MEN Syndromes

Question 1

In ridiculously simplified terms, which MEN involves the thyroid? Which involves other endocrine organs?

Answer 1

MEN2 involves the thyroid

MEN 1 involves other endocrine organs

Question 2

What are the three main manifestations of MEN 1? Which is most common?

Answer 2

THE THREE Ps

parathyroid hyperplasia (most common)

pancreatic endocrine tumors

pituitary adenoma

Question 3

How will the parathyroid hyperplasia present?

Answer 3

hypercalcemia

Question 4

What are the two pancreatic endocrine tumors that will typically occur in MEN 1?

Answer 4

gastrinoma

insulinoma

Question 5

True or false: pancreatic endocrine tumors are usually metastatic at diagnosis.

Answer 5

true

Question 6

What kind of pituitary adenoma usually occurs in MEN 1?

Answer 6

prolactinoma

Question 7

Besides those three main manifestations, what else can MEN 1 cause?

Answer 7

duodenal gastrinoma
carcinoid syndrome
thyroid and adrenal adenomas
lipomas

Question 8

What are the genetics behind MEN 1?

Answer 8

It’s a MEN 1 gene mutation, which is a tumor suppressor gene that encodes menin

the mutation turns off the gene

Question 9

What does Brad Pitt have to do with MEN1?

Answer 9

He’s the #1 sexiest man in the world (per most)

It causes PITT-uitary adenoma (and the other Ps)

The MEN1 gene is the one that’s a tumor suppressor gene, so it gets inactivate (at the time he was a run of the mill actor, and Krafts finds him a turnoff)

Question 10

What are the three main manifestations of MEN 2a?

Answer 10

medullary thyroid carcinoma
pheochromocytoma
parathyroid hyperplasia

Question 11

True or false: virtually all patients with MEN 2 have medullary thyroid carcinoma.

Answer 11

true

so they can take out their thyroid and basically avoid all the badness form this syndrome.

note - it’s agressive in this context, often multifocal, and often associated with C-cell hyperplasia as a precursor

Question 12

What cells are hyperplasic in medullary thyroid carcinoma?

Answer 12

C cells (the ones that release epinephrine and noepinrphrine)

Question 13

What proportion of patient’s with MEN 2a will have pheochromocytoma?

Answer 13

about half of them

Question 14

True or false: the pheochromocytomas of MEN 2a can be bilateral. Extra-adrenal?

Answer 14

true and true

remember this is the 10% tumor - more likely in MEN. it still could be benign though

Question 15

What proportion of patients with MEN 2a will have parathyroid hyperplasia?

Answer 15

10-20%

Question 16

What gene is mutated in MEN 2a?

Answer 16

it’s a RET gene mutaiton

this is a proto-oncogene which is turned on by the mutations

encodes a tyrosine kinase receptor

Question 17

How is MEN 2B similar to 2A?

Answer 17

1. associated with medullary thyroid carcinoma
2. associated with pheochromocytoma
3. RET gene mutation

Question 18

How is MEN 2B different from 2A?

Answer 18

1. no parathyroid hyperplasia
2. associated with neuromas
3. Marfanoid habitus

Question 19

What does John Cleese have to do with MEN 2?

Answer 19

He’s maybe the second sexiest man (on average)

Cleese-cell hyperplasia (C cell hyperplasia = medullary thyroid carcinoma)

bRETon gene (he’s british)

he’s also one of a kine and always turned on (super funny)

Question 20

Is it better to have a tumor in the context of MEN syndrome or sporadic occurrence/

Answer 20

sporadic

MEN tumors happen in younger people, in multiple organs and are more aggressive