Gastrinoma Health Article

Surgery

Because of the likelihood that gastrinomas may be malignant, in both sporadic tumors and those associated with the inherited MEN-1 syndrome, surgery to locate and remove gastrinomas is frequently advised. It is now known that complete surgical removal of gastrinomas can cure the overproduction of gastrin, even in patients who have metastases to the lymph nodes. Surgery in patients with MEN-1 and ZES, however, remains controversial since the benefit is less clear.

Freedom from disease after surgery is judged by improved symptoms, reduced gastric acid production, reduced need for drug therapy, normalization of serum gastrin levels, and normalization of results from the secretin stimulation test and imaging studies.

Prognosis

Medical therapy often controls symptoms, and surgery may or may not cure gastrinoma. About 50% of ZES patients in whom gastrinomas are not removed will die from malignant spread of the tumor. In patients with gastrinomas as part of MEN-1 syndrome, the cure rate is extremely low.

A NIH study of patients who had surgical removal of gastrinomas found that 42% were disease-free one year after surgery and 35% were disease-free at five years. Disease recurrences can often be detected with a serum gastrin test or secretin stimulation test.

When gastrinomas are malignant, they often grow slowly. The principal sites of metastasis are the regional lymph nodes and liver, but they may also spread to other structures. About one quarter of patients with gastrinomas have liver metastases at the time of diagnosis. This appears to be more frequent with pancreatic gastrinomas than duodenal gastrinomas.

Metastases of malignant gastrinomas to the liver is very serious. Survival five years after diagnosis is 20–30%, however patients with gastrinomas found only in the lymph nodes have been known to live as long as 25 years after diagnosis, without evidence of further tumor spread. In fact, the life expectancy of patients with gastrinomas that have spread to the lymph nodes is no different from that of patients with gastrinomas that cannot even be found at surgery for about 90%, five years after diagnosis.

BOOKS

Friedman, Lawrence S., and Walter L. Peterson. "Peptic Ulcer and Related Diseases." In Harrison's Principles of Internal Medicine, ed. Anthony S. Fauci, et al. New York: McGraw-Hill, 1997.

PERIODICALS

Delcore, R., and S. R. Friesen. "The Place for Curative Surgical Procedures in the Treatment of Sporadic and Familial Zollinger-Ellison Syndrome." Current Opinion in General Surgery (1994): 69-76.

Meko, J. B., and J. A. Norton. "Management of Patients with Zollinger-Ellison Syndrome." Annual Review of Medicine 46 (1995): 395-411.

ORGANIZATIONS

National Digestive Diseases Information Clearinghouse. 2 Information Way, Bethesda, MD 20892-3570. (800) 891-5389. <http://www.niddk.nih.gov/health/digest/nddic.htm>.

Caroline A. Helwick

KEY TERMS

Gastrin—A hormone secreted in the stomach that is involved in the production of gastric acid. Over-production of gastric acid contributes to peptic ulcer formation.

Multiple endocrine neoplasia type 1 (MEN-1)— An inherited condition marked by multiple malignancies of the pituitary gland, parathyroid gland, and islet cells of the pancreas. About half of MEN-1 patients with pancreatic islet cell tumors will have gastrinomas, gastrin-producing tumors that lead to ulcer disease.

Peptic ulcer—An eroded area in the stomach lining or in the first part of the duodenum (beginning of the small intestine).

Serum gastrin test—A laboratory test that is performed on a blood sample to determine that level of the hormone gastrin. High levels of gastrin indicate the presence a duodenal ulcer or a gastrinoma.

Sporadic—Occurring at random or by chance, and not as a result of a genetically determined, or inherited, trait.