of adjuvant chemotherapy (HR 0.1, 95%CI: 0.03–0.34, p  = 0.003). In patients who progressed after radiation and/or chemotherapy the administration of a second-line systemic treatment had a significantly favorable impact on survival (8.0 vs. 3.2 months, HR 0.2, 95%CI 0.1–0.65, p  = 0.004). Conclusion In our series, adjuvant treatment after radiotherapy can be useful in improving OS of patients with H3K27-altered DMG. When feasible another systemic treatment after treatment progression could be proposed.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>38457090</pmid><doi>10.1007/s11060-024-04589-3</doi><tpages>10</tpages><orcidid>https://orcid.org/0000-0003-4441-9834</orcidid></addata></record>
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subjects Biopsy
Chemotherapy
Glioma
Medicine
Medicine & Public Health
Multivariate analysis
Neurology
Oncology
Patients
Radiation therapy
Survival
Temozolomide
title The role of adjuvant chemotherapy in patients with H3K27 altered diffuse midline gliomas: a multicentric retrospective study
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