Multiple Endocrine Neoplasia

Question 1

What are multiple endocrine neoplasia (MEN) syndromes?

Answer 1

3 well characterized, inherited pluriglandular disorders in which several endocrine glands simultaneously undergo neoplasic transformation and become hyperfunctional.

Question 2

How are MEN syndromes genetically transmitted?

Answer 2

autosomal dominant

Question 3

What are the types of MEN syndromes?

Answer 3

MEN-1, MEN-2A, and MEN-2B

Question 4

What is MEN-1?

Answer 4

hyperplasia or neoplastic transformation of the parathyroid glands, pancreatic islets, and pituitary

Question 5

What is MEN-2A?

Answer 5

hyperplasia or neoplastic transformation of the thyroid parafollicular cells (medullary thyroid carcinoma [MTC]), parathyroid glands, and adrenal medulla (pheochromocytoma)

Question 6

What is MEN-2B?

Answer 6

hyperplasia or neoplastic transformation of the thyroid parafollicular cells (MTC) and adrenal medulla (pheochromocytoma) with concomitant development of mucosal neuromas

Question 7

What is Wermer syndrome?

Answer 7

another name for MEN-1

Question 8

What is the prevalence of MEN syndromes?

Answer 8

MEN1 - 2-20/100,000
MEN2- less common
type 2A - more common form
type 2B - 5% of all type 2

Question 9

What cells do MEN tumors arise from?

Answer 9

amine precursor uptake and decarboxylation (APUD) group

Question 10

What tumors are associated with MEN1?

Answer 10

parathyroid adenoma 95%
pancreatic endocrine tumors
pituitary adenoma
carcinoid tumors

Question 11

What tumors are associated with MEN2A?

Answer 11

medullary thyroid carcinoma 100%
bilateral phaeochromocytoma
parathyroid adenoma

Question 12

What tumors are associated with MEN2B?

Answer 12

neuromas 100%
medullary carcinoma of the thyroid 100%
phaeochromocytoma

Question 13

Which syndrome is associated with ZE syndrome?

Answer 13

MEN1

Question 14

What is MEN1 caused by?

Answer 14

mutation in MEN1 gene –> menin protein (tumor supressor)

Question 15

What are the locations of MEN1 carcinoid tumors?

Answer 15

men: thymus
women: bronchial carcinoids

Question 16

What are the diagnosis requirements for MEN1?

Answer 16

• clinical: 2 or more MEN1 associated tumors
• familial: pt with one MEN1 associated tumor and a 1st degree relative with MEN1
• genetic: asymptomatic carrier of MEN1 mutation

Question 17

What is the most frequently involved organ of MEN1?

Answer 17

parathyroid gland 95%

Question 18

What is usually the first manifestation of MEN1?

Answer 18

hyperparathyroidism from hyperplasia of all 4 glands

Question 19

What is the second most common manifestation of MEN1?

Answer 19

neoplastic transformation of pancreatic islet cells

• gastrinoma
• insulinoma
• glucagonomas, somatostatinomas, and vasoactive intestinal polypeptide secreting tumors (VIPomas)

Question 20

What do most pituitary tumors in MEN1 secrete?

-symptoms

Answer 20

prolactin

• galactorrhea
• amenorrhea
• impotence

Question 21

What is treatment for MEN1?

Answer 21

• surgical resection of hyperplastic parathyroid tissue
• surgical resection of pituitary adenomas or pharmacological management (bromocriptine for prolactinomas and octreotide for acromegaly)
• subtotal pancreatectomy

Question 22

MEN2 cause

Answer 22

activating mutation of the RET protooncogene –> receptor tyrosine kinase for cellular development

Question 23

What is the most common sign of MEN2? Another sign?

Answer 23

malignant transformation of parafollicular c cells = medullary thyroid carcinoma (MTC)
50% develop pheochromocytoma

Question 24

Which is more severe? MEN 2A or 2B?

Answer 24

2B - avg age of death 30 years

2A -avg age of death 60 years

Question 25

What is another name for MEN2A?

Answer 25

sipple syndrome

Question 26

What is an itchy skin condition? What MEN syndrome is this associated with?

Answer 26

cutaneous lichen amyloidosis; MEN2A

Question 27

What MEN syndromes have associated hyperparathyroidism?

Answer 27

1 and 2A

Question 28

What MEN syndrome has a marfanoid body habitus?

Answer 28

MEN2B