Path 9 MEN syndromes Flashcards Preview

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Flashcards in Path 9 MEN syndromes Deck (17):
1

Delta cells

Somatostatin - suppress insulin and glucagon release

2

D1 cells

VIP - induce glycogenolysis/hyperglycemia and GI fluid secretion

3

Pancreatic enterochromaffin cells

Serotonin

4

Insulinoma

Most common pancreatic endocrine neoplasm
-90% benign

5

Insulinoma findings

Whipple's triad: Hypoglycemia, CNS symptoms, reversal by glucose administration

6

Insulinoma histo

Enlarged islet
-No anaplasia
-Amyloid deposition

7

2 tumors with amyloid deposition

Insulinoma
Medullary thyroid carcinoma (calcitonin)

8

Gastrinoma

Zollinger-Ellison Syndrome
-Gastrinoma triangle (mostly duodenum)
-Multiple peptic ulcers
-Usually metastatic to liver

9

Glucagonoma

a-Cell tumor
-Mild diabetes (hyperglycemia)
-Necrolytic migratory erythema
-Anemia

10

VIPoma

WDHA (pancreatic cholera) syndrome
-Watery Diarrhea, Hypokalemia, Achlorhydria

11

MEN type 1

Wermer syndrome
-3 Ps (Parathyroid, Pituitary, Pancreas)

12

MEN type 1 more specific

-Parathyroid hyperplasia/adenomas
-Pituitary Prolactin or GH adenomas
-Pancreas Gastrinomas and Insulinomas

13

MEN1 gene

Tumor suppressor gene
-Product is menin, cell cycle regulator

14

MEN type 2a

Sipple syndrome
-Familial Medullary Thyroid Cancer (slow)
-Pheochromocytomas
-Parathyroid hyperplasia

15

MEN2a/b gene

RET proto-oncogene mutation

16

RET gain of function v loss of function

MEN2 v Hirschsprung

17

MEN type 2b

-Medullary Thyroid Carcinoma (aggressive)
-Neuromas
-Marfanoid habitus