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Endocrine > MEN > Flashcards

Flashcards in MEN Deck (26):
1

MEN definition

occurrence of tumors involving 2 or more endocrine glands

2

MEN inheritance pattern

AD or sporadic 

3

Tumors and findings in MEN syndrome (6)

Parathyroid adenomas

Pheochromocytoma

Enteropancreatic tumors

Pituitary adenomas

Medulalry thyroid cancer

Marfarnoid habitus

4

Pituitary adenomas secrete

ACTH

Prolactin

Growth Hormone

LH/FSH

TSH

(from anterior pituitary)

5

Medullary thyroid cancer tumors secrete

C cells > calcitonin

6

marfanoid habitus =

low upper body / lower body ration

abnl posterior position of manible

low head width and length

(no ectopic lens or aortic abnls as in marfans)

7

parathyroid adenomas lead to

high serum Ca

low serum Phos

non suppressed or elevated PTH

8

pheochromocytomas secrete

 

arise from

secrete catecholamines

 

arise from chromaffin cells of adrenal medulla

9

presentation pheochromocytoma

HTN

headaches

palpitations

diaphoresis

10

examples of enteropancreatic tumors

gastrinoma

insulinmoma

glucagonoma

nonfunctioning pancreatic tumors

11

mutation and Tumors of MEN1

 

MEN1 = 11q13

 

Parathyroid adenoma (Same as MEN4)

Pituitary adenoma (Same as MEN4)

Enteropancreatic tumors

12

mutation and tumors of MEN2A

MEN2 A = 11cen-10q11.2

 

Medullary thyroid cancer (Same as MEN2B)

Pheochromocytoma (Same as MEN2B)

Parathyroid hyperplasia (10-20%)

13

mutation and tumors of MEN 2B

MEN2B = 110cen-10q11.2

 

Medullary Thyroid Cancer (Same as MEN2 B)

Pheochromocytoma (Same as MEN2A)

Mucosal Neuromas

Marafanoid Habitus

14

mutation medullary thyroid cancer (solo)

10cen-10q11.2

15

mutation and tumors of MEN4

MEN4 = 12p14

Parathyroid adenoma (same as MEN1)

Pituitary adenoma (Same as MEN1)

Preproductive organ tumors

16

tumors shared by MEN1, MEN4

 

difference?

Parathyroid adenoma

Pituitary adenoma

MEN1 has enteropancreatic

MEN4 has reporductive organ tumors

17

tumors shared by MEN2A, MEN2B

 

DIfference?

Medullary Thyroid cancer

Pheochromocytoma

MEN2A has parathyroid hyperplasia

MEN2B has Mucosal neuromas and marfanoid habitus

 

18

3 methods of MEN1 dx

Clincial - patient with 2+ MEN1 tumors

 

Familial - tumor and first degree relative with MEN1

 

Genetic - MEN-1 mutation without manifestations, but mutant carrier

19

MEN1 gene + characteristic

11a13

regulated trnascription, genome stability, cell division and proliferation

 

most germline mutations are inactivating

20

MEN1 patients typically present with

Hypercalcemia

primary hyperparathyroidism 

21

MEN1 germline testing for

MEN1 pateints

first degree relatives (whether or not symptomatic) 

22

further testing protocol for index patient without MEN1 germinline mutation

identify first degree family members via chemistries:

Calcium, PTH

Gatrin

fasting glucose and insulin

prolactin

IGF-1

pancreases imaging

23

Mutations of MEN2 and characteristic

RET protoonco gene (10cen-10q11.2)

(RET REarranged during Transfection) 

24

mutations in ___ predict disease presentation

MEN2

25

Consider ____ in younger patients presenting with primary hyperparathyroid (most will not have it)

MEN1

26

all patients with medulalry thyroid cancer should have ___ screening

RET proto oncogene screening