MULTIPLE ENDOCRINE NEOPLASIA Flashcards

1
Q

what is multiple endocrine neoplasias (MEN) also known as?

A

familial endocrine adenomatosis

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2
Q

what is multiple endocrine neoplasias (MEN)?

A

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

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3
Q

what are the types of multiple endocrine neoplasias (MEN)?

A
  1. MEN 1
  2. MEN 2A
  3. MEN 2B
  4. MEN 4
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4
Q

what is the definition of MEN 1 and what glands does it affect?

A

development of tumors, or excessive growth and activity, of 2 or more of the following glands:

  1. parathyroid glands
  2. pancreas
  3. pituitary gland
  4. thyroid gland
  5. adrenal glands
    these 2 are less often affected
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5
Q

almost all patients have tumors in what gland in MEN 1?

A

parathyroid glands

most tumors non-cancerous

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6
Q

what is the first clinical manifestation of a patient who has parathyroid gland tumor in MEN 1?

A

hyperparathyroidism (kidney stones)

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7
Q

what are some other common tumors seen in MEN 1?

A
  1. gastrinomas
  2. insulinomas
  3. pituitary adenomas
  4. lipomas and facial angiofibromas (non-endocrine)
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8
Q

what is the pathophys of a gastrinoma?

A

secrete excessive amoutns of gastrin that stimulates overproduction of of stomach acid (peptic ulcers w/ bleeding/perforation)

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9
Q

what is the pathophys of a insulinoma?

A

secrete insulin (hypoglycemia)
tumor with the highest frequency for malignancy

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10
Q

what is the definition of a MEN 2A tumor and what does it affect?

A

development of tumors or excessive growth and activity in 2 or 3 of the following glands:
1. thyroid
2. adrenal (“TAP”)
3. parathyroid

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11
Q

what type of cancer do almost everyone with MEN 2A develop?

A

medullary thyroid cancer

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11
Q

what should patients with MEN and diabetes NOT receieve therapy with?

A

glucagon- like peptide 1 (GLP- 1) agonist because it may increase risk for development of medullary thyroid cancer

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12
Q

what happens with overactive parathyroid glands in MEN 2A?

A

hypercalcemia (kidney stones)

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13
Q

what another HUGE common tumors in MEN 2A?

A

Pheochromocytoma!

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14
Q

what is a pheochromocytoma?

A

a neuroendocrine tumor of the adrenal glands that secretes catecholamine hormones leading to increased adrenaline (high BP, tachycardia, HA, sweating)

often bilat. and noncancerous

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15
Q

what is MEN 2B associated with?

A

marfan-like habitus

(men want 2b tall)

16
Q

what cancer is MEN 2B the develpoment of?

A
  1. medullary thyroid cancer that develops at an early age, grows faster, and spreads more rapidly than those in MEN 2A
  2. pheochromocytoma
  3. neuromas (mucus membranes)
17
Q

what are neuromas?

A

glistening bumps around the lips, tongue and lining of mouth
eyelids and lips ay thicken and the lips may turn inside out (evert)

18
Q

which MEN type is the most recently discovered type?

A

MEN 4

19
Q

what does MEN 4 resemble?

A

MEN 1, but is caused by a different gene mutation

20
Q

what cancers develop through MEN 4?

A
  1. parathyroid adenoma -> hyperparathyroidism
  2. pituitary adenoma
  3. pancreatic neuroendocrine tumor
  4. gonadal, renal, and/or adrenal tumors
21
Q

what is involved in the diagnosis of MEN?

A
  1. genetic testing- pt. and family members
  2. hormone levels (blood and urine)
  3. imaging studies to access tumors
22
Q

what is the treatment for MEN?

A

no cure for any type
tumor removal if possible
total thyroidectomy for all those at risk for medullary thyroid cancer

23
Q

what is the MEN mnemonic?

A

men 1: 3 P’s
men 2a: 1 M 2 P’s
men 2b: 2M’s 1 P

24
Q

go back to notes

A

:) fuck you