Gastrinoma Treatment & Management

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

Medical Care

Individualize the selection of treatment. Base treatment on factors related to ulcer disease, diarrhea, and malignant properties of the tumor. Antisecretory medications are helpful for controlling the manifestations of peptic acid disease and secretory diarrhea (secondary to hyperacidity).

  • Proton pump inhibitors (eg, omeprazole, lansoprazole)
    • These are highly effective drugs and are the drugs of choice for suppressing acid secretion. Long duration of action, fewer adverse effects, and high potency make them superior to H2 blockers.
    • In 60% of patients, ulcer healing occurs within 2 weeks. In 90-100% of patients, healing occurs within 4 weeks.
    • The recommended initial dose of omeprazole is 60 mg/d. Divided, twice-a-day dosing is suggested for doses greater than 80 mg/d. Once an effective maintenance dose is achieved, tapering of the medication, while monitoring symptoms and acid output, is suggested.[3]
  • H2-receptor antagonists
    • The dose usually is 4-8 times higher than the dose administered to patients with peptic ulcer disease.
    • Although a good success rate exists, this treatment has been reported to fail in 50% of patients.
  • Chemotherapy
    • This is indicated in patients with metastatic disease and in patients who are not candidates for surgery; however, it is not indicated for metastatic disease confined to the lymph nodes.
    • Chemotherapy reduces tumor size and improves the symptoms secondary to metastatic effects of the tumor.
    • A combination of streptozocin, 5-fluorouracil, and doxorubicin has been used, with the response rate reported to be as high as 65%.
    • Granberg et al described a patient with almost complete response on treatment with Sandostatin LAR, a long-acting somatostatin analog.[4]
    • Interferon or targeted radiotherapy may also be considered in patients who are not candidates for chemotherapy.
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Surgical Care

  • Surgical care is indicated for localized disease.[5] Surgical resection of localized disease leads to a complete cure without any recurrence in 20-25% of patients with gastrinomas.
  • Patients who have an isolated lesion or patients in whom the preoperative workup fails to localize the tumor should undergo laparotomy (by an experienced surgeon) with the intent to resect.
  • It has been reported that Whipple pancreaticoduodenectomy affords the greatest probability for cure, particularly for MEN I associated gastrinomas, though also for sporadic tumors, since it results in removal of the entire gastrinoma triangle. However, the excellent long-term survival in patients with less complicated surgeries and the increased morbidity and mortality associated with the Whipple procedure make its general utility still unclear and recommended primarily for large, advanced tumors.
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Consultations

  • Gastroenterologist
  • Endocrinologist
  • Oncologist
  • Surgeon
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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

  1. Ruiz-Tovar J, Priego P, Martinez-Molina E, et al. Pancreatic neuroendocrine tumours. Clin Transl Oncol. Aug 2008;10(8):493-7. [Medline].

  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].

  4. Granberg D, Jacobsson H, Oberg K, Gustavsson J, Lehtihet M. Regression of a large malignant gastrinoma on treatment with Sandostatin LAR: a case report. Digestion. 2008;77(2):92-5. [Medline].

  5. Fendrich V, Bartsch DK. Surgical treatment of gastrointestinal neuroendocrine tumors. Langenbecks Arch Surg. Mar 2011;396(3):299-311. [Medline].

  6. Campana D, Piscitelli L, Mazzotta E. Zollinger-Ellison syndrome. Diagnosis and therapy. Minerva Med. Jun 2005;96(3):187-206. [Medline].

  7. Del Valle J, Scheiman J. Zollinger-Ellison Syndrome. Textbook of Gastroenterology, 4th Edition. 2003;1377-1388.

  8. Delvalle J, Yamada T. Zollinger-Ellison Syndrome. Textbook of Gastroenterology. 1995;1430.

  9. Feldman M, Sleisenger MW, McGuigan JE. Zollinger-Ellison syndrome and other hypersecretory states. In: Feldman M, Scharschmidt BF, Sleisenger M, Zorab R, eds. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 6th ed. Philadelphia, Pa: WB Saunders; 1998:. 679-90.

  10. Hirschowitz BI. Zollinger-Ellison syndrome: pathogenesis, diagnosis, and management. Am J Gastroenterol. Apr 1997;92(4 Suppl):44S-48S; discussion 49S-50S. [Medline].

  11. Jensen RT, Gibril F. Somatostatin receptor scintigraphy in gastrinomas. Ital J Gastroenterol Hepatol. Oct 1999;31 Suppl 2:S179-85. [Medline].

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  13. Nobels FR, Kwekkeboom DJ, Coopmans W. Chromogranin A as serum marker for neuroendocrine neoplasia: comparison with neuron-specific enolase and the alpha-subunit of glycoprotein hormones. J Clin Endocrinol Metab. Aug 1997;82(8):2622-8. [Medline].

  14. Norton JA. Gastrinoma: advances in localization and treatment. Surg Oncol Clin N Am. Oct 1998;7(4):845-61. [Medline].

  15. Norton JA. Surgical treatment and prognosis of gastrinoma. Best Pract Res Clin Gastroenterol. Oct 2005;19(5):799-805. [Medline].

  16. Norton JA, Fang TD, Jensen RT. Surgery for gastrinoma and insulinoma in multiple endocrine neoplasia type 1. J Natl Compr Canc Netw. Feb 2006;4(2):148-53.

  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].

  21. Sugg SL, Norton JA, Fraker DL. A prospective study of intraoperative methods to diagnose and resect duodenal gastrinomas. Ann Surg. Aug 1993;218(2):138-44. [Medline].

  22. Wong H, Yau T, Chan P, et al. PPI-delayed diagnosis of gastrinoma: oncologic victim of pharmacologic success. Pathol Oncol Res. Aug 20 2009;[Medline].

 
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