Intraventricular Gangliogliomas: A Review

Objective Gangliogliomas (GG) are benign, primary neoplasms most commonly noted in young adults. Intraventricular location is rare. We report a case of a multicentric intraventricular GG posing diagnostic and therapeutic challenges and in addition provide a detailed literature review of intraventricular GG. Methods A 15-year-old girl presented with features of raised intracranial pressure of short duration. Imaging revealed 2 separate lesions situated in the anterior and posterior third ventricle with hydrocephalus. The patient underwent ventriculoperitoneal shunt insertion, followed by excision of lesions in 2 stages. Both specimens revealed features of GG and the patient had an uneventful recovery. For the literature review, only pure intraventricular GG were included and intraventricular extensions of paraventricular/extraventricular GG were not considered. A brief comparison of clinicoradiologic characteristics between intraventricular and parenchymal GG is provided. Results Including ours, 21 cases were identified. Male/female ratio was 1.3:1. Peak age of occurrence was the third to fourth decade. Features of increased intracranial pressure were the most common presenting features and seizures were noted in one quarter of cases. Gross total resection was achieved in 90% and recurrences and mortality were noted in 10% each. Conclusions Intraventricular GG are rare tumors. Complete surgical excision achieves excellent results. The role of adjuvant therapy is controversial.