Treatment of Zollinger-Ellison Syndrome with Multiple Endocrine Neoplasia Type 1 (MEN-1)
Zollinger-Ellison syndrome (ZES) occurring in the context of multiple endocrine neoplasia type 1 (MEN-1) is a clinically distinct and more complex presentation. The management of gastrinomas in this setting is considered controversial, and the approach differs significantly from sporadic ZES.
Clinical scenario: Zollinger-Ellison syndrome with concurrent multiple endocrine neoplasia type 1 (MEN-1). In these patients, management is more complicated and the primary goal of surgical intervention is largely the prevention of metastatic disease rather than biochemical cure.
Management approach
Surgical management is central to this protocol, and the recommended approach involves a defined sequence of procedures that accounts for co-existing endocrine pathology before addressing the gastrinoma. The complete surgical criteria, procedural steps, and decision thresholds are available in the full protocol.
References
DOI: 10.1016/j.suc.2009.06.018
- In patients with MEN-1, the management of gastrinomas is somewhat controversial and more complicated.
- The goal of surgery for patients with ZES and MEN-1 is largely prevention of metastatic disease.
- For this reason, patients with hyperparathyroidism and ZES in the setting of MEN-1 should have their parathyroid surgery first.
- The recommended procedure is a three and one-half gland parathyroidectomy.
- For this reason, it is recommended that patients with ZES and MEN-1 undergo surgery to remove any pancreatic gastrinoma greater than 2 cm.