Clinical features of insulinoma in patients with multiple endocrine neoplasia type 1: analysis of the database of the MEN Consortium of Japan

Akihiro Sakurai; Masanori Yamazaki; Shinichi Suzuki; Toshihiko Fukushima; Tsuneo Imai; Toyone Kikumori; Takahiro Okamoto; Kiyomi Horiuchi; Shinya Uchino; Shinji Kosugi; Masanobu Yamada; Izumi Komoto; Kazuhiro Hanazaki; Masatsune Itoh; Tatsuya Kondo; Masatomo Mihara; Masayuki Imamura

doi:10.1507/endocrj.EJ12-0173

ORIGINALS

Clinical features of insulinoma in patients with multiple endocrine neoplasia type 1: analysis of the database of the MEN Consortium of Japan

Akihiro Sakurai, Masanori Yamazaki, Shinichi Suzuki, Toshihiko Fukushima, Tsuneo Imai, Toyone Kikumori, Takahiro Okamoto, Kiyomi Horiuchi, Shinya Uchino, Shinji Kosugi, Masanobu Yamada, Izumi Komoto, Kazuhiro Hanazaki, Masatsune Itoh, Tatsuya Kondo, Masatomo Mihara, Masayuki Imamura

Author information

Akihiro Sakurai
Department of Medical Genetics, Shinshu University School of Medicine, Matsumoto 390-8621, Japan
Division of Diabetes, Endocrinology and Metabolism, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto 390-8621, Japan
Masanori Yamazaki
Division of Diabetes, Endocrinology and Metabolism, Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto 390-8621, Japan
Shinichi Suzuki
Department of Breast, Endocrine and Thyroid Surgery, Fukushima Medical University, Fukushima 960-1295, Japan
Toshihiko Fukushima
Department of Breast, Endocrine and Thyroid Surgery, Fukushima Medical University, Fukushima 960-1295, Japan
Tsuneo Imai
Department of Breast and Endocrine Surgery, Nagoya University Hospital, Nagoya 466-8550, Japan
Toyone Kikumori
Department of Breast and Endocrine Surgery, Nagoya University Hospital, Nagoya 466-8550, Japan
Takahiro Okamoto
Department of Endocrine Surgery, Tokyo Women’s Medical University, Tokyo 162-8666, Japan
Kiyomi Horiuchi
Department of Endocrine Surgery, Tokyo Women’s Medical University, Tokyo 162-8666, Japan
Shinya Uchino
Noguchi Thyroid Clinic and Hospital Foundation, Beppu 874-0932, Japan
Shinji Kosugi
Department of Medical Ethics/Medical Genetics, Kyoto University School of Public Health, Kyoto 606-8501, Japan
Masanobu Yamada
Department of Medicine and Molecular Science, Gunma University Graduate School of Medicine, Maebashi 371-8511, Japan
Izumi Komoto
Department of Surgery, Kansai Electric Power Company Hospital, Osaka 553-0003, Japan
Kazuhiro Hanazaki
Department of Surgery, Kochi Medical School, Kochi University, Nankoku 783-8505, Japan
Masatsune Itoh
Department of Pediatrics, Kanazawa Medical University, Uchinada 920-0293, Japan
Tatsuya Kondo
Department of Metabolic Medicine, Kumamoto University Faculty of Life Sciences, Kumamoto 860-8556, Japan
Masatomo Mihara
Department of Molecular Endocrinology and Metabolism, Tokyo Medical and Dental University, Tokyo 113-8519, Japan
Masayuki Imamura
Department of Surgery, Kansai Electric Power Company Hospital, Osaka 553-0003, Japan

Keywords: Multiple endocrine neoplasia type 1 (MEN1), Database, Adolescence, Hypoglycemia

JOURNAL FREE ACCESS

2012 Volume 59 Issue 10 Pages 859-866

DOI https://doi.org/10.1507/endocrj.EJ12-0173

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Details

Abstract

More than 50% of patients with multiple endocrine neoplasia type 1 (MEN1) develop gastroenteropancreatic neuroendocrine tumors (GEPNETs), and insulinoma is the second most common functioning GEPNET. Compared to other functioning and nonfunctioning GEPNETs in MEN1, insulinoma is considered to develop at a younger age. To clarify the clinical features of insulinoma developed in Japanese patients with MEN1, a recently constructed database of Japanese MEN1 patients was analyzed. Among 560 registered cases, insulinoma was seen in 69 patients and information on age at diagnosis was available for 54 patients. Tumors predominantly occurred in the body and tail of the pancreas. The mean age at diagnosis of insulinoma (34.8 ± 16.7 yrs) was significantly younger than that of gastrinoma (50.6 ± 14.3 yrs) and nonfunctioning tumor (44.7 ± 13.3 yrs) in patients with MEN1. Patients diagnosed as having insulinoma during middle-age (30 - 49 yrs) tended to have a long period from appearance of hypoglycemic symptoms to diagnosis of the tumor. Of note, 13 patients (24%) were diagnosed as having insulinoma before 20 yrs of age. Such young onset was not seen in other GEPNETs. Since the development of GEPNETs during adolescence is quite rare, insulinoma diagnosed before 20 yrs strongly suggests the presence of MEN1 and warrants further investigation, including MEN1 genetic testing. Also, clinicians should be aware that insulinoma can often be missed in middle-aged patients.

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