Introduction
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.
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).
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.
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.
Clinical
History
- The symptoms in 90-95% of patients with gastrinomas are similar to the symptoms of common peptic ulcer disease. Usually, persistent abdominal pain exists that is less responsive to medical treatment.
- Sometimes, symptoms may relate to a complication of peptic ulcer disease, such as bleeding (eg, melena, hematemesis), gastric outlet obstruction (eg, vomiting), and perforation (eg, peritoneal irritation).
- Other symptoms include gastroesophageal reflux, diarrhea, steatorrhea, and weight loss, all of which are secondary to acid hypersecretion. Vitamin B-12 malabsorption, which is not correctable by oral intrinsic factor, may also be observed.
- Chronic acid reflux may lead to esophageal complications (eg, esophagitis, stricture formation, Barrett esophagus) in up to two thirds of patients with Zollinger-Ellison syndrome.
Physical
- Epigastric tenderness is the most frequent abnormal physical finding. Depending on the possible ulcer complications, signs may vary.
- Nearly 75% of ulcers in patients with gastrinomas are present in the first portion of the duodenum. These ulcers usually are single or multiple and are indistinguishable from peptic ulcer disease.
- Nearly 10% of patients with ZES have no demonstrable ulcer. Ulcers located in the second, third, or fourth portion of the duodenum or jejunum should increase the possibility of gastrinoma.
- The other factors that alert one to the presence of underlying gastrinomas are the following:
- Ulcers that are refractory to standard therapy
- Multiple ulcers
- Giant ulcers, larger than 2 cm
- Recurrent ulcers
- Ulcers with unexplained diarrhea
- Strong family history of ulcers
- Hypercalcemia
- Duodenal ulcer that is not related to Helicobacter pylori infection or nonsteroidal anti-inflammatory drug use
More on Gastrinoma |
 Overview: Gastrinoma |
| Differential Diagnoses & Workup: Gastrinoma |
| Treatment & Medication: Gastrinoma |
| Follow-up: Gastrinoma |
| References |
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References
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Further Reading
Keywords
Zollinger-Ellison syndrome, ZES, ZE syndrome, gastrin-secreting tumor, pancreatic tumor, pancreas tumor, duodenal tumor, duodenal wall tumor, lymph node tumor, tumor, pancreatic islet cell tumors, malignancy, malignant tumor, basal acid output, BAO, ulcer, severe ulcer disease, multiple endocrine neoplasia type I, MEN type I
