PMID- 11216636
OWN - NLM
STAT- MEDLINE
DCOM- 20010405
LR  - 20181113
IS  - 1355-008X (Print)
IS  - 1355-008X (Linking)
VI  - 13
IP  - 3
DP  - 2000 Dec
TI  - Large and asymptomatic pancreatic islet cell tumor in a patient with multiple 
      endocrine neoplasia type 1.
PG  - 263-6
AB  - The major phenotypes of multiple endocrine neoplasia type 1 (MEN 1) consist of 
      three lesions characterized by hyperparathyroidism, pituitary tumors, and 
      endocrine pancreatic tumors. The endocrine pancreatic tumors are a significant 
      cause of disease-related mortality in MEN 1. Although symptomatic pancreatic 
      tumors such as insulinoma and gastrinoma should be resected, the management of 
      asymptomatic pancreatic tumors is not established. In asymptomatic pancreatic 
      tumors, the most important factor is the propensity for malignant transformation 
      of the tumors. Although there are no means to foresee it, the size of the 
      pancreatic tumors might be predictive of malignant development in MEN 1. We 
      report here a patient with MEN 1 who had a large asymptomatic pancreatic tumor. 
      The patient (72-yr-old man) was diagnosed with primary hyperparathyroidism and 
      underwent a total parathyroidectomy. Genetic examination showed a germline 
      mutation of the MEN1 gene (E45G). Abdominal magnetic resonance imaging revealed a 
      large (>6 cm) tumor with a heterogeneous pattern in the tail of the pancreas. No 
      metastases of the tumor were evident. Serum levels of insulin, gastrin, and 
      glucagon were normal, and the patient had no symptoms. Operative resection was 
      performed, and microscopic examination revealed that the tumor was an islet cell 
      tumor stained with multiple hormones. This is a case indicating that asymptomatic 
      pancreatic tumors associated with MEN 1 might be indolent independent of their 
      size.
FAU - Sato, M
AU  - Sato M
AD  - First Department of Internal Medicine, Kagawa Medical University, Japan. 
      makoto@kms.ac.jp
FAU - Kihara, M
AU  - Kihara M
FAU - Nishitani, A
AU  - Nishitani A
FAU - Murao, K
AU  - Murao K
FAU - Kobayashi, S
AU  - Kobayashi S
FAU - Miyauchi, A
AU  - Miyauchi A
FAU - Takahara, J
AU  - Takahara J
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Endocrine
JT  - Endocrine
JID - 9434444
RN  - 0 (Gastrins)
RN  - 0 (Insulin)
RN  - 333DO1RDJY (Serotonin)
RN  - 9007-92-5 (Glucagon)
SB  - IM
MH  - Adenoma, Islet Cell/*diagnosis/genetics/surgery
MH  - Aged
MH  - Angiography
MH  - Gastrins/analysis
MH  - Germ-Line Mutation
MH  - Glucagon/analysis
MH  - Humans
MH  - Hyperparathyroidism/diagnosis/surgery
MH  - Immunohistochemistry
MH  - Insulin/analysis
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/*genetics
MH  - Pancreatic Neoplasms/*diagnosis/genetics/surgery
MH  - Parathyroidectomy
MH  - Serotonin/analysis
MH  - Spleen/blood supply
EDAT- 2001/02/24 12:00
MHDA- 2001/04/06 10:01
CRDT- 2001/02/24 12:00
PHST- 2000/05/03 00:00 [received]
PHST- 2000/06/05 00:00 [revised]
PHST- 2000/06/05 00:00 [accepted]
PHST- 2001/02/24 12:00 [pubmed]
PHST- 2001/04/06 10:01 [medline]
PHST- 2001/02/24 12:00 [entrez]
AID - ENDO:13:3:263 [pii]
AID - 10.1385/ENDO:13:3:263 [doi]
PST - ppublish
SO  - Endocrine. 2000 Dec;13(3):263-6. doi: 10.1385/ENDO:13:3:263.