Intraventricular neurocytoma: clinicopathological analysis of seven cases

A retrospective analysis of seven patients with intraventricular neurocytoma is presented. Patient age at diagnosis ranged from 15 to 38 years (mean 24.6 years) and the male:female ratio was 6:1. Raised intracranial pressure due to hydrocephalus was the main cause of the clinical manifestations. An isodense mass with multiple intratumoral cysts and homogeneous contrast enhancement was the characteristic computerized tomography finding. The lesions commonly involved the lateral ventricle with or without extension to the third ventricle. Cerebral angiography showed homogeneous vascular staining in five patients. Magnetic resonance images revealed a mass isointense with the cerebral cortex on both T1- and T2-weighted images. Gadolinium-diethylenetriaminepenta-acetic acid-enhanced images showed homogeneous enhancement. Total removal of the tumor was possible in four patients. Pathologically, six cases were initially diagnosed as oligodendroglioma and the remaining case as ependymoma. However, immunohistochemical studies demonstrated strong positivity for neuron-specific enolase in all seven cases and for synaptophysin in five cases. On electron microscopy, three cases showed well-defined neurosecretory granules and 10-nm microtubules in their cytoplasm and cytoplasmic processes. One patient developed a recurrent tumor 18 months after surgery. The remaining six patients are free of recurrent tumors at 2 to 62 months after surgery. It is suggested that neurocytoma must be included in the differential diagnosis of intraventricular lesions, and that electron microscopic and immunohistochemical studies should be undertaken.