Chordoid glioma: an entity occurring not exclusively in the third ventricle

Chordoid gliomas are extremely rare entities, which are generally considered occurring exclusively in the third ventricle. Despite the low-grade histological grade, aggressive behaviors have been reported in literatures. Due to the low morbidity, the origins, clinical, and radiological features, management and prognosis are still yet to be well elucidated. We retrospectively reviewed the clinical profiles from a series of 6 patients with chordoid gliomas. All patients underwent surgical treatment, and the diagnoses were based on histopathological examinations. Magnetic resonance imaging (MRI) was performed perioperatively. Follow-up outcomes were presented. This case series consisted of three male and three female patients (age range 27–67 years; mean age 43.3 years). MRI results showed tumors in the third ventricle (4/6), temporal-parietal-occipital lobe involving the lateral ventricle (1/6), and cerebellar hemisphere (1/6). Three tumors were solid, and the others were cystic-solid. Hydrocephalus was present in one patient. The T1-weighted imaging showed hypo- to isointensity, and T2-weighted imaging showed iso- to hyperintensity; enhancement was homogeneous (4/6) or heterogeneous (2/6). Diffusion-weighted imaging showed no evidence of restricted diffusion. Magnetic resonance spectrum showed an elevated choline value and reduced N-acetylaspartate value. Gross total resection was achieved in all patients, and during an average follow-up period of 35.8 months, no recurrence was noted. Chordoid gliomas can occur outside the third ventricle with a great diagnostic challenge. The MRI characteristics suggest a low-grade tumor, and the accurate diagnosis depends on pathological criteria. Complete surgical resection is associated with a favorable outcome.