Disorders of the Endocrine Pancreas

Question 1

what are the 4 cell types for Islets of Langerhans

Answer 1

Alpha cells
beta cells
delta cells
PP cells (pancreatic polypeptide)

Question 2

what is the function of α cells

Answer 2

Glucagon raises blood glucose through glycogenolysis in liver

Question 3

what is the function of β cells

Answer 3

–Insulin – reduces blood glucose and regulates glucose utilization in tissues

Question 4

what is the function of δ cells

Answer 4

Somatostatin – supress insulin & glucagon release

Question 5

what is the function of PP cells

Answer 5

( Pancreatic polypeptide) – gastric and intestinal enzyme
secretion & inhibition of intestinal motility

Question 6

what is the major insulin regulator

Answer 6

Blood glucose, other sugars minor to no effect

Question 7

what is the minor insulin regulator

Answer 7

Neural factors : Epinephrine in response to low blood glucose, strenuous exercise, cold exposure, psychological stress
-Non carbohydrate nutrients ( dietary amino acids ). Lipids and metabolites no effect
* Hormonal factors: Incretin

Question 8

what is Incretins

Answer 8

Glucagon like peptide 1
Gastric inhibitory peptide

Question 9

what is the process for glucagon secretion

Answer 9

1. Hypoglycemia
2. intracellular glucose concentration falls
3. reduction in glycolysis-generated ATP) in the cell
4. ATP-sensitive potassium (K+) channels closes
5. Intracellular K+ concentration rises
6. Depolarization of cell membrane
7. opening voltage-dependent Ca2+ channels
8. influx of Ca2+
9. Increase in intracellular Ca2+ concentration
10. triggers secretion of glucagon through exocytosis.

Question 10

Counter regulatory hormones: for glucagon

Answer 10

Glucagon, epinephrine, cortisol and growth hormone

Question 11

2% of all pancreatic neoplasms
* Most common in adults
* Single / multiple
* Most present with symptoms of hormone production

Answer 11

PANCREATIC ENDOCRINE NEOPLASMS / ISLET CELL TUMORS

Question 12

All except ________are regarded to have malignant potential – local invasion, distant metastasis.

Answer 12

Insulinomas

Question 13

what are the mutations for PANCREATIC ENDOCRINE NEOPLASMS / ISLET CELL TUMORS

Answer 13

• MEN & PTEN - Tumor suppressor gene
• ATRX- product maintains telomere length

Question 14

The three most common and distinctive clinical syndromes associated with functional pancreatic endocrine
neoplasms are

Answer 14

(1) Hyperinsulinism,
(2) Hypergastrinemia and the Zollinger-Ellison syndrome
(3) MEN

Question 15

what is the most common pancreatic endocrine neoplasm

Answer 15

INSULINOMA

Question 16

insulinoma is what type of cell tumor

Answer 16

• β-cell tumor

Question 17

what are the Whipple’s triad in insulinoma

Answer 17

1. Symptoms of hypoglycemia-Mental confusion, weakness, fatigue
2. Fasting blood glucose levels <50mg/dl
3. Relief of symptoms by glucose administration

Question 18

describe the gross morphology of insulinoma

Answer 18

Gross: Solitary <2 cm encapsulated, pale to red-brown nodule

Question 19

describe the microscopic morphology of insulinoma

Answer 19

* resemble giant islets – preserved cords of monotonous cells and vasculature
* deposition of amyloid

cells are monotonous in appearance and demonstrate
minimal pleomorphism or mitotic activity.
There is abundant amyloid deposition

Question 19

GLUCAGONOMAS is what type of cell tumor

Answer 19

alpha -cell tumor

Question 20

ALPHA -CELL TUMORS (GLUCAGONOMAS) is associated with

Answer 20

increased serum levels of glucagon
* most frequently in perimenopausal and postmenopausal women.

Question 21

what are 3 clinical features present in :ALPHA -CELL TUMORS (GLUCAGONOMAS)

Answer 21

• mild diabetes mellitus
• Characteristic skin rash (necrolytic migratory erythema)
• Anemia

Question 21

what are the 4 association for δ-CELL TUMORS (SOMATOSTATINOMAS)

Answer 21

➢Diabetes mellitus
➢Cholelithiasis
➢Steatorrhea
➢Hypochlorhydria.

Question 22

what are the cells involved in somatostatinomas

Answer 22

D-cell tumors

Question 23

what plasma levels are required for diagnosis in δ-CELL TUMORS

Answer 23

High plasma somatostatin levels are required for diagnosis.

Question 24

Endocrine tumor with vasoactive intestinal peptide secretion is characterized as a

Answer 24

VIPoma

Question 25

3 Features - VIPoma syndrome (WDHA ) / Pancreatic cholera syndrome

Answer 25

• Watery diarrhoea,
• Hypokalemia,
• Achlorhydria

Question 26

what are other cause of VIPoma syndrome

Answer 26

➢Neural crest tumors
- Neuroblastomas, Ganglioneuroblastomas, Ganglioneuromas
Pheochromocytomas

Question 27

Zollinger-Ellison syndrome refers to the association

Answer 27

association of pancreatic islet cell lesions
with hypersecretion of gastric acid and severe peptic ulceration

Question 28

Gastrinomas are marked hypersecretion of ______

Answer 28

gastrin

Question 29

ulcers present in gastrinomas are found in :

Answer 29

duodenal and gastric ulcers often are multiple;
* ulcers may occur in unusual locations such as the jejunum;
* unresponsive to usual therapy

Question 30

one-half of gastrin-producing tumors are locally _____

Answer 30

invasive

Question 31

one-half of gastrin-producing tumors are locally _____

Answer 31

invasive

Question 32

MEN-1–associated gastrinomas frequently are___, while sporadic are ___

Answer 32

multifocal , single

Question 33

what test helps differentiate Gastrinomas from Atrophic gastritis.

Answer 33

Secretin Injection test:
dramatic rise in serum gastrin levels in ZES. Normally gastric G cells are inhibited