Gastrinoma 

  • Author: Jennifer Lynn Bonheur, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 5, 2012
 

Background

A gastrinoma is a gastrin-secreting tumor that can occur in the pancreas, although it is most commonly found in the duodenum. Duodenal wall gastrinomas have been identified in 40-50% of patients. These duodenal wall tumors are frequently small and multiple. Sporadic tumors occurring in the pancreas tend to be solitary and have greater malignant potential as compared to duodenal gastrinomas.[1]

More than 80% of gastrinomas arise within the triangle defined as the confluence of the cystic and common bile duct superiorly, the second and third portions of the duodenum inferiorly, and the neck and body of the pancreas medially.

Rarely, primary tumors also occur in a variety of ectopic sites, including the body of the stomach, jejunum, peripancreatic lymph nodes, splenic hilum, omentum, liver, gallbladder, common bile duct, and the ovary.

Over 50% of gastrinomas are malignant and can metastasize to regional lymph nodes and the liver. One fourth of gastrinomas are related to multiple endocrine neoplasia (MEN) type I and are associated with hyperparathyroidism and pituitary adenomas. These MEN I associated tumors have been observed to occur at an earlier age than sporadic tumors and often follow a more benign course.

The triad of nonbeta islet cell tumors of the pancreas (gastrinomas), hypergastrinemia, and severe ulcer disease was described by Zollinger and Ellison in 1955, hence the eponym Zollinger-Ellison syndrome (ZES).

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Pathophysiology

Enormous secretion of gastrin from the tumor cells leads to hyperplasia of fundic parietal cells and increased basal acid secretion. This results in severe ulcer disease. Ulceration might even extend into the small intestine. The acidic content of the small intestine causes the release of secretin, which is responsible for the diarrhea, in part, caused by the outpouring of water and bicarbonate from the pancreas and small intestine.

A recent small study by Kohan et al describes duodenal bulb mucosa with hypertrophic heterotopia in patients with ZES.[2] The investigators evaluated the incidence of duodenal gastric oxyntic hypertrophic heterotopia in 7 patients with ZES over an average period of 5 years. The patients underwent endoscopic examinations with methylene blue staining and biopsy of the gastric and duodenal mucosa every 3-6 months.

Of the 7 patients with ZES and an intact stomach and duodenum, 2 had no evidence of duodenal mucosal hypertrophic heterotopia, but 5 did, in whom bowel mucosa biopsies revealed patchy replacement of surface epithelium by gastric-type epithelium with hypertrophic oxyntic glands in the lamina propria.[2] In addition, the patients with gastric oxyntic hypertrophic heterotopia had higher levels of average serum gastrin and basal acid output levels than those without gastric oxyntic hypertrophic heterotopia, a finding that Kohan et al suggest not only could hypertrophic and heterotopic gastric mucosa result from increased gastrin levels but that an increased incidence of postbulbar ulcers may result in affected patients.[2]

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Epidemiology

Frequency

International

The true incidence of ZES is not known. ZES constitutes 0.1% or more of cases of peptic ulcer disease. Although rare, gastrinomas are the most common pancreatic islet cell tumors.

Mortality/Morbidity

With the advent of antiulcer medications, the number of deaths secondary to ulcer complications decreased significantly. The primary determinants of survival for patients with gastrinomas are the size of the primary tumor and the occurrence of tumor metastasis.

  • Patients with hepatic metastases may have a remaining life span of less than 1 year.
  • In patients with liver metastasis, the 5-year survival rate is 20-30%.
  • In patients with localized disease or metastasis to local lymph nodes without liver metastasis, the 5-year survival rate is 90%.

Sex

Gastrinomas are more common in males than in females, with ratios from 1.5:1 to 2:1.

Age

Although gastrinomas can occur at any age, the initial clinical manifestation usually appears in people aged 30-50 years.

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Contributor Information and Disclosures
Author

Jennifer Lynn Bonheur, MD  Attending Physician, Division of Gastroenterology, Lenox Hill Hospital

Jennifer Lynn Bonheur, MD is a member of the following medical societies: American Gastroenterological Association, American Society for Gastrointestinal Endoscopy, New York Academy of Sciences, New York Society for Gastrointestinal Endoscopy, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Senthil Nachimuthu  MD, FACP

Senthil Nachimuthu is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Manoop S Bhutani, MD  Professor, Co-Director, Center for Endoscopic Research, Training and Innovation (CERTAIN), Director, Center for Endoscopic Ultrasound, Department of Medicine, Division of Gastroenterology, University of Texas Medical Branch; Director, Endoscopic Research and Development, The University of Texas MD Anderson Cancer Center

Manoop S Bhutani, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Institute of Ultrasound in Medicine, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Simmy Bank, MD  Chair, Professor, Department of Internal Medicine, Division of Gastroenterology, Long Island Jewish Hospital, Albert Einstein College of Medicine

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

References
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  2. Kohan E, Oh D, Wang H, et al. Duodenal bulb mucosa with hypertrophic gastric oxyntic heterotopia in patients with Zollinger Ellison syndrome. Diagn Ther Endosc. 2009;2009:298381. [Medline]. [Full Text].

  3. Poitras P, Gingras MH, Rehfeld JF. The Zollinger-Ellison Syndrome: Dangers and Consequences of Interrupting Antisecretory Treatment. Clin Gastroenterol Hepatol. Aug 24 2011;[Medline].

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  17. Oberg K, Eriksson B. Endocrine tumours of the pancreas. Best Pract Res Clin Gastroenterol. Oct 2005;19(5):753-81.

  18. Passaro E, Howard TJ, Sawicki MP. The origin of sporadic gastrinomas within the gastrinoma triangle: a theory. Arch Surg. Jan 1998;133(1):13-6; discussion 17. [Medline].

  19. Pellicano R, De Angelis C, Resegotti A. Zollinger-Ellison syndrome in 2006: concepts from a clinical point of view. Panminerva Med. Mar 2006;48(1):33-40.

  20. Price TN, Thompson GB, Lewis JT, Lloyd RV, Young WF. Zollinger-Ellison syndrome due to primary gastrinoma of the extrahepatic biliary tree: three case reports and review of the literature. Endocr Pract. Jun 2 2009;1-38. [Medline].

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