胰腺神经内分泌肿瘤较少见,在所有源于胰腺的癌瘤中所占比例小于 2%。 总体来讲,胃肠胰神经内分泌癌较少见,粗发病率为每年 3/10 万。[2]Oberg K, Jelic S; ESMO Guidelines Working Group. Neuroendocrine gastroenteropancreatic tumors: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol. 2012;23(suppl 7):vii124-30.http://annonc.oxfordjournals.org/content/19/suppl_2/ii104.full.pdf+htmlhttp://www.ncbi.nlm.nih.gov/pubmed/18456741?tool=bestpractice.com 据估计,美国神经内分泌肿瘤病例少于 3000 例。胰腺血管活性肠肽瘤是该组中更罕见的亚型,在美国,该病的年发病率为 1例/10万至 2 例/10万。[3]Friesen S. Update on the diagnosis and treatment of rare neuroendocrine tumors. Surg Clin North Am. 1987 Apr;67(2):379-93.http://www.ncbi.nlm.nih.gov/pubmed/3031836?tool=bestpractice.com 当前美国的 VIP 瘤患病率尚不清楚。 迄今为止、文献报道仅 300 例,[4]Ghaferi AA, Chojnacki KA, Long WD, et al. Pancreatic VIPomas: subject review and one institutional experience. J Gastointest Surg. 2008 Feb;12(2):382-93.http://www.ncbi.nlm.nih.gov/pubmed/17510774?tool=bestpractice.com[5]Smith SL, Branton SA, Avino AJ, et al. Vasoactive intestinal polypeptide secreting islet cell tumors: a 15-year experience and review of the literature. Surgery. 1998 Dec;124(6):1050-5.http://www.ncbi.nlm.nih.gov/pubmed/9854582?tool=bestpractice.com[6]Peng SY, Li JT, Liu YB, et al. Diagnosis and treatment of VIPoma in China: (case report and 31 cases review) diagnosis and treatment of VIPoma. Pancreas. 2004 Jan;28(1):93-7.http://www.ncbi.nlm.nih.gov/pubmed/14707737?tool=bestpractice.com[7]Nikou GC, Toubanankis C, Nikolaou P, et al. VIPomas: an update in diagnosis and management in a series of 11 patients. Hepatogastroenterology. 2005 Jul-Aug;52(64):1259-65.http://www.ncbi.nlm.nih.gov/pubmed/16001675?tool=bestpractice.com 胰腺血管活性肠肽瘤的全球年发病率为 5 例/100 万。[8]Yao JC, Eisner MP, Leary C, et al. Population-based study of islet cell carcinoma. Ann Surg Oncol. 2007 Dec;14(12):3492-500.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2077912/http://www.ncbi.nlm.nih.gov/pubmed/17896148?tool=bestpractice.com
男女发病率相似,发病年龄为 11~75 岁、中位年龄为 51 岁。[4]Ghaferi AA, Chojnacki KA, Long WD, et al. Pancreatic VIPomas: subject review and one institutional experience. J Gastointest Surg. 2008 Feb;12(2):382-93.http://www.ncbi.nlm.nih.gov/pubmed/17510774?tool=bestpractice.com