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Surgical strategies and long-term survival for third ventricle chordoid gliomas: a systematic review and clinical
Omar Alomari1, Beyzanur Güney1, Irem Uslu2,3
1Hamidiye International School of Medicine, University of Health Sciences, Istanbul, 3400, Türkiye.
Abstract:
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.
Insights
Gross Total Resection (GTR) for chordoid gliomas offers superior survival rates compared to Subtotal Resection (STR). However, individualized surgical strategies are crucial to balance tumor control with potential hypothalamic morbidity.
Area of Science:
- Neurosurgery
- Oncology
- Neuropathology
Background:
- Chordoid gliomas are rare WHO Grade II neoplasms affecting the third ventricle.
- Their adherence to critical structures like the hypothalamus poses surgical challenges, balancing oncological goals with neurological and endocrine risks.
Purpose of the Study:
- To conduct the largest systematic review on chordoid gliomas to date.
- To analyze clinical characteristics, surgical outcomes, and survival data to guide optimal management.
Main Methods:
- Systematic review following PRISMA 2020 guidelines, searching major databases (Web of Science, PubMed, Scopus, Embase).
- Analysis of 198 patients' data including demographics, presentation, surgical techniques, molecular profiles, and outcomes.
- Kaplan-Meier survival analysis to compare outcomes based on resection extent.
Main Results:
- Headache, visual disturbances, and cognitive deficits were common presentations.
- Gross Total Resection (GTR) was achieved in 56% of cases, Subtotal Resection (STR) in 32%, and biopsy in 10%.
- GTR demonstrated a significant survival advantage, with 5-year survival rates of 91.9% vs. 54.7% for STR (p=0.0089). PRKCA D463H was the predominant mutation.
Conclusions:
- Gross Total Resection (GTR) is associated with improved long-term survival for chordoid gliomas when anatomically feasible.
- Individualized surgical strategies are essential to mitigate hypothalamic morbidity and optimize outcomes.
