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Surgical intervention shows promise in selected patients with MEN1-associated Zollinger-Ellison syndrome, with new evidence highlighting improved gastrin control and the importance of early tumour management.
Managing Zollinger-Ellison syndrome (ZES) in patients with multiple endocrine neoplasia type 1 (MEN1) has remained challenging because gastrin-producing tumours are often multifocal and carry a risk of lymph node involvement and metastatic progression. While acid suppression therapy remains effective for symptom control, the potential role of surgery in long-term disease management continues to generate clinical debate.
New findings from a large AFCE (Association Francophone de Chirurgie Endocrinienne) and GTE (Groupe d’étude des Tumeurs Endocrines) nationwide cohort study provide further clarity on surgical decision-making in MEN1-associated ZES. The analysis showed that surgery was performed safely and provided meaningful biochemical benefits, particularly in carefully selected patients who required improved control of gastrin excess.
Among 233 patients with MEN1-associated ZES, 66 individuals underwent surgery for gastrinoma-related disease. Surgical approaches ranged from focused gastrinoma removal to more extensive procedures addressing gastrinomas associated with pancreatic neuroendocrine tumours. The outcomes revealed that operative management was feasible, with no perioperative deaths reported, supporting the safety of surgery in experienced centres.
A major clinical insight emerged from lymph node evaluation. Metastatic lymph node involvement was detected in 71% of patients undergoing lymphadenectomy, highlighting the hidden metastatic potential of MEN1-associated gastrinomas. Patients with metastatic disease experienced significantly poorer survival outcomes, emphasising the importance of timely risk assessment and appropriate treatment planning.
The study also identified differences between surgical interventions. More extensive resections involving the duodenum and pancreatic head achieved better gastrin levels compared with limited duodenal procedures, suggesting improved hormonal control with a more comprehensive surgical strategy in selected patients.
Although surgery did not illustrate a statistically significant survival advantage among non-metastatic patients, the findings supported its continued role in specific clinical scenarios. Younger and medically fit individuals, particularly those at risk of tumour progression or persistent hypergastrinaemia, were represented as potential candidates for surgical intervention. The findings broadened the understanding of MEN1-associated ZES management, suggesting that surgery may improve biochemical control while potentially minimizing the risk of disease progression.
World Journal of Surgery
Surgical Management of Zollinger‐Ellison Syndrome in Multiple Endocrine Neoplasia Type 1 an AFCE and GTE Cohort Study. (Association Francophone de Chirurgie Endocrinienne and Groupe d'étude des Tumeurs Endocrines)
Sébastien Gaujoux et al.
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