Zollinger–Ellison syndrome

A rare condition characterized by gastrin-secreting tumors

Zollinger–Ellison syndrome (ZES) is a rare disorder characterized by the formation of one or more tumors known as gastrinomas, which secrete excessive levels of the hormone gastrin. This leads to increased production of gastric acid, resulting in recurrent peptic ulcers. The condition is named after Robert Zollinger and Edwin Ellison, who first described it in 1955.

Pathophysiology

Endoscopic image of a peptic ulcer in Zollinger–Ellison syndrome

The primary feature of Zollinger–Ellison syndrome is the presence of gastrinomas, which are typically found in the duodenum, pancreas, or lymph nodes near the pancreas. These tumors secrete large amounts of gastrin, a hormone that stimulates the parietal cells of the stomach to produce gastric acid. The excessive acid production leads to the development of peptic ulcers, which can occur in the stomach, duodenum, and even the jejunum.

Clinical Presentation

Patients with Zollinger–Ellison syndrome often present with symptoms similar to those of peptic ulcer disease, including abdominal pain, diarrhea, and gastroesophageal reflux disease (GERD). The high levels of gastric acid can also lead to complications such as esophagitis, gastric bleeding, and perforation.

Diagnosis

The diagnosis of Zollinger–Ellison syndrome is based on clinical suspicion and confirmed through laboratory and imaging studies. Elevated serum gastrin levels are a hallmark of the condition. A secretin stimulation test may be performed to differentiate ZES from other causes of hypergastrinemia. Imaging studies such as endoscopic ultrasound, CT scan, and MRI are used to localize gastrinomas.

Treatment

The management of Zollinger–Ellison syndrome involves controlling gastric acid secretion and addressing the gastrinomas. Proton pump inhibitors (PPIs) are the mainstay of treatment to reduce acid production. Surgical resection of gastrinomas may be considered in certain cases, especially if the tumors are localized and resectable. In cases where surgery is not feasible, somatostatin analogs may be used to control symptoms.

Prognosis

The prognosis of Zollinger–Ellison syndrome varies depending on the presence of metastasis and the response to treatment. Patients with localized gastrinomas that can be surgically removed have a better prognosis. However, in cases where the tumors have spread, particularly to the liver, the condition can be more challenging to manage.

Related pages

• Peptic ulcer disease
• Gastrinoma
• Hypergastrinemia
• Multiple endocrine neoplasia type 1