Zollinger Ellison Syndrome

Question 1

Cause of ZES

Answer 1

• non beta islet cell, gastrin secreting tumor

- may occur sporadically (75%) or as part of MEN1 (25%) (autosomal dominant familial syndrome)

Question 2

Primary tumor of ZES location

Answer 2

Duodenum
Pancreas
Abdominal lymph nodes
Ectopic (heart, gallbladder, liver, kidney)

Question 3

Pathophysiology of ZES

Answer 3

Gastrinoma causes hypergastrinemia
Increase in number of parietal cells,
Increased maximal acid output
Gastrin also increases basal acid secretion
Also leads to diarrhea and malabsorption ( inactivation of pancreatic enzymes and precipitation of vile salts)

Question 4

MEN 1 - Wermer’s syndrome

Answer 4

Hyperparathyroidism
Pancreatic endocrine tumors
Pituaitary tumors (galactinome, GH)

Question 5

ZES in duodenal ulcers

Answer 5

0.1 - 1 % of patients with duodenal ulcers.

Question 6

ZES age of onset

Answer 6

40s for ZES
30s for MEN1/ZES
5-7 year delay to diagnosis
3% are younger than 20 years
7% are older than 60 years of age

Question 7

Survival of ZES

Answer 7

without metastasis :

80% survival at 15 years

Question 8

Complications / consequences

Answer 8

5% develop abdominal perforation, gastric outlet obstruction or esophageal stricture, gastro-intestinal bleeding.
Seldom :
- Gastric carcinoids (especially in MEN1)

Question 9

ZES Anamnesis

Answer 9

Nausea
Vomitting
Gastro-intestinal bleeding
Weight loss

Question 10

MEN 1 Anamnesis

Answer 10

Nephrolithiasis
Hypercalcemia
Pituitary disorders
Fam Hx :
- Nephrolithiasis
- Hyperparathyroidism
- Gastrinoma

Question 11

ZES physical exam

Answer 11

Pale if gastro-intestinal bleeding
Jaundice if compression of common bile duct (rare)
Epigastric tenderness
Dental erosion
Hepatomegaly if liver metastasis

Question 12

Lab Studies

Answer 12

1. Fasting serum gastrin levels 3x, diff days (no PPI, serial measurements, )
   Serum calcium (if elevated search for MEN1)
2. Basal gastric secretory volum > 140ml
   Gastric pH < 2 and >140ml over 1h is highly suggestive of ZES
3. Secretin stimulation test:
   - fasting night
   - 2U/kg of secretin
   - gastrin levels measured at 0,2,5,10,15 min
   - >200pg/ml is diagnostic
4. Somatostatin receptor scintigraphy (SRS)
5. Stage and localize (DOTATATE PETCT)