Multiple endocrine neoplasia (MEN) is a group of disorders that affect the body’s hormone-producing glands. MEN type 1 usually affects the parathyroid glands causing hyperparathyroidism, the pituitary gland, and the pancreas. Men type 2A can cause medullary thyroid cancer, hyperparathyroidism, and pheochromocytoma. Men type 2B is quite rare and can cause medullary thyroid cancer, pheochromocytoma, and mucosal neuromas (bumps on the lips and tongue).
Approximately 1/3 of pheochromocytoma cases are now known to be genetic, which means that they have a hereditary or familial association. The most common associated genetic conditions are Multiple Endocrine Neoplasia (MEN) type 2 and von Hippel-Lindau Syndrome, followed by Neurofibromatosis type 1 and Familial Paraganglioma Syndrome (which is caused by mutations in the succinate dehydrogenase gene). We recommend genetic counseling for all patients with pheochromocytoma or paraganglioma, as this can provide important information for the patient’s prognosis and earlier screening for family members.
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