Zollinger-Ellison syndrome: classical considerations and current controversies

Area of Science:

• Endocrinology
• Gastroenterology
• Surgical Oncology

Background:

• Zollinger-Ellison syndrome (ZES) is an endocrinopathy defined by gastrin-producing tumors.
• ZES leads to refractory peptic ulcers and can be sporadic or associated with Multiple Endocrine Neoplasia type 1 (MEN-1).

Purpose of the Study:

• To outline the diagnostic and management strategies for Zollinger-Ellison syndrome.
• To discuss the surgical and medical approaches for ZES, including considerations for sporadic and MEN-1 variants.

Main Methods:

• Diagnosis involves biochemical serum evaluation for elevated gastrin levels.
• Localization of the primary tumor is achieved through radiological or nuclear imaging.
• Treatment combines medical suppression of gastric acid and surgical resection of the primary tumor.

Main Results:

• Proton pump inhibitors have significantly reduced the need for surgical acid reduction.
• Surgical resection aims for a negative microscopic margin in both sporadic and MEN-1 ZES.
• Postoperative surveillance includes gastrin level monitoring and imaging for recurrence or metastasis.

Conclusions:

• Aggressive cytoreductive surgery is favored for recurrent or metastatic ZES.
• Management requires a tailored approach based on ZES variant and disease extent.
• Effective treatment relies on accurate diagnosis, tumor localization, and multimodal therapeutic strategies.