These tumors arise from cells in the pancreas that produce gastrin.
Symptoms are similar to those caused by peptic ulcers, including pain and bleeding.
Diagnosis includes blood and imaging tests.
The survival rate is high if the tumor is completely removed.
Treatment includes drugs to reduce acid in the stomach and sometimes surgery and chemotherapy.
Gastrinomas are a type of pancreatic endocrine tumor Overview of Pancreatic Endocrine Tumors The pancreas is an organ located in the upper part of the abdomen. It produces digestive juices that are secreted into the digestive tract. The pancreas also produces insulin, which helps control... read more . Most people with gastrinomas have several tumors clustered in or near the pancreas or the duodenum. About half of the tumors are cancerous. Sometimes a gastrinoma occurs as part of multiple endocrine neoplasia Multiple Endocrine Neoplasia Syndromes (MEN) Multiple endocrine neoplasia syndromes are rare, inherited disorders in which several endocrine glands develop noncancerous (benign) or cancerous (malignant) tumors or grow excessively without... read more , a hereditary disorder in which tumors arise from the cells of various endocrine glands, such as the insulin-producing cells of the pancreas.
The excess gastrin secreted by the gastrinoma causes the stomach to produce far too much acid. This overproduction of acid can results in Zollinger-Ellison syndrome. In Zollinger-Ellison syndrome, a person has symptoms of aggressive peptic ulcers Peptic Ulcer Disease A peptic ulcer is a round or oval sore where the lining of the stomach or duodenum has been eaten away by stomach acid and digestive juices. Peptic ulcers can result from Helicobacter pylori... read more (such as pain or bleeding) in the stomach, duodenum, and elsewhere in the intestine. However, as many as 25% of people with Zollinger-Ellison syndrome may not have an ulcer when the diagnosis is made. Rupture, bleeding, and obstruction of the intestine can occur and can be life threatening. For more than half of the people with a gastrinoma, symptoms are no worse than those experienced by people with ordinary peptic ulcer disease. In 25 to 40% of people, diarrhea resulting from the excess acid production is the first symptom.
A doctor suspects a gastrinoma when a person has frequent peptic ulcers or several peptic ulcers that do not respond to the usual ulcer treatments. Blood tests to detect abnormally high levels of gastrin are the most reliable diagnostic tests.
Once blood tests diagnose gastrinoma, doctors try to locate the tumor using several imaging techniques, such as computed tomography Computed Tomography and Magnetic Resonance Imaging of the Digestive Tract Computed tomography (CT—see also Computed Tomography (CT)) and magnetic resonance imaging (MRI—see also Magnetic Resonance Imaging (MRI)) scans are good tools for assessing the size and location... read more (CT) of the abdomen, scintigraphy (a type of radionuclide scanning Radionuclide Scanning In radionuclide scanning, radionuclides are used to produce images. A radionuclide is a radioactive form of an element, which means it is an unstable atom that becomes more stable by releasing... read more ), endoscopic ultrasonography Ultrasound Scanning (Ultrasonography) of the Abdomen Ultrasound scanning uses sound waves to produce pictures of internal organs (see also Ultrasonography). An ultrasound scan can show the size and shape of many organs, such as the liver and pancreas... read more , positron emission tomography Positron Emission Tomography (PET) Positron emission tomography (PET) is a type of radionuclide scanning. A radionuclide is a radioactive form of an element, which means it is an unstable atom that becomes more stable by releasing... read more (PET) scans, and arteriography (an x-ray taken after a radiopaque dye is injected into an artery). These tumors may be difficult to find, however, because usually they are small.
High doses of proton pump inhibitors, which are acid-reducing drugs Drug Treatment of Stomach Acid Stomach acid plays a role in a number of disorders of the stomach, including peptic ulcer, gastritis, and gastroesophageal reflux disease (GERD). Although the amount of acid present in the stomach... read more (see Table: Drug Treatment of Stomach Acid Drug Treatment of Stomach Acid Stomach acid plays a role in a number of disorders of the stomach, including peptic ulcer, gastritis, and gastroesophageal reflux disease (GERD). Although the amount of acid present in the stomach... read more ) may be effective for reducing acid levels and relieving symptoms temporarily. If these drugs are not effective enough, injections of octreotide may help. If there is only one tumor and the person does not have multiple endocrine neoplasia, doctors usually do surgery to remove the gastrinoma. In such cases, surgical removal cures about 20% of people.
If these treatments do not work, an operation to remove the stomach completely (total gastrectomy) may be necessary. This operation does not remove the tumor, but the gastrin can no longer create ulcers after the acid-producing stomach is removed. If the stomach is removed, daily iron and calcium supplements taken by mouth and monthly injections of vitamin B12 are needed, because absorption of these nutrients is impaired when stomach juices that prepare these nutrients for absorption are no longer available.
If cancerous tumors have spread to other parts of the body, chemotherapy may help reduce the number of tumor cells and the levels of gastrin in the blood. However, such therapy does not cure the cancer, which is ultimately fatal.