ZollingerEllison

Zollinger-Ellison syndrome (ZES) is a rare disorder caused by gastrin-secreting neuroendocrine tumors called gastrinomas, most often in the pancreas or duodenum. The tumors autonomously secrete gastrin, causing marked gastric acid hypersecretion and peptic ulcers. ZES may occur alone or with MEN1 (multiple endocrine neoplasia type 1).

Pathophysiology and presentation: Excess gastrin stimulates parietal cells, producing multiple, often refractory ulcers that may be distal in the duodenum or jejunum. Diarrhea and weight loss are common. The combination of ulcers with acid-related symptoms that are difficult to treat raises suspicion for ZES.

Diagnosis and localization: Clinicians suspect ZES in patients with severe, refractory ulcers or ulcers in unusual locations. Fasting serum gastrin is elevated, and a secretin stimulation test supports the diagnosis. Localize tumors with CT or MRI, somatostatin receptor scintigraphy, endoscopic ultrasound, or PET with 68Ga-DOTATATE. Evaluate for MEN1.

Treatment and prognosis: Control of acid hypersecretion with high-dose proton pump inhibitors is essential. Localized gastrinomas may be surgically resected; metastatic disease requires systemic therapy and palliative measures. Prognosis depends on tumor stage and spread; outcomes are better for localized disease.

History: Zollinger and Ellison first described the syndrome in 1955. It remains a rare cause of peptic ulcer disease and is managed by a multidisciplinary team.