PURPOSE

Craniopharyngiomas are rare benign brain tumors originating from errors in differentiation during embryogenesis. Given current interest in treatments that target genetic and molecular signatures of specific craniopharyngioma subtypes, updated and comprehensive epidemiologic data of these subtypes are necessary to inform and direct resources.

METHODS

We utilized data from the Central Brain Tumor Registry of the United States (CBTRUS), which represents 100% of the US population. Incidence by demographics was calculated only for histologically-confirmed cases. Age-adjusted annual incidence was calculated and is reported per 100,000 persons. Annual percent change (APC) in incidence rates from 2004 to 2016 was calculated to assess trends.

RESULTS

From 2004 to 2016, 7441 craniopharyngiomas were diagnosed in the United States, representing approximately 620 new cases each year. The incidence for histologically-confirmed cases was 0.16 per 100,000 persons. The age distribution was bimodal, with one peak in 5- to 9-year-olds and another in 55- to 69-year-olds. Compared with adamantinomatous tumors, papillary craniopharyngiomas only represented 5.5% of the histologically diagnosed craniopharyngiomas in 0- to 29-year-olds, 30.6% in 30- to 59-year-olds, and 30.4% in 60 + year-olds. Incidence was highest amongst Blacks (0.22), followed by Whites (0.15), Asians or Pacific Islanders (0.14), and American Indians/Alaska Natives (0.10). No significant difference was discovered in incidence rates between males and females or Hispanic and non-Hispanic ethnicities.

CONCLUSIONS

Craniopharyngiomas are rare tumors with a bimodal age distribution and an equal male-to-female incidence. Black patients had the highest incidence, and adamantinomatous craniopharyngiomas were significantly more common than papillary tumors in adolescent, adult, and elderly populations.