- Zollinger-Ellison syndrome (ZES) is a rare but serious medical condition characterized by the development of one or more gastrin-secreting tumors, known as gastrinomas, typically located in the pancreas or duodenum. These tumors lead to excessive production of gastrin, a hormone that stimulates the stomach to secrete hydrochloric acid. The overproduction of gastric acid results in severe peptic ulceration and gastrointestinal discomfort, often resistant to standard ulcer therapies.
- The primary pathophysiological feature of ZES is hypergastrinemia, which leads to hypersecretion of gastric acid. This acidic environment damages the mucosal lining of the stomach and small intestine, causing ulcers, most commonly in the duodenum, but sometimes extending into the jejunum. The increased acid load can also impair nutrient absorption, leading to diarrhea, steatorrhea, and malnutrition in some cases. Symptoms frequently include abdominal pain, nausea, vomiting, gastroesophageal reflux, and chronic diarrhea.
- Most gastrinomas are sporadic, but in about 25% of cases, they occur in association with Multiple Endocrine Neoplasia type 1 (MEN1), a genetic disorder involving tumors of the endocrine glands. In ZES, gastrinomas may be benign or malignant, and they often metastasize, especially to the liver and lymph nodes, which complicates management and prognosis. The diagnosis is typically established through fasting serum gastrin levels, secretin stimulation testing, and imaging techniques such as endoscopic ultrasound, somatostatin receptor scintigraphy, or MRI/CT to locate the tumors.
- Management of Zollinger-Ellison syndrome requires both control of acid hypersecretion and treatment of the underlying tumor(s). Proton pump inhibitors (PPIs), such as omeprazole or pantoprazole, are the cornerstone of acid suppression therapy, dramatically reducing acid production and healing ulcers. Surgical removal of the gastrinoma may be possible, especially if localized, though metastatic disease often requires systemic therapy. In cases involving MEN1, management can be more complex due to multiple endocrine tumors.
- In summary, Zollinger-Ellison syndrome is a gastrin-secreting tumor disorder that leads to excessive gastric acid production, resulting in severe peptic ulcers and gastrointestinal symptoms. Prompt recognition and effective acid suppression are crucial for symptom control, while tumor management depends on location, size, and metastasis. Early diagnosis and a multidisciplinary approach are essential for improving outcomes in affected patients.
