Treatment of Biochemically Diagnosed Sporadic Zollinger-Ellison Syndrome Without MEN-1
Clinical Scenario
This protocol addresses patients with Zollinger-Ellison syndrome confirmed through biochemical diagnosis who have the sporadic form of the disease — specifically those without multiple endocrine neoplasia type 1 (MEN-1). In sporadic ZES, the goal of surgery is to cure the disease, and all patients with a biochemical diagnosis of sporadic ZES should undergo surgical exploration.
Approach Overview
The recommended management centres on surgical exploration, using a structured operative approach to systematically identify and address tumors in the pancreas and duodenum — the full procedural sequence, operative decision points, and all components of the technique are detailed in the complete protocol.
References
- In sporadic ZES, the goal of surgery is to cure the disease.
- All patients with a biochemical diagnosis of sporadic ZES should undergo surgical exploration.
- The recommended surgical approach is exploratory laparotomy with extended Kocher maneuver and careful palpation of the pancreas and duodenum for nodules.
- Intraoperative ultrasound (IOUS) should be used for identification of small pancreatic lesions.
- Enucleation of pancreatic head tumors should be performed, or distal pancreatectomy for tail lesions, and duodenotomy and regional lymphadenectomy.
- Duodenotomy is indicated in all patients undergoing surgery for ZES.
- Removal of all regional lymph nodes is recommended.
DOI: 10.1016/j.suc.2009.06.018
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