Diffuse hemispheric glioma, H3G34 mutant (DHG) is a rare, high-grade, infiltrative WHO grade 4 brain tumor most often found in adolescents and young adults.[1][2] The majority are found in the frontal, parietal, and temporal lobes.[3]
| Diffuse hemispheric glioma | |
|---|---|
| Other names | DHG, DHG-G34R/V |
| Specialty | Neuro-oncology |
| Causes | Mutation in the H3F3A gene, Non-inherited |
| Treatment | Chemotherapy (Temozolomide), Radiotherapy, Surgery |
Diagnosis
editPrior to 2021, most DHG's fell into the classification of Glioblastoma (GBM). However, modern sequencing techniques have revealed that the molecular alterations driving the cancer are entirely distinct to GBM, leading to changes in the World Health Organization classification system.[4][5][2] They are further distinguished from GBM by their radiological presentations, including no or faint contrast enhancement.[3] A diagnosis can only be definitively made with genetic testing of the tumor.[6]
Treatment
editAvailable treatment for DHG will vary depending on the location in the brain that the tumor arises, but typically the first stage of treatment is surgical resection, with the aim of removing as much of the tumor as possible (i.e. gross- or near-total resection). Concomitantly, patients will undergo radiotherapy or proton therapy. Following this, patients will undergo chemotherapy, most commonly with Temozolomide.[7]
Prognosis
editMedian time to progression is 10 months. After progression, the median time to death is 5 months. Non-pediatric patients and patients with near-or-gross total resection have increased survival duration.[8]
Pathology
editDiffuse hemispheric gliomas have a distinct mutation in the histone gene H3F3A.[8]
References
edit- ^ Picart, Thiébaud; Barritault, Marc; Poncet, Delphine; Berner, Lise-Prune; Izquierdo, Cristina; Tabouret, Emeline; Figarella-Branger, Dominique; Idbaïh, Ahmed; Bielle, Franck; Bourg, Véronique; Vandenbos, Fanny Burel; Moyal, Elizabeth Cohen-Jonathan; Uro-Coste, Emmanelle; Guyotat, Jacques; Honnorat, Jérôme (2021). "Characteristics of diffuse hemispheric gliomas, H3 G34-mutant in adults". Neuro-Oncology Advances. 3 (1): vdab061. doi:10.1093/noajnl/vdab061. ISSN 2632-2498. PMC 8156974. PMID 34056608.
- ^ a b Weller, Michael; van den Bent, Martin; Preusser, Matthias; Le Rhun, Emilie; Tonn, Jörg C.; Minniti, Giuseppe; Bendszus, Martin; Balana, Carmen; Chinot, Olivier; Dirven, Linda; French, Pim; Hegi, Monika E.; Jakola, Asgeir S.; Platten, Michael; Roth, Patrick (March 2021). "EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood". Nature Reviews Clinical Oncology. 18 (3): 170–186. doi:10.1038/s41571-020-00447-z. ISSN 1759-4782. PMC 7904519. PMID 33293629.
- ^ a b Shao, Hanbing; Gong, Jing; Su, Xiaorui; Chen, Ni; Li, Shuang; Yang, Xibiao; Zhang, Simin; Huang, Zhangfeng; Hu, Wei; Gong, Qiyong; Liu, Yaou; Yue, Qiang (2024-03-01). "MRI characteristics of H3 G34-mutant diffuse hemispheric gliomas and possible differentiation from IDH-wild-type glioblastomas in adolescents and young adults". Journal of Neurosurgery. Pediatrics. 33 (3): 236–244. doi:10.3171/2023.10.PEDS23235. ISSN 1933-0715. PMID 38157540.
- ^ d’Amati, Antonio; Bargiacchi, Lavinia; Rossi, Sabrina; Carai, Andrea; Bertero, Luca; Barresi, Valeria; Errico, Maria Elena; Buccoliero, Anna Maria; Asioli, Sofia; Marucci, Gianluca; Del Baldo, Giada; Mastronuzzi, Angela; Miele, Evelina; D’Antonio, Federica; Schiavello, Elisabetta (2024-03-13). "Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?". Frontiers in Molecular Neuroscience. 17. doi:10.3389/fnmol.2024.1268038. ISSN 1662-5099. PMC 10966132. PMID 38544524.
- ^ Osborn, A. G.; Louis, D. N.; Poussaint, T. Y.; Linscott, L. L.; Salzman, K. L. (2022-07-01). "The 2021 World Health Organization Classification of Tumors of the Central Nervous System: What Neuroradiologists Need to Know". American Journal of Neuroradiology. 43 (7): 928–937. doi:10.3174/ajnr.A7462. ISSN 0195-6108. PMC 9262075. PMID 35710121.
- ^ Antonelli, Manila; Poliani, Pietro Luigi (December 2022). "Adult type diffuse gliomas in the new 2021 WHO Classification". Pathologica. 114 (6): 397–409. doi:10.32074/1591-951X-823. ISSN 1591-951X. PMC 9763975. PMID 36534419.
- ^ "" Systematic review of diffuse hemispheric glioma, H3 G34-mutant: Outcomes and associated clinical factors "". academic.oup.com. Retrieved 2024-06-09.
- ^ a b Kurokawa, Ryo; Baba, Akira; Kurokawa, Mariko; Pinarbasi, Emile S.; Makise, Naohiro; Ota, Yoshiaki; Kim, John; Srinivasan, Ashok; Moritani, Toshio (January 2022). "Neuroimaging features of diffuse hemispheric glioma, H3 G34-mutant: A case series and systematic review". Journal of Neuroimaging. 32 (1): 17–27. doi:10.1111/jon.12939. hdl:2027.42/171545. ISSN 1051-2284. PMID 34632671.