Management of Zollinger-Ellison Syndrome

Abstract

Zollinger-Ellison syndrome (ZES) is a disease caused by gastrinoma. Two-thirds of cases are located within the gastrinoma triangle and associated with symptoms of peptic ulcer disease and diarrhea. The diagnosis of ZES is established by measuring fasting levels of serum gastrin, gastric pH, and secretin provocative gastrin stimulation. ZES is frequently associated with multiple endocrine neoplasia type 1; hence, this must be excluded in all patients. Treatment of ZES consists of medical control of symptoms with proton pump inhibitors and evaluation for potentially curative surgery. Preferred preoperative imaging studies include cross-section magnetic resonance imaging or computed tomography and dotatate positron emission tomography computed tomography scan. Endoscopic ultrasound may be performed to further evaluate primary tumors if other testing is negative. All patients with resectable sporadic gastrinoma should undergo surgical exploration. The goal of surgery is tumor control. Intraoperative tumor localization requires methodical exposure of the pancreas and duodenotomy for possible duodenal gastrinoma. In patients with multiple endocrine neoplasia type 1, surgical resection should be pursued only if there is a tumor is localized on imaging in the absence of metastatic disease. Patients with sporadic ZES with negative imaging should undergo resection. In sporadic patients with synchronous or metachronous liver metastases, surgery should be performed if all visible tumor can be removed safely. Patients with recurrent ZES should be restaged and operation considered if localized tumor is identified. Patients with liver metastases benefit from coordinated care with a multidisciplinary team and not infrequently will have survival prolonged by surgical resection.