DIAGNOSTIC AND THERAPEUTIC APPROACH IN ZOLLINGER-ELLISON SYNDROME: ADVANCES AND CHALLENGES

Abstract

Zollinger-Ellison syndrome (ZES) is a rare condition characterized by the formation of tumors called gastrinomas, which secrete excess gastrin, stimulating the overproduction of gastric acid. This condition results in severe peptic ulcers, often refractory to conventional treatment, and may cause symptoms such as severe abdominal pain, chronic diarrhea, gastroesophageal reflux, and weight loss. ZES is frequently associated with multiple endocrine neoplasia type 1 (MEN-1), a genetic syndrome that increases the risk of multiple endocrine tumors. Early diagnosis is essential and involves measuring elevated serum gastrin levels, acid secretion tests, as well as imaging studies, such as computed tomography, magnetic resonance imaging, and somatostatin receptor scintigraphy to locate the gastrinomas. Treatment is primarily based on the use of proton pump inhibitors (PPIs), which control acid hypersecretion and prevent complications such as perforations and bleeding. In cases where gastrinomas can be identified and surgically resected, there is potential for cure, especially when the disease is localized. In patients with metastatic disease, most commonly affecting the liver, the approach includes chemotherapy, somatostatin analog therapy, and, in selected cases, hepatic embolization. Despite advances in the management of ZES, the presence of metastases and association with MEN-1 complicates treatment and worsens prognosis, making continuous monitoring and strict control of acid production essential. Prognosis depends on the extent of the disease, and advancements in diagnosis and treatment have improved the quality of life and survival of patients.