Multiple Endocrine Neoplasia

Question 1

Where are the main mass’s in MEN-1 (AKA Wermer)?

Answer 1

Parathyroid adenoma

(neck)

Question 2

Where are the main mass’s in MEN-2a (AKA Sipple)?

Answer 2

Neck

Medullary thyroid carcinoma

Question 3

Where are the main mass’s in MEN-2b?

Answer 3

Mouth + Neck

Mucosal Neuromas (mouth)

Medullary carcinoma of the thyroid (neck)

Question 4

What symptoms are associated with MEN 1?

Answer 4

Hyperparathyroidism

Endopancreatic tumors (neoplastic pancreatic beta cells)(gastrinoma –> ZE)

Pituitary adenomas (hyperprolactemia)

Question 5

What symptoms are associates with MEN 2A?

Answer 5

Medullary thyroid carcinoma (malignant transformation of parafollicular C cells)

Pheochromocytomas (adrenal medulla)

Hyperparathyroidism

Question 6

What symptoms are associated with MEN 2B?

Answer 6

Mucosal neuromas

Marfanoid habitus

Pheochromocytoma

Question 7

What tumors are in MEN 1 (acronym)

Answer 7

3 P’s: pituitary, parathyroid, pancreas

*has to occur in at least 2*

Question 8

What are the 2 P’s in MEN 2a?

Answer 8

Parathyroid hyperplasia

Pheochromocytoma

Question 9

What is the one P in MEN 2b?

Answer 9

Pheochromocytoma (adrenal medulla)

Question 10

How can so many various organs be affected in these syndromes?

Answer 10

The cells that compromise many endocrine organs come from “APUD” cells (amine precursor and decraboxylation. These cells are neuroectodemal in orgin and when they get mutated and migrate to their specific developing organs, the genetic mutation is distributed to those organs.