Random Medical Fact #30: Multiple Endocrine Neoplasia type 1 (MEN1)
01/08/08 17:00
MEN1 is a rare, heritable (autosomal dominant)
disorder, with a prevalence of approximately
2/100,000 people.
It is classically associated with a classic triad of tumors (parathyroid glands, anterior pituitary gland, and pancreatic islet cells) but has been expanded to include tumors of other organs as well (duodenum, thymus, lung (bronchial carcinoid), stomach, adrenal glands). Around 40% of patients also have Zollinger-Ellison syndrome or asymptomatic elevation in serum gastrin concentrations.
The initial presentation is most commonly primary hyperthyroidism (1-2% of all cases are due to MEN1). Treatment is variable and dependent on pathophysiology (similar to isolated tumor therapy). It also depends on the symptoms and severity (can be surgical or medical).
It is classically associated with a classic triad of tumors (parathyroid glands, anterior pituitary gland, and pancreatic islet cells) but has been expanded to include tumors of other organs as well (duodenum, thymus, lung (bronchial carcinoid), stomach, adrenal glands). Around 40% of patients also have Zollinger-Ellison syndrome or asymptomatic elevation in serum gastrin concentrations.
The initial presentation is most commonly primary hyperthyroidism (1-2% of all cases are due to MEN1). Treatment is variable and dependent on pathophysiology (similar to isolated tumor therapy). It also depends on the symptoms and severity (can be surgical or medical).
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