Citations 1
Clinical Outcomes and Prognostic Features of Diffuse Hemispheric Glioma, H3 G34-Mutant: An International Multi-institutional Study
Crowell, Cameron , Mankuzhy, Nikhil P. , Bennett, Julie , Bandopadhayay, Pratiti , Sturm, D., Green, Adam L. , Gupta, Tejpal , Chatterjee, Abhishek , Epari, Sridhar , Chinnaswamy, G., Prasad, Maya , Patel, Sonal, MacNeil, Mary , Vanan, Magimairajan , Larouche, Valérie , Renzi, Samuele , Jones, Jacquelyn , Perreault, Sébastien , Mata-Mbemba, Daddy , Sangatsuda, Yuhei , Yoshimoto, Koji , Lin, Emily , Feddersen, Charlotte R., Pavon-Mengual, Miriam, Vizzini, Olivia, Zápotocký, Michal, Popovacki, Aimee, Schlosser, Mary-Pat , Omene, Egiroh , Alexander, Kimberley L., Satgunaseelan, Laveniya, Williams, Andrew , Yao, Kristin, Ronsley, Rebecca , Cheng, Sylvia, Eisenstat, David, Andre, N., Matheson, K., Chan, Aimee , Lim-Fat, Mary Jane, Lapointe, Sarah , Cayrol, Romain , Coleman, Christina , Juretic, Nikoleta , McThenia, Sheila, Khan, Sara , Salloum, Ralph , Galvin, Robert , Hawkins, Cynthia, Karajannis, Matthias A. , Jones, David T.W., Ligon, K. L., Jabado, Nada, Erker, Craig , Sturm, Dominik , Chinnaswamy, Girish , Patel, Sohil H , Vanan, Magimairajan Issai , Feddersen, Charlotte , Cruz, Ofelia , Pavon-Mengual, Miriam , Vizzini, Olivia , Zápotocký, Michal , Popovacki, Aimee , Klawinski, Darren , Hansford, Jordan R , Schlosser, Mary-Pat , Omene, Egiroh , Alexander, Kimberley L , Satgunaseelan, Laveniya , Williams, Andrew , Yao, Kristin , Ronsley, Rebecca , Cheng, Sylvia , Ludlow, Louise , Eisenstat, David , Khuong-Quang, Dong-Anh , Tabouret, Emeline , André, Nicolas , Matheson, Kara , Chan, Aimee , Lim-Fat, Mary Jane , Lapointe, Sarah , Cayrol, Romain , Coleman, Christina , Juretic, Nikoleta , McThenia, Sheila , Khan, Sara , Wright-Nadkarni, Mariah , Salloum, Ralph , Galvin, Robert T , Sener, Ugur , Hawkins, Cynthia , Imber, Brandon S , Karajannis, Matthias A , Jones, David T W , Ligon, Keith L , Jabado, Nada , Erker, Craig , Larouche, Valérie , Hansford, Jordan R. , Satgunaseelan, Laveniya , Sener, Uğur , Hawkins, Cynthia
Clinical Cancer Research | March 9, 2026 | Volume: 32 | Issue: 11 | Pages: 2243-2254
Abstract Purpose: Knowledge of prognostic factors and long-term survival in patients with diffuse hemispheric glioma, H3 G34–mutant (DHG, H3 G34), remains limited in this tumor with a poor prognosis. Experimental Design: This retrospective, multi-institutional study investigated prognostic variables for patients with DHG, H3 G34, and their association with progression-free survival (PFS) and overall survival (OS). Uni- and multivariable Cox proportional hazard models were applied with multiple imputed datasets. Results: A total of 153 patients (142 G34R, 9 G34V, 2 via DNA methylation) were included. The median age at diagnosis was 17 years (range, 2–45). Initial gross/near total resection (GTR/NTR) was achieved in 43% of patients. Radiation was given in 91% (85% focal irradiation), and initial chemotherapy was given in 87% [70% temozolomide-based (TMZ), 25% TMZ/lomustine, 5% non-TMZ]. Median OS was 24 months [interquartile range (IQR), 22–28] with a median PFS of 14 months (IQR, 12–19). Twelve patients (8%) were found to be long-term survivors (≥5 years). Exploratory multivariable analysis showed that adjuvant radiotherapy [HR, 0.076; 95% confidence interval (CI), 0.033–0.17] and achieving GTR/NTR compared with < NTR (HR, 0.51; 95% CI, 0.33–0.78) were associated with improved PFS. Multivariable analysis showed improved OS with increasing age at diagnosis (HR, 0.70; 95% CI, 0.57–0.87), initial radiotherapy (HR, 0.38; 95% CI, 0.15–0.96), and initial GTR/NTR compared with < NTR (HR, 0.60; 95% CI, 0.37–0.97). Conclusions: This cohort highlights prognostic factors for patients with DHG, H3 G34, and describes relapse patterns and therapy approaches. Clinical trials and prospective registries are needed to improve outcomes.