Descriptive Epidemiology of Craniopharyngiomas in the United States

Introduction: Craniopharyngiomas (CPs) are rare benign brain tumors originating in Rathke's pouch or odontogenetic epithelium. Given current interest in genetic and molecular targeting of specific CP subtypes, updated and comprehensive epidemiologic data on incidence of specific subtypes and differences by age, sex, and race is necessary to inform, and direct resources. Prior studies utilize databases that cover a much smaller proportion of the U.S. population.

Methods: We utilized data from the Central Brain Tumor Registry of the United States (CBTRUS). CBTRUS represents the largest collection of primary brain tumor incidence data in the United States, and represents over 99% of the U.S. population. Age-adjusted annual incidence was calculated using SEER*Stat 8.1.5 and is reported per 100,000 persons. Annual percent change (APC) in incidence rates from 2004 to 2016 was calculated using Joinpoint Regression Program 4.0.1.

Results: From 2004 to 2016, 6,643 CPs were diagnosed in the United States, representing approximately 600 new cases each year. Overall incidence was 0.16 per 100,000 persons. The age distribution was bimodal, with one peak at 5 to 9 years old and another at 65 to 69 years old. Compared with the adamantinomatous subtype, papillary CPs only represented 5.5% of the histologically-identified CPs in 0 to 29 years old, 30.6% in 30 to 59 years old, and 30.4% in 60+ years age. 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.

Conclusion: CPs are rare tumors with bimodal age distribution and equal male-to-female incidence. Black patients had highest incidence of CPs. Adamantinomatous is significantly more common than papillary subtype in adolescent, adult, and elderly populations.

Fig. 1 Craniopharyngioma incidence rate ratios (gender and ethnicity). Shown are the craniopharyngioma incidence rate ratios for gender and ethnicity. These ratios are derived from Central Brain Tumor Registry of the United States (CBTRUS) data that represents the entire U.S. population during the time frame of 2004 to 2016. Neither gender nor ethnicity incidence rate ratios are statistically significant.

Fig. 2 Craniopharyngioma incidence rate ratios by race. Shown are the craniopharyngioma incidence rate ratios by race. These ratios are derived from Central Brain Tumor Registry of the United States (CBTRUS) data that represents the entire US population during the time frame of 2004 to 2016. Using ‘White’ as the reference, the incidence rate ratios for the “Black” and “American Indian/Alaskan Native” races are statistically significant. The incidence rate ratio for “Asian/Pacific Islander” is not statistically significant.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021