Zollinger Ellison Syndrome Information - symptom, cause, picture, treatment of
Zollinger Ellison Syndrome
Zollinger Ellison Syndrome Information
Zollinger-Ellison syndrome is caused by gastrin-secreting gut neuroendocrine tumors (gastrinomas), which result in hypergastrinemia and acid hypersecretion. Less than 1% of peptic ulcer disease is caused by gastrinomas. Primary gastrinomas may arise in the pancreas (25%), duodenal wall (45%), or lymph nodes (5-15%), and in other locations or of unknown primary in 20%. Approximately 80% arise within the "gastrinoma triangle" bounded by the porta hepatis, the neck of the pancreas, and the third portion of the duodenum.
Most gastrinomas are solitary or multifocal nodules that are potentially resectable. Over two-thirds of gastrinomas are malignant, and one-third have already metastasized to the liver at initial presentation. Approximately 25% of patients have small multicentric gastrinomas associated with MEN 1 that are more difficult to resect.
Zollinger Ellison Syndrome symptom
Over 90% of patients with Zollinger-Ellison syndrome develop peptic ulcers. In most cases, the symptoms are indistinguishable from other causes of peptic ulcer disease and therefore may go undetected for years. Ulcers usually are solitary and located in the duodenal bulb, but they may be multiple or occur more distally in the duodenum. Isolated gastric ulcers do not occur. Gastroesophageal reflux symptoms occur often. Diarrhea occurs in one-third of patients, in some cases in the absence of peptic symptoms. Gastric acid hypersecretion can cause direct intestinal mucosal injury and pancreatic enzyme inactivation, resulting in diarrhea, steatorrhea, and weight loss; nasogastric aspiration of stomach acid stops the diarrhea.
Screening for Zollinger Ellison syndrome with fasting gastrin levels should be obtained in patients with ulcers that are refractory to standard therapies, giant ulcers (> 2 cm), ulcers located distal to the duodenal bulb, multiple duodenal ulcers, frequent ulcer recurrences, ulcers associated with diarrhea, ulcers occurring after ulcer surgery, and patients with ulcer complications. Ulcer patients with hypercalcemia or family histories of ulcers (suggesting MEN 1) should also be screened. Finally, patients with peptic ulcers who are H pylori negative and who are not taking NSAIDs should be screened.
Zollinger Ellison Syndrome Treatment
Zollinger Ellison Syndrome Metastatic Disease
The most important predictor of survival is the presence of hepatic metastases. In patients with multiple hepatic metastases, initial therapy should be directed at controlling hypersecretion. Proton pump inhibitors (omeprazole, rabeprazole, pantoprazole, or lansoprazole) are given at a dose of 40–120 mg/d, titrated to achieve a basal acid output of < 10 meq/h. At this level, there is complete symptomatic relief and ulcer healing. In patients with isolated hepatic metastases, surgical resection may decrease the need for antisecretory medications and may prolong survival. Owing to the slow growth of these tumors, 30% of patients with hepatic metastases have a survival of 10 years.
Zollinger Ellison Syndrome Localized Disease
Cure can be achieved only if the gastrinoma can be resected before hepatic metastatic spread has occurred. Lymph node metastases do not adversely affect prognosis. Laparotomy should be considered in all patients in whom preoperative studies fail to demonstrate hepatic or other distant metastases. A combination of preoperative studies and intraoperative palpation and sonography allows successful localization and resection in the majority of cases. The 15-year survival of patients who do not have liver metastases at initial presentation is over 80%.