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Zollinger-Ellison syndrome

  1. A non-beta islet cell tumor that produces gastrin and is associated with gastric acid hypersecretion and peptic ulcer disease. (LQ 2012)
  2. The tumors are biologically malignant in 60% of cases.
  3. Tumor size varies from 2 mm to 20 cm.
Tumor Distribution
  1. Most common site is duodenum. Majority of gastrinomas occurred within the Hypothetical gastrinoma triangle (confluence of the cystic and common bile ducts superiorly, junction of the second and third portions of the duodenum inferiorly, and junction of the neck and body of the pancreas medially. (Ref. Hari. 18th ed., Pg - 2454)
  2. Second most common site is pancreas.
Clinical Manifestations:
  1. Peptic ulcer (more than 90%)
    1. Duodenal bulb
    2. Unusual locations (second part of the duodenum and beyond).
    3. Ulcers refractory to standard medical therapy
    4. Ulcer recurrence after acid-reducing surgery
    5. Ulcers in the absence of H. pylori or NSAID ingestion. 
  2. Diarrhea
  3. Presence of MEN I (Gastrinoma can develop in 25% patients of MEN I) (LQ 2012)
Important Points:
  1. Most common site for ZES is gastrinoma triangle (80%)Q
  2. Most common site inside the gastrinoma triangle is pancreas.
  3. 2nd most common site or most common extra pancreatic site is duodenum.
  4. Duodenal tumors are smaller, slower growing, and less likely to metastasize than pancreatic lesions. Less-common extrapancreatic sites include stomach, bones, ovaries, heart, liver, and lymph nodes. (Ref. Hari. 18th ed., Pg - 2455)
  1. Fasting gastrin level.
    Fasting gastrin levels are usually <150 pg/mL. Virtually all gastrinoma patients have a gastrin level > 150-200 pg/mL.
Extra Edge Other causes of elevated fasting gastrin level.
  1. Gastric hypochlorhydria or achlorhydria (the most frequent), with or without pernicious anemia; (LQ 2012)
  2. Retained gastric antrum; 
  3. G cell hyperplasia; 
  4. Gastric outlet obstruction; 
  5. Renal insufficiency; 
  6. Massive small-bowel obstruction; 
  7. Conditions such as rheumatoid arthritis, vitiligo, diabetes mellitus, and pheochromocytoma.
  1. A BAO/MAO ratio >0.6 is highly suggestive of ZES, but a ratio <0.6 does not exclude the diagnosis.
  2. Gastrin provocative tests
    1. Secretin injection test
    2. An increase in gastrin of ≥200 pg within 15 min of secretin injection has a sensitivity and specificity of >90% for ZES.
    3. Calcium infusion test study is less sensitive and specific than the secretin test.
  3. Tumor Localization:
    1. Endoscopic ultrasound (EUS) permits imaging of the pancreas with a high degree of resolution (<5 mm). EUS is the most sensitive test for primary gastrinoma. PNQ.
    2. Somatostatin analogue 111In-pentreotide (Octreoscan) with sensitivity and specificity rate of >75%.
    3. For metastases: Abdominal CT scan, MRI, or Octreoscan. Out of these, octreoscan is the most sensitive for metastasis. PNQ.
    4. Selective Arterial Secretin Injection (SASI) is also an important imaging study for ZES.Q
  1. PPls are the treatment of choice
  2. Surgery
Therapy of metastatic lesion
  1. Streptozocin
  2. 5-fluorouracil
  3. Doxorubicin
  4. IFN-alpha
  5. Hepatic artery embolization
  6. IIIIn-pentetreotide
Recent Advances (Ref. Hari. 18th ed., Pg - 2457)
New therapies for ZES
  1. Radiofrequency ablation
  2. Cryoablation of liver lesions
  3. Use of agents that block the vascular endothelial growth receptor pathway (bevacizumab, sunitinib)
  4. To block the mammalian target of rapamycin

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