Path 9 MEN syndromes

Question 1

Delta cells

Answer 1

Somatostatin - suppress insulin and glucagon release

Question 2

D1 cells

Answer 2

VIP - induce glycogenolysis/hyperglycemia and GI fluid secretion

Question 3

Pancreatic enterochromaffin cells

Answer 3

Serotonin

Question 4

Insulinoma

Answer 4

Most common pancreatic endocrine neoplasm

-90% benign

Question 5

Insulinoma findings

Answer 5

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

Question 6

Insulinoma histo

Answer 6

Enlarged islet

• No anaplasia
• Amyloid deposition

Question 7

2 tumors with amyloid deposition

Answer 7

Insulinoma

Medullary thyroid carcinoma (calcitonin)

Question 8

Gastrinoma

Answer 8

Zollinger-Ellison Syndrome

• Gastrinoma triangle (mostly duodenum)
• Multiple peptic ulcers
• Usually metastatic to liver

Question 9

Glucagonoma

Answer 9

a-Cell tumor

• Mild diabetes (hyperglycemia)
• Necrolytic migratory erythema
• Anemia

Question 10

VIPoma

Answer 10

WDHA (pancreatic cholera) syndrome

-Watery Diarrhea, Hypokalemia, Achlorhydria

Question 11

MEN type 1

Answer 11

Wermer syndrome

-3 Ps (Parathyroid, Pituitary, Pancreas)

Question 12

MEN type 1 more specific

Answer 12

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

Question 13

MEN1 gene

Answer 13

Tumor suppressor gene

-Product is menin, cell cycle regulator

Question 14

MEN type 2a

Answer 14

Sipple syndrome

• Familial Medullary Thyroid Cancer (slow)
• Pheochromocytomas
• Parathyroid hyperplasia

Question 15

MEN2a/b gene

Answer 15

RET proto-oncogene mutation

Question 16

RET gain of function v loss of function

Answer 16

MEN2 v Hirschsprung

Question 17

MEN type 2b

Answer 17

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