MEN Flashcards

1
Q

What are the three areas involved with MEN 1?

A
  • Pituitary adenomas
  • Parathyroid tumors (most common)
  • Pancreatic Endocrine Tumors
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2
Q

Hypersecretion of acid from the stomach and Ulcers is associated with what MEN?

A
  • Wermer Syndrome (MEN 1)

- pancreatic tumor secreting gastrin (aka Zollinger-Ellison syndrome)

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3
Q

What shape does MEN 1 look like?

A

-regular diamond

think about gland involvement

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4
Q

What MEN syndromes involve the ret gene?

A

only MEN 2A and 2B

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5
Q

Describe the ret gene and its involvement in embryology.

A
  • expressed in neural crest cells

- therefore, mutations in it can affect things with neural crest origin

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6
Q

MEN 2A involves what spots?

A
  • Thyroid (medullary carcinoma)
  • Parathyroid tumors
  • Pheochromocytoma
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7
Q

Medullary carcinomas of the thyroid gland originate from what type of cell?

A
  • C cells (parafollicular cells) of the thyroid

- they secrete calcitonin

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8
Q

What types of MEN are associated with amyloidosis?

A
  • MEN 2A and 2B

- due to the association with medullary CA of thyroid

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9
Q

Describe the pathogenesis of amyloid deposition in medullary thyroid carcinoma.

A
  • proliferation of C cells makes tons of Calcitonin

- calcitonin can aggregate as amyloid

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10
Q

What type of secondary structure does amyloid present?

A

-Beta pleated sheets

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11
Q

How does amyloid stain?

A

-Congo red stain reveals apple green birefringence under polarized light

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12
Q

What shape should you think of with Sipple disease?

A
  • MEN 2A

- home plate

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13
Q

How many P’s do each MEN have?

A
  • MEN 1: 3 Ps
  • MEN 2A: 2 Ps
  • MEN 2B: 1 P
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14
Q

MEN 2B is shaped how?

A

-tall and skinny diamond

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15
Q

What organs are involved in MEN 2B?

A
  • thyroid (medullary CA)
  • Pheochromocytomas
  • Ganglioneuromas (colon/oral)
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16
Q

Which MEN syndrome is associated with Marfan syndrome?

A

-MEN 2B

17
Q

How are the MEN syndromes passed down?

A

Autosomal dominant