Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH, USA.
Department of Neurological Surgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA.
Pituitary. 2021 Aug;24(4):517-522. doi: 10.1007/s11102-021-01127-6. Epub 2021 Jan 28.
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.
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.
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.
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.
颅咽管瘤是一种罕见的良性脑肿瘤，起源于胚胎发生过程中的分化错误。鉴于目前对针对特定颅咽管瘤亚型的遗传和分子特征的治疗方法的兴趣，更新和全面的这些亚型的流行病学数据对于提供信息和指导资源是必要的。
我们利用了美国中央脑肿瘤登记处（CBTRUS）的数据，该数据代表了美国 100%的人口。仅对组织学确诊病例计算了按人口统计学划分的发病率。计算了年龄调整后的年发病率，并以每 10 万人为单位报告。计算了 2004 年至 2016 年发病率的年百分变化（APC），以评估趋势。
2004 年至 2016 年，美国诊断出 7441 例颅咽管瘤，每年约有 620 例新发病例。组织学确诊病例的发病率为每 10 万人 0.16 例。年龄分布呈双峰型，一个高峰在 5 至 9 岁，另一个在 55 至 69 岁。与造釉细胞瘤相比，在 0 至 29 岁的患者中，乳头状颅咽管瘤仅占组织学诊断的颅咽管瘤的 5.5%，在 30 至 59 岁的患者中占 30.6%，在 60 岁以上的患者中占 30.4%。黑人的发病率最高（0.22），其次是白人（0.15）、亚洲人或太平洋岛民（0.14）和美洲印第安人/阿拉斯加原住民（0.10）。男性和女性或西班牙裔和非西班牙裔之间的发病率无显著差异。
颅咽管瘤是一种罕见的肿瘤，具有双峰型年龄分布和相等的男女发病率。黑人患者的发病率最高，在青少年、成年和老年人群中，造釉细胞瘤明显比乳头状肿瘤更为常见。
