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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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Language:English
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Summary: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  
ISSN:0167-594X
1573-7373
DOI:10.1007/s11060-024-04589-3