Epidemiology of Supratentorial Central Neurocytomas in Children

Incidence and Prevalence

• 0.25–0.5% of intracranial tumors: Population-based incidence rates for central neurocytomas are not available. In large surgical series, the incidence ranged from 0.25–0.5% of all intracranial tumors (9, 10, 11).

Age Distribution

• Relatively evenly spread across life: The age at diagnosis ranged from 8 days to 67 years, with a mean age of 29 years. 70% were diagnosed in the third and fourth decades of life (10). The age range was slightly lower in one series (9).

Sex Predilection

• None: Although one of the earlier series quoted a male to female ratio of 1:4 (9), it is now believed that both sexes are equally affected.

Geographic Distribution

• None evident: No specific geographic distribution has been reported.

Risk Factors

• None identified: Risk factors have not been identified.

Relationships to Other Disease States and Syndromes

• Often confused with other neuronal tumors: These tumors were previously described as ependymomas of the foramen of Munro or as intraventricular oligodendrogliomas. Primary cerebral neuroblastomas have a similar course and need to be distinguished from these tumors (10). Some tumors presenting with seizures and diagnosed as cerebral neurocytomas (11) may represent DNETs. Supratentorial central neurocytomas have been classified as “neuronal and mixed neuronal-glial tumors” in the recent WHO classification of brain tumors (12).