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A. Characteristics navigator

  1. Most common pancreatic endocrine tumor
  2. Autonomous gastrin secretion by tumor
  3. Development of Zollinger-Ellison Syndrome (ZES)
    1. Syndrome is due to production of gastrin and acid hypersecretion
    2. Peptic Ulcers including distal duodenal and jejunal ulcers
    3. Severe reflux esophagitis may be present
    4. Diarrhea (may be severe) is common
  4. Tumor of variable malignancy; this determines long term survival
  5. Male : Female ~ 2:1; mean age in 50's
  6. Types of Gastric Carcinoids
    1. Type I: associated with chronic atrophic gastritis (CAG) [7]
    2. Type II: develops in patients with multiple endocrine neoplasia I (MEN I) or ZES
    3. Type III: sporadic
  7. Chronic Atrophic Gastritis Type A [7]
    1. Autoimmune destruction of parietal cells
    2. Leads to pernicious anemia and gastrin hypersecretion
    3. Prolonged gastrin hypersecretion may lead to gastrinoma

B. Location of Tumors (in decreasing frequency) [9]navigator

  1. Duodenum (46%)
  2. Pancreatic Head (13%)
  3. Lymph node tumor only (12%)
  4. Pancreatic body and tail
  5. Other Site (11%)
  6. Primary Tumor not found (18%)
  7. Metastases to lymph nodes and liver most common

C. Symptomsnavigator

  1. Multiple duodenal and jejunal ulcers [1]
    1. Zollinger-Ellison Syndrome (ZES) common in patients with jejunal ulcers
    2. May be first presentation for multiple endocrine neoplasia type I (MEN I)
    3. Patients ZES should be screened for other MEN-I endocrinopathies
    4. These include Pituitary Adenoma, Parathyroid Hyperplasia, other pancreatic islet tumors
  2. Stricture formation, perforation may occur as first presentation
  3. Painful ulceration
  4. Liver Disease - hepatitis, obstruction, pain

D. Diagnosisnavigator

  1. Multiple ulcerations on endoscopy or Upper GI with small bowel studies
  2. Serum Gastrin Levels
    1. Fasting levels highly elevated in most cases
    2. Normal level <110 pg/mL [5]
    3. Secretin provocative test: gastrin elevated but < 200 pg/mL on secretin stimulation
    4. Secretin (SecreFlo®) is now available commercially
  3. Standard Radiographic Methods
    1. CT scan of abdomen
    2. Ultrasound - least sensitive
    3. Magnetic Resonance Imaging (MRI) - sensitivity close to that of angiography
  4. Selective venous gastrin sampling
  5. Somatostatin Receptor Scintography [6]
    1. Radiolabelled octreotide (a somatostatin analog) is given iv
    2. Most sensitive test for detection of primary and metastatic lesions
    3. Recommended as initial screening method for patients with suspected gastrinoma
  6. Bone Scanning may be performed
  7. Search for other components of Multiple Endocrine Neoplasia

E. Treatment [2,3]navigator

  1. Surgical Resection [9]
    1. Surgical exploration in all patients with ZES without MEN-1 or metastatic disease
    2. Surgery is curative in these patients with 94% survival at 10 years
    3. Palliative surgery in MEN-1 and/or metastatic disease should be considered
  2. Acid Control
    1. Omeperazole (Prilosec®) or lansoprazole (Prevacid®): high dose controls acid secretion
    2. Most patients controlled on Omeprazole <80mg per day or less
    3. Some patients require up to 100mg po bid omperazole
    4. H-2 Histamine Blockers are rarely effective
    5. Vitamin B12 levels should be monitored in patients on long term acid suppression [8]
  3. Octreotide (somatostatin analog)
    1. Improves symptoms in Zollinger-Ellison Syndrome [10]
    2. Causes regression of Types II and III gastric carcinoids [11]
  4. Assess recurrence with secretin provocative test and fasting serum gastrin determination
  5. Imaging studies + calcium provocative test are not useful for initial recurrence detection

References navigator

  1. Benya RV, Metz DC, Venzon DJ, et al. 1994. Am J Med. 97(5):436 abstract
  2. Fishbeyn VA, Norton JA, Benya RV, et al. 1993. Ann Intern Med. 119(3):199 abstract
  3. Meko JB and Norton JA. 1995. Annu Rev Med. 46:395 abstract
  4. Kvols LK, Buck M, Moertel CG, et al. 1987. Ann Intern Med. 107:162 abstract
  5. Zimmer T, Stolzel U, Bader M, et al. 1995. NEJM. 333(10):634 abstract
  6. Gibril F, Reynolds JC, Doppman JL, et al. 1996. Ann Intern Med. 125(1):26 abstract
  7. Toh BH, van Driel KR, Gleeson PA. 1997. NEJM. 337(20):1441 abstract
  8. Termanini B, Gibril F, Sutliff VE, et al. 1998. Am J Med. 104(5):422 abstract
  9. Norton JA, Fraker DL, Alexander R, et al. 1999. NEJM. 341(9):635 abstract
  10. Yun D and Heywood JT. 1994. Ann Intern Med. 120(1):45 abstract
  11. Tomassetti P, Migliori M, Caletti GC, et al. 2000. NEJM. 343(8):551 abstract