MULTIPLE ENDOCRINE NEOPLASIA Flashcards
what is multiple endocrine neoplasias (MEN) also known as?
familial endocrine adenomatosis
what is multiple endocrine neoplasias (MEN)?
rare group of AUTO DOM inherited syndromes that cause a predisposition to the development of hormone-producing tumors in 2 or more different endocrine glands
tumors may be benign or malignant
what are the types of multiple endocrine neoplasias (MEN)?
- MEN 1
- MEN 2A
- MEN 2B
- MEN 4
what is the definition of MEN 1 and what glands does it affect?
development of tumors, or excessive growth and activity, of 2 or more of the following glands:
- parathyroid glands
- pancreas
- pituitary gland
- thyroid gland
- adrenal glands
these 2 are less often affected
almost all patients have tumors in what gland in MEN 1?
parathyroid glands
most tumors non-cancerous
what is the first clinical manifestation of a patient who has parathyroid gland tumor in MEN 1?
hyperparathyroidism (kidney stones)
what are some other common tumors seen in MEN 1?
- gastrinomas
- insulinomas
- pituitary adenomas
- lipomas and facial angiofibromas (non-endocrine)
what is the pathophys of a gastrinoma?
secrete excessive amoutns of gastrin that stimulates overproduction of of stomach acid (peptic ulcers w/ bleeding/perforation)
what is the pathophys of a insulinoma?
secrete insulin (hypoglycemia)
tumor with the highest frequency for malignancy
what is the definition of a MEN 2A tumor and what does it affect?
development of tumors or excessive growth and activity in 2 or 3 of the following glands:
1. thyroid
2. adrenal (“TAP”)
3. parathyroid
what type of cancer do almost everyone with MEN 2A develop?
medullary thyroid cancer
what should patients with MEN and diabetes NOT receieve therapy with?
glucagon- like peptide 1 (GLP- 1) agonist because it may increase risk for development of medullary thyroid cancer
what happens with overactive parathyroid glands in MEN 2A?
hypercalcemia (kidney stones)
what another HUGE common tumors in MEN 2A?
Pheochromocytoma!
what is a pheochromocytoma?
a neuroendocrine tumor of the adrenal glands that secretes catecholamine hormones leading to increased adrenaline (high BP, tachycardia, HA, sweating)
often bilat. and noncancerous