Object: Diffuse midline glioma (DMG) is a highly aggressive and lethal brain tumor predominantly affecting children and young adults. Previously known as diffuse intrinsic pontine glioma (DIPG) or grade IV brain stem glioma, DMG has recently been reclassified as “diffuse midline glioma” according to the WHO CNS5 nomenclature, expanding the DMG demographic. Limited therapeutic options result in a poor prognosis, despite advances in diagnosis and treatment. Radiotherapy has historically been the primary treatment modality to improve patient survival. Methods: This systematic literature review aims to comprehensively compile information on the diagnosis and treatment of DMG from 1 January 2012 to 31 July 2023. The review followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement and utilized databases such as PubMed, Cochrane Library, and SciELO. Results: Currently, molecular classification of DMG plays an increasingly vital role in determining prognosis and treatment options. Emerging therapeutic avenues, including immunomodulatory agents, anti-GD2 CAR T-cell and anti-GD2 CAR-NK therapies, techniques to increase blood–brain barrier permeability, isocitrate dehydrogenase inhibitors, oncolytic and peptide vaccines, are being explored based on the tumor’s molecular composition. However, more clinical trials are required to establish solid guidelines for toxicity, dosage, and efficacy. Conclusions: The identification of the H3K27 genetic mutation has led to the reclassification of certain midline tumors, expanding the DMG demographic. The field of DMG research continues to evolve, with encouraging findings that underscore the importance of highly specific and tailored therapeutic strategies to achieve therapeutic success.

Background: Lesions in the temporomesial region can be reached by various approaches: subtemporal, transsylvian, transcortical, interhemispheric parieto-occipital, or supracerebellar transtentorial (SCTT). The choice varies according to the characteristics of the lesion and neighboring structures. Case Description: In this clinical case, it is presented a 56-year-old man with long-term evolution of drug-resistant epilepsy secondary to a cavernoma in the left parahippocampal gyrus. After assessing the lesion, it was decided a SCTT approach for its resection in a semi-sitting position, to avoid language disorders or visual damage. The surgery was uneventful and the patient did not present epileptic seizures during 6-month follow-up. Conclusion: Performing a SCTT is safe and feasible option for resection of lesions located in the basal temporomesial region without causing damage to neighboring structures, especially those located in the middle and posterior two-thirds of temporal region.