MEN Syndromes

Question 1

Define what a men syndrome is?

Answer 1

Inherited disease resulting in proliferative lesions (hyperplasia, adenomas, and carcinomas) of multiple endocrine organs

Question 2

5 generalities of MEN syndromes?

Answer 2

1. Tumors present at younger age
2. Multiple endocrine organs are involved
3. Bilateral
4. Hyperplasia is seen first usually
5. Aggressive

Question 3

What are the 3 classic tumors making up MEN type 1 and what is the additional tumor they have recently added? What is the mutations for MEN 1 syndrome?

Answer 3

Primary hyperparathyroidism, pancreatic endocrine tumors, and pituitary adenomam (specially lactotroph and somatotroph)

The additional ones are gastrinomas found in the duodenum.

Men 1 tumor suppressor gene

Question 4

What 3 tumors are found in MEN type 2A and what is the mutation?

Answer 4

Pheo, medullary thyroid carcinoma and parathyroid hyperplasia.
RET

Question 5

What are the 3 types of tumors in MEN type 2B and what is the mutation?

Answer 5

Pheo, medullary thyroid carcinoma, and mucosal neuromas.

Specific point mutation of RET

Question 6

Explain what familial medullary thyroid carcinoma is?

Answer 6

Variant of MEN 2a, but only with the medullary thyroid carcinoma.

Question 7

What is the function of the pineal gland?

Answer 7

Secrete melatonin.

Question 8

What are the three most common tumors of the pineal gland?

Answer 8

Germ cell tumors, pineocytomas and pineoblastomas