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)

AUTHORS:
S. Gaujoux (APHP, France) , F. Pattou (APHP, France) , R. Caiazzo (APHP, France) , S. Deguelte (APHP, France) , G. Donatini (APHP, France) , J. Lifante (APHP, France) , E. Mirallié (APHP, France) , N. Santucci (APHP, France) , F. Sebag (APHP, France) , P. Goudet (APHP, France)
Background:
Objective: Management of ZES in MEN1 remains controversial.To describe surgical indications, procedures and outcome in patients operated for Zollinger-Ellison syndrome (ZES) in Multiple Endocrine Neoplasia type 1 (MEN1) using a large nationwide cohort
Methods:
All patients with ZES diagnosed through the MEN1 AFCE/GTE network during the 1985-2015 period.
Results:
Among 233 ZES patients, 66 (28%) were operated for ZES-related gastrinoma(s). Thirty-three (51%) procedures aimed at removing gastrinoma(s) and associated pancreatic neuroendocrine tumor(s) (pNET(s)) by appropriate resection(s). Thirty-two procedures (49%) aimed at removing gastrinoma(s) alone (ZES group). Survival was decreased in metastatic patients at ZES diagnosis (p<0.001). Fifteen-year survival among non-metastatic patients was not significantly better in operated patients (82% (95% CI:67-90) versus 70% (95% CI:60-78)) (p=0.2). Operative mortality was nil. Metastatic lymph nodes were found in 30/42 lymphadenectomies (71%). The choice between duodenopancreatectomy versus duodenal focused surgery in the ZES group was associated with pre-operative detection of adenopathies (p>0.001), leading to more frequent lymphadenectomies (p<0.01). Previous pancreatic surgeries (30%) may have influenced the choice of ZES procedures.
Conclusions:
The high rate of invaded nodes in lymphadenectomies in MEN1 patients operated for ZES, the absence of operative mortality, and the decreased survival in metastatic patients are indirect arguments for surgery. Duodenopancreatectomy was chosen more frequently than duodenal focused surgery in case of suspected lymph nodes in the ZES group. Duodenopancreatectomy may be indicated in young and fit patients to prevent metastatic evolution in the ZES group. Removing gastrinoma(s) when associated with large pNET(s) is justified.