Insulinoma and hypoglycaemia

2011 ◽  
pp. 914-919
Author(s):  
Puja Mehta ◽  
Jeannie F. Todd
Keyword(s):  
Islet Cell ◽  
Multiple Endocrine Neoplasia ◽  
Clinical Syndrome ◽  
Complete Resection ◽  
Pancreatic Islet Cell ◽  
Hyperinsulinaemic Hypoglycaemia ◽  
Endocrine Neoplasia ◽  
Calcium Stimulation ◽  
Surgical Cure

Hypoglycaemia is a clinical syndrome with diverse aetiology. Insulinomas, although rare, are the most common functioning pancreatic islet cell tumour and may be part of the multiple endocrine neoplasia type 1 (MEN 1) syndrome. Patients present with symptoms of neuroglycopenia and a catecholamine response. Diagnosis is confirmed by evidence of endogenous hyperinsulinaemic hypoglycaemia. Tumours are localized by ultrasonography, CT and/or intra-arterial calcium stimulation with venous sampling. Most tumours are benign and solitary, making surgical cure possible with complete resection. Medical options, including diazoxide or octreotide, are available for multifocal tumours.

Large and Asymptomatic Pancreatic Islet Cell Tumor in a Patient with Multiple Endocrine Neoplasia Type 1

Endocrine ◽  
2000 ◽  
Vol 13 (3) ◽  
pp. 263-266 ◽  
Author(s):  
Makoto Sato ◽  
Minoru Kihara ◽  
Akiko Nishitani ◽  
Koji Murao ◽  
Shoji Kobayashi ◽  
...  
Keyword(s):  
Pancreatic Islet ◽  
Islet Cell ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Pancreatic Islet Cell ◽  
Endocrine Neoplasia ◽  
Pancreatic Islet Cell Tumor

Cystic Insulinoma and Nonfunctioning Islet Cell Tumor in Multiple Endocrine Neoplasia Type 1

Pancreas ◽  
1994 ◽  
Vol 9 (3) ◽  
pp. 393-395 ◽  
Author(s):  
MASAYOSHI GOTO ◽  
ITSURO NAKANO ◽  
KAYO SUMI ◽  
HIROYA YAMAGUCHI ◽  
YASUHIRO SAKO ◽  
...  
Keyword(s):  
Islet Cell ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Endocrine Neoplasia ◽  
Endocrine Neoplasia Type ◽  
Nonfunctioning Islet Cell Tumor

MULTIPLE NONFUNCTIONAL PANCREATIC ISLET CELL TUMOR IN MULTIPLE ENDOCRINE NEOPLASIA TYPE I

1988 ◽  
Vol 38 (5) ◽  
pp. 667-682
Author(s):  
Hitoshi Takahashi ◽  
Kazuro Nakano ◽  
Yukihiko Adachi ◽  
Norihiko Aoki ◽  
Kiyoshi Hajiro ◽  
...  
Keyword(s):  
Pancreatic Islet ◽  
Islet Cell ◽  
Multiple Endocrine Neoplasia ◽  
Multiple Endocrine Neoplasia Type ◽  
Islet Cell Tumor ◽  
Cell Tumor ◽  
Type I ◽  
Pancreatic Islet Cell ◽  
Endocrine Neoplasia ◽  
Pancreatic Islet Cell Tumor

scholarly journals Do patients with multiple endocrine neoplasia syndrome type 1 benefit from periodical screening?

2003 ◽  
pp. 577-582 ◽  
Author(s):  
EA Geerdink ◽  
RB Van der Luijt ◽  
CJ Lips
Keyword(s):  
Multiple Endocrine Neoplasia ◽  
Premature Death ◽  
Age At Death ◽  
Pancreatic Islet Cell ◽  
Clinical Screening ◽  
Men 1 ◽  
Endocrine Neoplasia ◽  
Increased Risk ◽  
Design And Methods

OBJECTIVE: To determine the benefit of periodical clinical screening of carriers of a mutation in the multiple endocrine neoplasia type 1 (MEN-1) gene, because any useful discussion requires more concrete data. DESIGN AND METHODS: Our study population consisted of all the patients with MEN-1 (n=58) who were treated at the University Medical Centre Utrecht, The Netherlands, during the period 1975-2003, and their affected relatives (n=29). Records of affected individuals who died were analysed for morbidity, cause of death and age at death. We discuss our results in the light of the literature on MEN-1 regarding the benefit of screening. RESULTS: Over a period of 28 Years, we identified 87 individuals affected with MEN-1, from 16 families. A mutation in the MEN-1 gene was detected in 57%, 18% were obligate carriers, and in 24% the diagnosis was only clinically confirmed. Thirty individuals died, 17 from MEN-1-related causes, including malignancies (n=12: pancreatic islet cell tumours n=6 and carcinoid tumours n=6), the Zollinger-Ellison syndrome (n=4) and Cushing's disease (n=1). The remaining patients died of causes probably related to MEN-1 (n=3), unrelated to MEN-1 (n=7) or of unknown causes. Mean ages at death from MEN-1 were 55.4 Years for men and 46.8 Years for women, in both cases significantly lower than the mean age at death in the average Dutch population (P<0.05). CONCLUSIONS: We feel that the significantly increased risk of premature death found in patients with MEN-1 justifies the periodical clinical screening of carriers of the MEN-1 gene mutation. Early detection and treatment of abnormalities will probably reduce this risk.

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