类癌综合征在瑞典中部人群的发病率预计大约为0.5/10万人/年[1]Norheim I, Oberg K, Theodorsson-Norheim E, et al. Malignant carcinoid tumors. An analysis of 103 patients with regard to tumor localization, hormone production, and survival. Ann Surg. 1987 Aug;206(2):115-25.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1493111/http://www.ncbi.nlm.nih.gov/pubmed/2440390?tool=bestpractice.com 类癌综合征发生于患有功能性类癌(即症状由释放的激素引起)的患者。 这在所有胃肠道胰腺神经内分泌肿瘤(NETs)中发生率明显增高,尤其是中肠类癌,但是原因不明。[2]Perez EA, Koniaris LG, Snell SE, et al. 7201 carcinoids: increasing incidence overall and disproportionate mortality in the elderly. World J Surg. 2007 May;31(5):1022-30.http://www.ncbi.nlm.nih.gov/pubmed/17429568?tool=bestpractice.com 至今最大的流行病学研究估算出美国人口患病率是3.85/10万人。 从 1999 年起,监测、流行病学和最终结果 (SEER) 项目得到的数据显示,男性患者比例稍高,为 1:1.14,[3]Merrill RM, Capocaccia R, Feuer EJ, et al. Cancer prevalence estimates based on tumour registry data in the Surveillance, Epidemiology, and End Results (SEER) Program. Int J Epidemiol. 2000 Apr;29(2):197-207.http://ije.oxfordjournals.org/cgi/content/full/29/2/197http://www.ncbi.nlm.nih.gov/pubmed/10817114?tool=bestpractice.com 年龄 50-60 岁者占多数,与白人相比,黑人发病率较高,亚洲人和西班牙人发病率较低。[4]Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer. 2003 Feb 15;97(4):934-59.http://www3.interscience.wiley.com/cgi-bin/fulltext/102528538/HTMLSTARThttp://www.ncbi.nlm.nih.gov/pubmed/12569593?tool=bestpractice.com 类癌肿瘤是最常见的功能性神经内分泌肿瘤 (NET)。虽然 NET 的发病率相对较低,年发病率为 5/100,000,但由于该疾病相对惰性的性质,其总体患病率应该高得多。根据监测、流行病学和结果数据库 (SEER) 估算的 NET 患病率大约是 35/100,000。[5]Yao JC, Hassan M, Phan A, et al. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008 Jun 20;26(18):3063-72.http://www.ncbi.nlm.nih.gov/pubmed/18565894?tool=bestpractice.com