Resolved and unresolved controversies in the surgical management of patients with Zollinger-Ellison syndrome

Abstract

Objective: Highlight unresolved controversies in the management of Zollinger-Ellison syndrome (ZES).

Summary background data: Recent studies have resolved some of the previous controversies including the surgical cure rate in patients with and without Multiple Endocrine Neoplasia-type1 (MEN1), the biological behavior of duodenal and pancreatic gastrinomas, role of imaging studies to localize tumor, and gastrectomy to manage acid output.

Methods: Review of the literature based on computer searches in Index Medicus, Pubmed and Ovid.

Results: Current controversies as identified in the literature include the role of endoscopic ultrasound (EUS), surgery in ZES patients with MEN1, pancreaticoduodenectomy (Whipple procedure), lymph node primary gastrinoma, parietal cell vagotomy, reoperation and surgery for metastatic tumor, and the use of minimally invasive surgical techniques to localize and remove gastrinoma.

Conclusions: It is hoped that future studies will focus on these issues to improve the surgical management of ZES patients.

FIGURE 1. Comparison of CT scan, MRI scan, and SRS in a patient with ZES. Neither the CT scan (top) nor MRI (middle) localizes a gastrinoma. SRS however, showed a focus in the left lobe of the liver. At surgery the patient had two 1 cm left lobe liver metastases and a small duodenal (0.3 cm gastrinoma plus an adjacent lymph node. This result demonstrates the enhanced sensitivity of SRS but also shows that it frequently misses small tumors

FIGURE 2. Results of SRS (top) and endoscopic ultrasound (EUS) localization of a gastrinoma. SRS localized a gastrinoma to the duodenum/pancreatic head area (arrow-labeled tumor). EUS localized a gastrinoma in the pancreatic head (labeled tumor) situated between the pancreatic duct (Pan duct) and common bile duct (CBD).

FIGURE 3. Algorithm showing patients with sporadic ZES based on the primary location and likely lymph node primary gastrinoma. Of the 138 patients, 36 had a lymph node only removed and 22 (16% of total) were disease free, suggesting a possible lymph node primary tumor. Subsequently, the patients were reevaluated with a yearly assessment for cure over a mean of 11.1 ± 1.3 years, 16 patients (12% of total) remained disease-free and are considered to have lymph node primary gastrinomas. Of the 6 patients who relapsed, 2 had duodenal primaries.

FIGURE 4. Comparison of the disease-free rate after initial surgical exploration and after reoperation for recurrent tumor. Plotted are the disease-free rates within 1 to 2 weeks of surgery and at a follow-up of at least 2.5 years. Results are plotted from data for initial surgery in 123 patients, and for reoperation in 15 patients

FIGURE 5. Effect of the extent of liver tumor on the survival of 212 patients. Disease-related survival is shown plotted in the form of Kaplan and Meier. Numbers in parenthesis refer to the number of patients in each group. Figure is drawn from the data in Yu et al.