Therapeutic Approach to Chordoid Glioma of the Third Ventricle

Abstract

Chordoid glioma of the third ventricle is considered to be a benign glial tumor located exclusively in the mid-anterior portion of the third ventricle near the hypothalamus and optic nerves, with the histological features of a chordoma and immuno-labeling for glial fibrillary acidic protein. Unfortunately, the clinical outcome of chordoid glioma has been poor, even in patients receiving gross total or partial removal with or without radiotherapy. Three cases of chordoid glioma of the third ventricle were treated with less invasive microsurgery for pathological diagnosis or partial removal without neuro-endocrinological complication, followed by gamma knife radiosurgery using a lower marginal dose for the optic nerves and hypothalamus. Gamma knife radiosurgery was performed after open biopsy in two patients, and after partial removal in the third patient using a lower marginal dose of 10.5 to 12 Gy. Serial magnetic resonance imaging revealed gradual decrease or at least no change in the tumor size, without significant complication at follow up 70 and 66 months later in two cases. The third patient accidentally died 13 months after gamma knife treatment. We conclude that low dose gamma knife radiosurgery after less invasive microsurgery is both safe and effective for the control of chordoid glioma of the third ventricle over a very long follow-up period.

Expression of thyroid transcription factor 1 in a chordoid glioma

“…To date only 55 cases were reported in the literature [2]. The exact pathogenesis of this tumour remains unclear.…”

“…Optimal treatment includes gross total resection whenever feasible. The prognosis of this histologically benign tumour is often poor [2,3]. In a recent review 18 out of 55 patients died within 6 years after surgery [2].…”

Chordoid Glioma of Third Ventricle Presenting as Primary Polydipsia With Aggressive Behavior: A Rare Case With Rare Presentation

“…The histogenesis of chordoid glioma remains uncertain. The immunohistochemical positivity for GFAP and vimentin favors glial origin [9].…”

Chordoid Glioma Originating in the Intrasellar and Suprasellar Regions: Case Report

“…CGs are reported to have poor prognosis. It was reported that 74% of patients with CG died from postoperative complications or tumor recurrence; the most common cause of death was pulmonary embolism (10).…”

“…Adjuvant radiation therapy has been performed in a limited number of cases; Kobayashi et al (10) reported effective residual tumor control with gamma knife radiosurgery in three patients.…”