MEN Flashcards

1
Q

chromosome mutated in MEA I

A
  • chromosome 11
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2
Q

chromosome mutated in MEA II (A and B)

A
  • chromosome 10
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3
Q

mutation theory

A
  • 2 “hits” required
  • first in germ cell line, second in somatic cell line
  • first hit causes hyperplasia
  • second hit causes tumor development
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4
Q

glandular involvement of MEA 1

A
  • 3 Ps
  • anterior pituitary
  • parathyroid
  • pancreas
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5
Q

parathyroid involvement in MEA 1

A
  • most common gland involved in MEA 1
  • hyperparathyroidism leads to hypercalcemia in 70%
  • primary lesion is an adenoma
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6
Q

pancreatic cell types involved in MEA 1

A
  • hyperplasia, carcinoma, adenoma
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7
Q

symptoms of pancreatic involvement in MEA 1

A
  • PUD is most common: multiple ulcers associated with ZES
  • hypoglycemia: insulin secreting tumors
  • hyperglycemia, dermatitis, anemia, wt loss: glucagonoma
  • watery diarrhea and hypokalemia: VIPoma
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8
Q

anterior pituitary cell types involved in MEA 1

A
  • adenoma
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9
Q

symptoms of anterior pituitary involvement in MEA

A
  • Cushing’s syndrome: excessive weight gain, bruises easily, slow healing of cuts, acne
  • acromegaly
  • hyperprolactinemia
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10
Q

screening for MEA 1

A
  • prolactin
  • calcium
  • PTH
  • phosphate
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11
Q

thyroid involvement in MEA IIA

A
  • bilateral
  • multicentric
  • diffuse or nodular hyperplasia of C-cells
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12
Q

screening for MEA IIA

A
  • calcitonin
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13
Q

treatment of thyroid carcinoma

A
  • total thryoidectomy is best in patient and in family
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14
Q

parathyroid involvement in MEA IIA

A
  • very low incidence of hyperparathyoidism
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15
Q

adrenal involvement in MEA IIA

A
  • can lead to hypertensive crisis and cardiac dysrythmia
  • treated with bilateral adrenalectomy
  • pheochromocytoma - second most common lesion in MEA IIA, usually bilateral
  • NO SCREENING TEST AVAILABLE
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16
Q

thyroid involvement in MEA IIB

A
  • medullary carcinoma (C-cell)
17
Q

distinctive features of MEA IIB

A
  • marfanoid habitus

- multiple ganglioneuromas - ocular, mucocutaneous, gastrointestinal

18
Q

GI involvement of MEA IIB

A
  • multiple neuromatous lesions
  • occur anywhere in GI tract
  • present at birth
  • may lead to megacolon