Original Article—Liver, Pancreas, and Biliary Tract

Journal of Gastroenterology

, Volume 45, Issue 2, pp 234-243

First online:

Epidemiological study of gastroenteropancreatic neuroendocrine tumors in Japan

  • Tetsuhide ItoAffiliated withDepartment of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University Email author 
  • , Hironobu SasanoAffiliated withDepartment of Pathology, Tohoku University School of Medicine
  • , Masao TanakaAffiliated withDepartment of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University
  • , R. Yoshiyuki OsamuraAffiliated withDepartment of Pathology, Tokai University School of Medicine
  • , Iwao SasakiAffiliated withDepartment of Surgery, Tohoku University Graduate School of Medicine
  • , Wataru KimuraAffiliated withCourse of Organ Functions and Controls, Department of Gastroenterological and General Surgery, Yamagata University School of Medicine
  • , Koji TakanoAffiliated withDepartment of Nephrology and Endocrinology, University of Tokyo Faculty of Medicine
  • , Takao ObaraAffiliated withDepartment of Endocrine Surgery, Tokyo Women’s Medical University
  • , Miyuki IshibashiAffiliated withDepartment of Medicine, Takatsu General Hospital
    • , Kazuwa NakaoAffiliated withDivision of Endocrinology and Metabolism, Department of Medicine and Clinical Science, Kyoto University Graduate School of Medicine
    • , Ryuichiro DoiAffiliated withDepartment of Surgery, Kyoto University
    • , Akira ShimatsuAffiliated withClinical Research Institute, National Hospital Organization Kyoto Medical Center
    • , Toshirou NishidaAffiliated withDepartment of Surgery, Osaka University Graduate School of Medicine
    • , Izumi KomotoAffiliated withDepartment of Surgery, Osaka Saiseikai Noe Hospital
    • , Yukio HirataAffiliated withDepartment of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School
    • , Kazuhiko NakamuraAffiliated withDepartment of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
    • , Hisato IgarashiAffiliated withDepartment of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University
    • , Robert T. JensenAffiliated withDigestive Diseases Branch, National Institutes of Diabetes, Digestive and Kidney Diseases, National Institutes of Health
    • , Bertram WiedenmannAffiliated withDepartment of Hepatology and Gastroenterology, University Medicine Berlin
    • , Masayuki ImamuraAffiliated withDepartment of Surgery, Osaka Saiseikai Noe Hospital

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Abstract

Background

There have been few epidemiological studies on gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in Japan.

Methods

We examined the epidemiology of GEP-NETs [pancreatic endocrine tumors (PETs) and gastrointestinal neuroendocrine tumors (GI-NETs)] in Japan in 2005 using a nationwide stratified random sampling method.

Results

A total of 2,845 individuals received treatment for PETs. Prevalence was estimated as 2.23/100,000 with an annual onset incidence of 1.01/100,000. Non-functioning tumor (NF)-PET constituted 47.4%, followed by insulinoma (38.2%) and gastrinoma (7.9%). Distant metastases were reported in 21% patients with NF-PETs and occurred more frequently as tumor size increased (>2 cm). Multiple endocrine neoplasia type 1 (MEN-1) was detected in 10% of PETs but only in 6.1% of NF-PETs. NF-PETs were detected incidentally by physical examination in 24% patients. In 2005, an estimated 4,406 patients received treatment for GI-NETs. Prevalence was estimated as 3.45/100,000, with an annual onset incidence of 2.10/100,000. The locations of GI-NETs varied: foregut, 30.4%; midgut, 9.6%; and hindgut, 60.0%. Distant metastases were observed in 6%. Lymph node metastases occurred more frequently as tumor size increased (>1 cm). The frequency of MEN-1 complications was 1%. Physical examination revealed GI-NETs in 44% patients. The frequency of symptomatic GI-NETs was 3.4%. Interestingly, 77.1% of patients with foregut GI-NETs had type A gastritis.

Conclusion

Our results show there are large differences in GEP-NETs between Japan and Western nations, primarily due to differences in the presence of MEN-1 in NF-PETs and the location, symptomatic status, and prevalence of malignancy in GI-NETs.

Keywords

Neuroendocrine tumor Endocrine pancreatic tumor Gastrointestinal neuroendocrine tumors Nationwide survey Epidemiology