Background: Central Neurocytomas (CNs) are rare brain tumors, making up less than 1% of all primary tumors within the CNS. They are commonly located in the lateral ventricles, and often present with visual changes and symptoms of obstructive hydrocephalus. Histopathology shows characteristics similar to ependymomas and oligodendrogliomas, however tumor cells display neuronal differentiation, and immunohistochemical stains typically for synaptophysin. Gross total resection is the most important prognostic indicator of survival.

Case Description: We describe the case of a 48-year-old male with a CN originating in the third ventricle with expansion through the cerebral aqueduct into the fourth ventricle. He presented with bi-frontal headaches, imaging revealed an avidly enhancing tumor occupying the inferior third ventricle, cerebral aqueduct, with expansion into the fourth ventricle. An interhemispheric craniotomy with a transcallosal transchoroidal approach to the third ventricle was performed, this provided a trajectory that paralleled the long axis of the tumor. Post-operative imaging confirmed a near total resection with linear residual enhancement on the anterior wall of the fourth ventricle. Intensity modulated radiotherapy was performed, 7-month follow-up imaging was clean.

Conclusion: CNs are rare brain tumors, most commonly located within the lateral ventricles. We describe a rare case of a CN spanning from the third ventricle into the cerebral aqueduct and fourth ventricle. To our knowledge, this is only the fourth reported case of such a tumor. Surgical approach must be carefully selected, as gross total resection is the most important prognostic indicator.