Zollinger-Ellison syndrome

Background

• Also known as "S-E syndrome"
• Caused by secretion of gastrin by duodenal/pancreatic neuroendocrine tumors (gastrinomas) —> high gastric acid output by parietal cells
• Associated with peptic ulcers and diarrhea
• 60-90% malignant , only 20% resectable; 25% of gastrinoma patients have multiple endocrine neoplasia type I; >80% of gastrinomas found in gastrinoma triangle.

Clinical Features

• Hematemesis
• Chronic diarrhea that is responsive to PPIs
• Gastroesophageal reflux disease
• Steatorrhea
• Weight loss
• Postprandial abdominal pain
• Nausea
• Wheezes on auscultation
• Evidence of malnourishment and decreased oral intake

Differential Diagnosis

Epigastric Pain

• Gastroesophageal reflux disease (GERD)
• Peptic ulcer disease with or without perforation
  • Zollinger-Ellison syndrome
• Gastritis
• Pancreatitis
• Gallbladder disease
  • Acute cholecystitis
  • Cholangitis
  • Symptomatic cholelithiasis
  • Acalculous cholecystitis
• Myocardial Ischemia
• Splenic Infarctionenlargement/rupture/aneurysm
• Pericarditis/Myocarditis
• Aortic dissection
• Hepatitis
• Pyelonephritis
• Pneumonia
• Pyogenic liver abscess
• Fitz-Hugh-Curtis Syndrome
• Hepatomegaly due to CHF
• Bowel obstruction
• SMA syndrome
• Pulmonary embolism
• Bezoar
• Ingested foreign body

Upper gastrointestinal bleeding

• Peptic ulcer disease (most common cause)
  • Zollinger-Ellison syndrome
• Gastritis/esophagitis
• Gastric/esophageal varices
• Mallory-Weiss tear
• Malignancy
• Aortoenteric fisulta
• Boerhaave
• Dieulafoy's lesion
• Angiodysplasia
• Hemobilia
• Hemorrhagic gastritis, EtOH
• Celiac disease
• Dengue
• Other intrabdominal bleeds
  • Lower GI bleeding
  • Hemorrhagic pancreatitis
  • Splenic rupture
  • Subcapsular cavernous hemangiomas
  • Peliosis hepatis

Mimics of GI Bleeding

• Hemoptysis
• Vaginal/Urethra bleeding
• ENT bleeding
• Dietary (Iron, bismuth, beets)
• Swallowed maternal blood (in neonate)

Evaluation

Diagnosis

• Fasting serum gastrin concentration (>10x upper limit of normal) in the presence of gastric pH <2 is diagnostic
• If findings not diagnostic for ZES, will require secretin stimulation test

Management

• Proton-Pump Inhibitors
• Somatostatin analog (Octreotide) if no improvement
  • Direct inhibition of gastric secretion
• Surgical resection