Multiple Endocrine Neoplasia Syndromes
Multiple endocrine neoplasias are familial syndromes of endocrine tumors occurring in endocrine organs throughout the body. Despite its acronym, the risk for acquiring MEN is about the same in men and women and also across geographic and racial and ethnic groups. These are rare syndromes, affecting about 1 in 30,000 people. Genetic mutations cause the MEN syndromes, which are then inherited in an autosomal dominant pattern. This means several members of the same family are often affected; an affected parent has a 50% chance of passing down the disease to his or her children. Unlike cancers without a hereditary basis, tumors associated with MEN often occur in younger patients (under age 40 years) and affect multiple organ systems all at once, making them particularly troublesome. Generally, tumors may arise in the pituitary gland, parathyroid glands, thymus, pancreas, thyroid, or adrenal glands (Figure 1). The three major types of multiple endocrine neoplasia are MEN1, MEN2A, and MEN2B, and each has a characteristic picture of endocrine or neurologic disorders and tumors.
After listening to this AudioBrick, you should be able to:
- Define multiple endocrine neoplasia (MEN), and list the three MEN syndromes.
- Compare and contrast the main clinical features of MEN1, MEN2A, and MEN2B.
- Describe the genetic defects that cause MEN1, MEN2A, and MEN2B.
- Briefly describe the management of the MEN syndromes.
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