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Flashcards in multiple endocrine noeplasia Deck (15):
1

MEN-1: what diseases?

parathyroid adenomas most common
pancreatic islet and other GI problems (gastrinoma, insulinoma, glucagonooma, VIPoma, PPoma, somatostatinomoa)
Pituitary neoplasias (esp. prolactinomas)
autosomal dominant mutation of menin tumor suppressor

2

What is a gastroma? What should I know about MEN-1 gastromas? how do you test? other symtpoms?

most common islet cell tumor
aka zollinger ellison
most MEN-1 gastrinomas are malignant and are often multiple
cause raised fasting gastrin and icnreased basal gastric acid secretion
test with secretin
often see ulcers and diarrhea

3

Tx of gastroma

resection, H2 blockers, PPI

4

What features are seen with insulinoma?

hypoglycemai with incr. C peptide/ insulin, and proinsulin

5

Features of glucagonoma

necrolytic migratory erythema
hyperglycemia

6

VIPoma

watery diarrhea, hypokalemia, achlorhydria

7

features of somatostatinoma

hyperglycemia, gallstones, steatorrhea, low acid output (inhibits glucugon and insulin, but insulin more than glucugon, so you get the hyperglycemia)

8

Screening in a pt with MEN-1

calcium and PTH, prolactin, and gastrin screening

9

MEN 2a

medullary thyroid cancer: 95%
pheochromocytoma: 60%
parathyroid
gain of functiuon ret tyrosine kinase gene mutation

10

variants of MEN-2a

1. cutaneous liche amyloidosis: causes pruritis, upper back, and amyloid deposits: dark spots on back
2. Hirschprung's syndrome

11

Hereditary Pheochromoctyomas

Von hippel lindau
MEN-2: often bilateral
Neurfibromatosis I
hereditary paraganglioma syndromes

12

MEN-2b

medullary thyroid carcinoma
pheochromocytoma
oral mucosal neuromas and intestinal ganglioneuromatosis
associated with gain of function tyrosine kinase ret mutation

13

hereditary paraganglioma syndromes

SDH B, C, and D
D is maternally imprinted, so transmitted exclusively by dad. foten at skull and base of neck.
B and C are autosomal dominant

14

screening in pts with MEN2

look for medullary thyroid carcionma
look for pheos with fractionated catecholamines
look for hyperparathyroidism by looking at serum calcium

15

treatment for MEN2

thyroidectomy if pt has MEN 2
parathyroids: 3 and a half gland removal