Overview
Zollinger-Ellison Syndrome (ZES) is a rare disorder in which tumors called gastrinomas develop in the pancreas or duodenum. These tumors produce excessive amounts of gastrin, a hormone that causes the stomach to secrete large quantities of acid. The result is severe peptic ulcers, acid reflux, and abdominal pain. Early detection and treatment are essential to manage the condition and prevent complications.
What is Zollinger-Ellison Syndrome
Zollinger-Ellison Syndrome (ZES) is a condition characterized by the formation of one or more gastrin-secreting tumors (gastrinomas), usually found in the pancreas or upper small intestine (duodenum). These tumors lead to excessive gastric acid production, which causes multiple, recurring, and often difficult-to-treat peptic ulcers. ZES can occur sporadically or be associated with a genetic disorder known as Multiple Endocrine Neoplasia type 1 (MEN1).
Symptoms
- Severe or recurrent peptic ulcers
- Abdominal pain, especially between meals or at night
- Chronic diarrhea or steatorrhea (fatty stools)
- Nausea and vomiting
- Gastroesophageal reflux disease (GERD)
- Unexplained weight loss
- Bleeding in the digestive tract in advanced cases
Causes
Zollinger-Ellison Syndrome is caused by gastrinomas, tumors that secrete excessive gastrin. This hormone overstimulates the stomach lining to produce acid, which damages the gastrointestinal tract.
- In most cases, gastrinomas develop spontaneously
- About 25% of ZES cases are associated with MEN1, a hereditary condition that involves multiple endocrine tumors
- The tumors can be benign or malignant, and some may metastasize, especially to the liver or lymph nodes
Risk Factors
- MEN1 genetic syndrome (autosomal dominant inheritance)
- Family history of endocrine tumors
- Age between 20 and 50 years
- Male gender (slightly higher prevalence)
- Personal history of persistent or treatment-resistant ulcers
Complications
- Recurrent peptic ulcers leading to gastrointestinal bleeding or perforation
- Severe acid reflux and esophagitis
- Malabsorption and chronic diarrhea
- Tumor metastasis, particularly to the liver
- Nutritional deficiencies due to poor digestion and absorption
- Increased risk of gastric cancer in long-standing untreated cases
Prevention
Zollinger-Ellison Syndrome cannot be prevented, especially in cases with a genetic link. However, early diagnosis and effective management can help reduce the risk of complications:
- Genetic counseling for families with MEN1 history
- Regular monitoring in high-risk individuals
- Early evaluation of persistent or severe peptic ulcers
- Avoidance of NSAIDs which can worsen ulcers
- Proactive acid suppression therapy when ZES is suspected
Treatment Options in Korea
Treatment of Zollinger-Ellison Syndrome in Korea involves both medical and surgical approaches tailored to tumor control and acid suppression:
- Proton pump inhibitors (PPIs): High-dose PPIs are the mainstay for controlling stomach acid and healing ulcers
- Somatostatin analogs (e.g., octreotide): Used to suppress gastrin secretion in some cases
- Surgical resection: Removal of gastrinomas when possible, particularly if localized and non-metastatic
- Radiofrequency ablation or embolization: Used for tumors that have spread to the liver
- Targeted therapy and chemotherapy: For malignant or metastatic tumors
- Monitoring and follow-up: Ongoing imaging and gastrin level checks to detect recurrence or progression
Patients in Korea benefit from advanced imaging, minimally invasive surgical options, and coordinated care through endocrinology and gastroenterology specialists.
