PMID- 24499825 OWN - NLM STAT- MEDLINE DCOM- 20160204 LR - 20220408 IS - 1435-5922 (Electronic) IS - 0944-1174 (Linking) VI - 50 IP - 1 DP - 2015 Jan TI - Epidemiological trends of pancreatic and gastrointestinal neuroendocrine tumors in Japan: a nationwide survey analysis. PG - 58-64 LID - 10.1007/s00535-014-0934-2 [doi] AB - BACKGROUND: Although neuroendocrine tumors (NETs) are rare, the number of patients with NET is increasing. However, in Japan, there have been no epidemiological studies on NET since 2005; thus, the prevalence of NET remains unknown. METHODS: We reported the epidemiology of gastroenteropancreatic neuroendocrine tumors (GEP-NETs) [pancreatic neuroendocrine tumors (PNETs) and gastrointestinal neuroendocrine tumors (GI-NETs)] in Japan in 2005. Here, we conducted the second nationwide survey on patients with GEP-NETs who received treatment in 2010. RESULTS: A total of 3,379 patients received treatment for PNETs in 2010, representing a 1.2-fold increase in the number of patients from 2005 to 2010. The prevalence was estimated to be 2.69/100,000, with an annual onset incidence of 1.27/100,000 in 2010. Non-functioning tumor (NF)-PNETs comprised 65.5% of cases followed by insulinoma (20.9%) and gastrinoma (8.2%). Interestingly, the number of patients with NF-PNETs increased ~1.8 fold since 2005. A total of 19.9% of patients exhibited distant metastasis at initial diagnosis; 4.3% had complications with multiple endocrine neoplasia type 1 (MEN-1), and only 4.0% had NF-PNETs associated with MEN-1. Meanwhile, an estimated 8,088 patients received treatment for GI-NETs, representing a ~1.8-fold increase since 2005. The prevalence was estimated to be 6.42/100,000, with an annual onset incidence of 3.51/100,000. The locations of GI-NETs varied: foregut, 26.1%; midgut, 3.6%; and hindgut, 70.3%. Distant metastasis and complications with MEN-1 were observed in 6.0 and 0.42% at initial diagnosis, respectively. The frequency of carcinoid syndrome in patients with GI-NETs was 3.2%. CONCLUSION: We clarified the epidemiological changes in GEP-NETs from 2005 to 2010 in Japan. FAU - Ito, Tetsuhide AU - Ito T AD - Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan, itopapa@intmed3.med.kyushu-u.ac.jp. FAU - Igarashi, Hisato AU - Igarashi H FAU - Nakamura, Kazuhiko AU - Nakamura K FAU - Sasano, Hironobu AU - Sasano H FAU - Okusaka, Takuji AU - Okusaka T FAU - Takano, Koji AU - Takano K FAU - Komoto, Izumi AU - Komoto I FAU - Tanaka, Masao AU - Tanaka M FAU - Imamura, Masayuki AU - Imamura M FAU - Jensen, Robert T AU - Jensen RT FAU - Takayanagi, Ryoichi AU - Takayanagi R FAU - Shimatsu, Akira AU - Shimatsu A LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140206 PL - Japan TA - J Gastroenterol JT - Journal of gastroenterology JID - 9430794 SB - IM MH - Gastrointestinal Neoplasms/*epidemiology/pathology MH - Health Surveys MH - Humans MH - Japan/epidemiology MH - Malignant Carcinoid Syndrome/epidemiology MH - Multiple Endocrine Neoplasia Type 1/epidemiology MH - Neuroendocrine Tumors/*epidemiology/pathology/secondary MH - Pancreatic Neoplasms/*epidemiology/pathology MH - Prevalence EDAT- 2014/02/07 06:00 MHDA- 2016/02/05 06:00 CRDT- 2014/02/07 06:00 PHST- 2013/07/29 00:00 [received] PHST- 2014/01/10 00:00 [accepted] PHST- 2014/02/07 06:00 [entrez] PHST- 2014/02/07 06:00 [pubmed] PHST- 2016/02/05 06:00 [medline] AID - 10.1007/s00535-014-0934-2 [doi] PST - ppublish SO - J Gastroenterol. 2015 Jan;50(1):58-64. doi: 10.1007/s00535-014-0934-2. Epub 2014 Feb 6.