Patient characteristics and clinical outcomes following initial surgical intervention for MEN1 associated pancreatic neuroendocrine tumours: A systematic review and exploratory meta-analysis of the literature

Pancreatology. 2019 Apr;19(3):462-471. doi: 10.1016/j.pan.2019.03.002. Epub 2019 Mar 12.

Abstract

Background: This systematic review aimed to define the outcomes of different pancreatic resection procedures for multiple endocrine neoplasia type 1 (MEN1) associated pancreatic neuroendocrine neoplasms (pNENs).

Methods: A search of PubMed, MEDLINE and SCOPUS databases were performed in accordance with PRISMA guidelines.

Results: Twenty-seven studies including 533 patients undergoing initial pancreatic resection for MEN1 associated pNENs were included in this systematic review. Three hundred and sixty-six (68.7%) distal pancreatectomies (DP), 120 (22.5%) sole enucleations (SE) and 47 (8.8%) pancreaticoduodenectomies (PD) were identified. SE was associated with a higher rate of recurrence than DP (25/67, 37% vs 40/190, 21% respectively, P = 0.008) but a lower rate of endocrine insufficiency than PD (1/20, 5% vs 8/21, 38% respectively, P = 0.010). A meta-analysis of major pancreatic resections (PD or DP) vs SE in 15 studies showed that SE is associated with an increased rate of recurrence (Major resection 42/184, 23% vs SE 20/53, 38% RR 0.65 CI 0.43-0.96 P = 0.032) but reduced rate of postoperative endocrine insufficiency (Resection 37/93, 40% vs SE 0/24, 0% RR 7.37 CI 1.57-34.64 P = 0.008). Similarly, insulinomas and functional pNENs overall had lower rates of recurrence and reoperation with major resection. There was no difference in the reoperation rates or survival outcomes after SE compared with major pancreatic resections at follow-up (pooled overall mean duration: 85 months).

Conclusion: Major pancreatic resections for MEN1 associated pNENs have a lower risk of recurrence and a higher risk of postoperative endocrine insufficiency when compared to sole enucleation, but a similar rate of reoperation and survival.

Keywords: Multiple endocrine neoplasia type 1; Neuroendocrine tumour; Surgery.

Publication types

  • Systematic Review

MeSH terms

  • Gene Expression Regulation, Neoplastic*
  • Humans
  • Neuroendocrine Tumors / metabolism*
  • Pancreatectomy
  • Pancreatic Neoplasms / metabolism*
  • Proto-Oncogene Proteins / metabolism*

Substances

  • MEN1 protein, human
  • Proto-Oncogene Proteins