Abstract / Summary
Chordoid gliomas are rare World Health Organization Grade II neoplasms of the third ventricle. While Gross Total Resection (GTR) has traditionally been the primary surgical objective, the intimate adherence to the hypothalamus and optic apparatus of these tumors creates a therapeutic dilemma for balancing oncological control against the risk of severe neurological and endocrine morbidity. This study aims to guide optimal management by bridging the evidence gap with the largest systematic review to date, analyzing clinical characteristics, surgical outcomes, and survival data. A systematic review was conducted according to PRISMA 2020 guidelines, searching Web of Science, PubMed, Scopus, and Embase for studies from database inception to November 2025. Data included patient demographics, clinical presentation, radiological phenotypes, surgical techniques, molecular profiles, and follow-up outcomes. Kaplan-Meier survival estimates and log-rank tests were used to assess survival outcomes by extent of resection. All analyses were performed using R-software (version 4.3.1). The cohort (N = 198; mean age 41.8 years; female-to-male ratio 2:1) predominantly presented with headache (51.3%), visual disturbances (37.5%), and cognitive deficits (24.4%). GTR was achieved in 56% of patients, while 32% underwent Subtotal Resection (STR), and 10% biopsy only. Kaplan-Meier analysis revealed a significant survival advantage for GTR, with a stable 5-year survival rate of 91.9% compared to 54.7% for STR (p = 0.0089). Molecular profiling identified PRKCA D463H as the predominant driver mutation, with BRAF V600E observed in a minority of cases. GTR is associated with superior long-term survival in the literature and may be considered when anatomically feasible. However, because this association may be confounded by tumor adherence and surgical selection, resection strategies must be strictly individualized to balance tumor control against hypothalamic morbidity.
Primary Source
Neurosurgical review
Ask Prognia AI
Have questions about this review article?
Prognia AI can search this source alongside 35M+ PubMed papers and current ESC, AHA, NICE, and ADA guidelines to give you a fully cited clinical answer.