Multiple Endocrine Neoplasia Type 1

Pathophysiology: MEN1 is an inherited disorder that causes tumors in the endocrine glands and the duodenum, the first part of the small intestine.  MEN1 is sometimes called multiple endocrine adenomatosis or Wermer's syndrome. MEN1 is rare, occurring in about one in 30,000 people.1  The disorder affects both sexes equally and shows no geographical, racial, or ethnic preferences. MEN1 is detected by gene testing or, when gene testing is unavailable or yields a negative result, by laboratory tests that measure hormone levels.

Statistics:
Patients with multiple endocrine neoplasia type 1 (MEN1) have a decreased life expectancy, with a 50% probability of death by age 50 years. Half the deaths result directly from a malignant process or the sequela of an endocrine disorder. Malignant pancreatic neuroendocrine tumors and thymic carcinoid tumors have been associated with a marked increase in the risk of death in MEN1 patients.

Treatment:
Treatment for MEN 1 depends on each person's situation and related conditions. In the early stages, specialists may recommend surgery to remove tumors as well as drug therapy to regulate hormones. If a tumor is cancerous and has spread, doctors may use surgery, radiation, chemotherapy and treatments such as radiofrequency ablation or chemoembolization.

Major Research:
Magnetic resonance imaging (MRI) scan of the brain is one of the more advanced procedure is used for diagnosis for every three to five years, beginning between ages 5 to 10, or at any time the results of the tests for serum prolactin or insulin-like growth factor is abnormal.