ZOLLINGER-ELLISON SYNDROME

Question 1

Overview (4)

Answer 1

1- Gastrinoma (tumor in the pancreas producing gastrin) —> increased gastric acid and HCL secretion by parietal cells in the stomach due to high levels of gastrin —> ulcer formation
2- Ulcer like symptoms (burning gnawing abdominal pain, cough, may —> perforation or bleeding) with chronic diarrhea or virulent ulcer diathesis
3- Causes multiple duodenal ulcers or ulcers in uncommon locations
4- Ask about personal or family history of refractory PUD or endocrine disease and screen for MEN 1:
- 50% associated with MEN1
- P3: Parathyroid, Pituitary, Pancreas
- Must check serum calcium levels, if high check PTH levels - high penetrance of parathyroid gland)

Question 2

Diagnosis (6)

Answer 2

1- Hypergastrinemia (fasting gastrin levels >1000 - PPIs should be stopped for 1 week prior to test)
2- Secretin stimulation test (paradoxical increase in gastrin level after secretin administration)
3- Gastric pH and endoscopy (visualization of multiple ulcers in abnormal locations)
4- Tumor localization by abdominal CT, MRI, U/S
5- Most commonly located in the gastrinoma triangle (junction of cystic duct and CBD, 2nd and 3rd part of the duodenum, and neck of the pancreas)
6- Liver is most common site for metastasis

Question 3

Management (4)

Answer 3

1- High dose PPI and H2 blockers to suppress acid
2- Surgical exploration if sporadic with no metastatic - proximal gastric vagotomy done to decrease acid secretion and search for tumor and resect if found
3- MEN1 rarely curable so surgery not recommended
4- For metastasis do debulking surgery, hepatic embolization, chemo. Also, somatostatin analogues like octreotide are used to decrease gastric levels and slow tumor growth