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Also known as Gastrinoma, Zollinger Ellison syndrome is relatively rare condition characterized by gastrin producing tumors resulting in excessive secretion of gastrin. Gastrin producing cells (G cells) are most abundant in stomach. The most common site of gastrinoma is duodenum > Pancreases > stomach.
Clinical features:
- Epigastric pain.
- Use/ abuse of proton pump inhibitors (PPIs).
- Diarrhea: Diarrhea is usually not seen in Acid peptic disease, H. pylori infection.
- Gastro-esophageal reflux disease.
- Family/ Previous history of Nephrolithiasis.
- In advanced cases and liver metastasis: hard consistency of liver.
- Hematemesis, melena.
Zollinger Ellison syndrome can be associated with Multiple endocrine neoplasia I (MEN-I). MEN-I consist of pituitary adenoma (increased prolactin), Parathyroid adenoma (Increase calcium), pancreatic adenoma (Zollinger Ellison syndrome).
Pathophysiology of diarrhea in Zollinger Ellison Syndrome:
Workup and Diagnosis:
- Upper Gastrointestinal (UGI) endoscopy: Ulcers due to H. pylori are usually present at first part of duodenum and are solitary. Ulcers in Zollinger Ellison syndrome are:
- Multiple duodenal ulcers.
- Giant duodenal ulcers.
- Ulcers at atypical sites.
- Breathe urea test: rule out H. pylori infection.
- PTH (Parathyroid hormone) assay.
- Rule out/ investigations for association with MEN I:
- Serum calcium, PTH (Parathyroid hormone) assay.
- Pancreatic polypeptide.
- Prolactin levels.
- Secretin study (investigation of choice).
- Fasting gastrin > 1000pg/mL.
- Basal acid output: >15 mEq/hr.
- Ratio of basal acid output : Maximum acid output >0.6.
- Endoscopic ultrasonography: best investigation for tumor localization. Most of the tumors are too small to be picked by CT, MRI. Most common sites of gastrinoma are duodenum > Pancreases > stomach >> mesentry >>> heart, ovaries.
- Somatostatin scintigraphy: best imaging modality for gastrinoma.
- For concomitant hypercalcemia:
- Serum PTH levels.
- SESTAMiBi scan for parathyroid adenoma.
Triangle of gastrinoma/ Passaro’s Triangle:
Management:
Proton pump inhibitors: for 4-6 weeks. They provide time for healing of ulcers.
Lanreotride: It is long acting derivative of octreotide (Somatostatin analogue).
Surgery: If size more than 1.5cm-2cm, or picked by CT, MRI.
References:
- Harrison’s Principles of Internal Medicine, 21st edition.
- Medical Physiology Textbook – 14th Edition.
- Robbins Basic Pathology: 10th Edition.
- Bailey & Love’s Short Practice of Surgery – 28th Edition.
