Therapeutic approach to chordoid glioma of the third ventricle

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.