A unique case of Central Neurocytoma and Concomitant Brain AVM in a 12- year-old girl with long term follow up and review of the literature.

Central neurocytoma (CNCy) is an exceedingly rare brain tumor occurring mainly within the ventricles in young adults. Anecdotal cases of CNCy occurring simultaneously with other brain lesions have been reported but to the best of our knowledge, no one was accompanied by brain AVM. A 12-year-old girl presented with signs of increased intracranial pressure. A multi-bleb CNCy originated from the septum in the midline, expanding to the lateral and third ventricles, back to the incisura and infratentorial- supra-cerebellar cistern concomitant with a left frontal parafalx AVM. The tumor was removed successfully, and hydrocephalus controlled by ventriculoperitoneal shunting. The AVM bled after 7 years and was extirpated surgically. She is tumor free after 15 years. Presenting the first case of CNCy concomitant with brain AVM in a 12-year-old girl with more than 15 years surgical follow up. Even though we could not find any evidence regarding a genetic problem in our case but “Concomitance of CNCY and AVM” can suggest a congenital origin and a common genetic basis only as a hypothesis.