PMID- 22785103
OWN - NLM
STAT- MEDLINE
DCOM- 20130415
LR  - 20220331
IS  - 1348-4540 (Electronic)
IS  - 0918-8959 (Linking)
VI  - 59
IP  - 10
DP  - 2012
TI  - Clinical features of insulinoma in patients with multiple endocrine neoplasia 
      type 1: analysis of the database of the MEN Consortium of Japan.
PG  - 859-66
AB  - 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.
FAU - Sakurai, Akihiro
AU  - Sakurai A
AD  - Department of Medical Genetics, Shinshu University School of Medicine, Matsumoto 
      390-8621, Japan. aksakura@shinshu-u.ac.jp
FAU - Yamazaki, Masanori
AU  - Yamazaki M
FAU - Suzuki, Shinichi
AU  - Suzuki S
FAU - Fukushima, Toshihiko
AU  - Fukushima T
FAU - Imai, Tsuneo
AU  - Imai T
FAU - Kikumori, Toyone
AU  - Kikumori T
FAU - Okamoto, Takahiro
AU  - Okamoto T
FAU - Horiuchi, Kiyomi
AU  - Horiuchi K
FAU - Uchino, Shinya
AU  - Uchino S
FAU - Kosugi, Shinji
AU  - Kosugi S
FAU - Yamada, Masanobu
AU  - Yamada M
FAU - Komoto, Izumi
AU  - Komoto I
FAU - Hanazaki, Kazuhiro
AU  - Hanazaki K
FAU - Itoh, Masatsune
AU  - Itoh M
FAU - Kondo, Tatsuya
AU  - Kondo T
FAU - Mihara, Masatomo
AU  - Mihara M
FAU - Imamura, Masayuki
AU  - Imamura M
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20120616
PL  - Japan
TA  - Endocr J
JT  - Endocrine journal
JID - 9313485
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age of Onset
MH  - Aged
MH  - Child
MH  - Databases, Factual
MH  - Female
MH  - Humans
MH  - Insulinoma/*epidemiology/etiology/pathology
MH  - Japan/epidemiology
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 
      1/*complications/epidemiology/genetics/*pathology
MH  - Neuroendocrine Tumors/genetics
MH  - Pancreatic Neoplasms/genetics/pathology
EDAT- 2012/07/13 06:00
MHDA- 2013/04/16 06:00
CRDT- 2012/07/13 06:00
PHST- 2012/07/13 06:00 [entrez]
PHST- 2012/07/13 06:00 [pubmed]
PHST- 2013/04/16 06:00 [medline]
AID - DN/JST.JSTAGE/endocrj/EJ12-0173 [pii]
AID - 10.1507/endocrj.ej12-0173 [doi]
PST - ppublish
SO  - Endocr J. 2012;59(10):859-66. doi: 10.1507/endocrj.ej12-0173. Epub 2012 Jun 16.