Is periaquaductal gray matter a potential origin for extralateral ventricular central neurocytomas? Reporting a pediatric periaquaductal central neurocytoma and review of the literature

Central neurocytoma (CN) is a rare, typically intraventricular neuronal tumor most often diagnosed in young adults. Pediatric cases and those arising outside the lateral ventricles are exceptionally uncommon. Here, we report a rare case of a 15-year-old girl with a CN originating from the periaqueductal region, extending into the posterior third and upper fourth ventricles. The lesion was resected through a staged surgical approach involving a supracerebellar infratentorial route followed by endoscopic tumor removal and third ventriculostomy. Histopathological analysis confirmed the diagnosis of central neurocytoma with low proliferative activity. A thorough review of previously reported third and fourth ventricular CNs suggests that the periaqueductal gray matter may serve as a potential origin for extralateral ventricular CNs. Our case reinforces the need to consider such atypical locations in pediatric patients and highlights the value of tailored surgical strategies for achieving gross total resection while preserving neurological function. Close radiological follow-up remains essential due to the uncertain prognosis of these rare variants.