Clinical Outcomes and Prognostic Features of Diffuse Hemispheric Glioma, H3 G34-Mutant: An International Multi-institutional Study.
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Crowell Cameron, Mankuzhy Nikhil P, Bennett Julie, Bandopadhayay Pratiti, Sturm Dominik, Green Adam L, Gupta Tejpal, Chatterjee Abhishek, Epari Sridhar, Chinnaswamy Girish, Prasad Maya, Patel Sohil H, MacNeil Mary, Vanan Magimairajan Issai, Larouche Valérie, Renzi Samuele, Jones Jacquelyn, Perreault Sébastien, Mata-Mbemba Daddy, Sangatsuda Yuhei, Yoshimoto Koji, Lin Emily, 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
Аннотация
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.