Zollinger-Ellison Syndrome

Triad:
- gastric acid hypersecretion
- severe PUD
- islet cell tumors

- Rare, 50% are malignant

Diagnosis:
- serum gastrin off PPIs
- secretin stimulation testing
- Basal acid output
- Somatostatin Receptor Scintigraphy (localizes gastrinoma in 85%)

Passaro's Triangle: (70-90% of gastrinomas will be found within these limits)
- cystic duct
- 2nd/3rd part of duodenum
- jxn of head and body of pancreas

Rule out MEN1 prior to OR:
- in MEN1 pts resection rarely normalizes gastrin levels and does not affect long-term survival
- manage hypergastrinemia with very high doses of PPI
- in comparison sporadic cases should be treated aggressively with surgery as 50% 5-yr disease free survival

Surgical Management:
- warranted in absence of metastatic disease
- sporadic gastrinomas
- resection will largely depend on site of tumor
- limited hepatic resection for mets may be considered
- if widespread disease may consider vagotomy and antrectomy to avoid need for long-term PPIs