Abstract
Multiple endocrine neoplasia type 1 (MEN-1) is a rare autosomal-dominant disease. It is associated with a broad range of endocrine tumours, most frequently arising in the parathyroid glands, the pituitary and the pancreas. Most neuroendocrine tumours will be diagnosed in the pancreas as non-functioning neuroendocrine tumours or insulinomas. Forty-two percent of the patients will develop a gastrin-secreting neuroendocrine tumour, a gastrinoma. Gastrinomas in MEN-1 tend to be small, multiple and preferentially located in the duodenum. This paper will focus on the specific characteristics of gastrinomas in the setting of MEN-1 compared to sporadic gastrinomas. The developments in understanding the tumorigenesis of these tumours and the consequences for diagnosis and therapy will be discussed.
Highlights
Multiple endocrine neoplasia type 1 (MEN-1) is a rare autosomal-dominant disease
In patients with Zollinger-Ellison syndrome with MEN-1 and hypercalcaemia omeprazole 40 mg b.i.d. or equivalent is recommended as a staring dose and followed by a reduction to 20 mg b.i.d. if possible
It has been demonstrated recently that gastrinomas associated with MEN-1 are preferentially located in the duodenum, arise from gastrin cell hyperplasia, so-called precursor lesions that develop to micro-tumours after loss of heterozygosity
Summary
Multiple endocrine neoplasia type 1 (MEN-1) is a rare autosomal-dominant disease. MEN-1 is associated with a broad range of endocrine tumours, most frequently arising in the parathyroid glands, the pituitary and the pancreas. Penetrance for neuroendocrine tumours is up to 70%–100%. Half of the patients with MEN-1 will present with a pancreatico-duodenal neuroendocrine tumour at the age of 50 years. Most will arise in the pancreas as non-functioning neuroendocrine tumours or insulinomas. In contrast to sporadic gastrinomas that occur predominantly in the pancreas, in MEN-1 most of these tumours reside in the duodenum [1]. Pancreatic gastrinomas associated with MEN-1 are very rare [2], as are other uncommon extra-pancreatic, extra-duodenal locations that have been associated with gastrinomas
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
