ICEECE2012 Poster Presentations Endocrine tumours and neoplasia (112 abstracts)
Insulinoma in patients with multiple endocrine neoplasia type 1
A. Sakurai 1 , T. Imai 2 , T. Kikumori 2 , K. Horiuchi 3 , T. Okamoto 3 , S. Kosugi 4 , S. Suzuki 5 , S. Uchino 6 , M. Yamada 7 , T. Katabami 8 , T. Igarashi 9 , T. Iwatani 10 , A. Miya 11 , I. Komoto 12 , A. Miyauchi 11 & M. Imamura 12
Kansai Electric Power Company Hospital, Osaka, Japan.
More than half of patients with multiple endocrine neoplasia type 1 (MEN1) develop gastroenteropancreatic neuroendocrine tumors (GEPNETs). Among GEPNETs in MEN1, insulinoma is the second common functioning tumor and often diagnosed by its pathognomonic clinical feature, i.e. hypoglycemia. Compared to other functioning and nonfunctioning GEPNETs in MEN1, insulinoma is known to develop in younger age. We have recently constructed a database of Japanese patients with MEN1 and here report clinical features of patients with MEN1 who developed insulinoma. Among 582 registered patients, 560 cases were considered to be eligible for analysis. GEPNETs were seen in 314 patients and 69 patients had insulinoma. Tumors predominantly occurred in body and tail of pancreas. Among 54 patients whose age at diagnosis of insulinoma was recorded, only 11 patients (20.4%) had a single tumor at the diagnosis; that complicates surgical decision making. Surgery was performed for 51 patients and 6 patients needed multiple surgery. Age at diagnosis of insulinoma was 38.9±18.1 yrs for proband (including sporadic cases) and 30.9±13.9 yrs for family members. It is to be noted that 13 patients (24.1%) were diagnosed of insulinoma before 20 yrs of age. Five were probands and their parents were diagnosed as having MEN1 after diagnosis of their offsprings. Such young onset was not seen in other GEPNETs. According to recent epidemiological study which analyzed more than 3,000 cases with pancreas NET in Japan, only 1% of patients developed pancreas NETs at younger than 20 yrs. It also revealed that 10% of patients with pancreas NETs had MEN1. These results indicate that insulinoma diagnosed before 20 yrs strongly suggests the presence of MEN1 and warrants further investigation including MEN1 genetic testing.
Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.
Funding: This work was supported, however funding details unavailable.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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