title: Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis.
abstract: OBJECTIVE
  Neuroendocrine tumours occur very rarely in the ampulla of Vater and their clinical behaviour is unknown. The aim of this study is to assess the clinico-pathological features, surgical approach and prognosis of these patients.
  
  
  METHODS
  Six patients with neuroendocrine tumours of the ampulla of Vater treated with curative intent surgery at a single centre were retrospectively analysed. A univariate analysis of potential prognostic factors was also performed (data provided from the present study and literature review).
  
  
  RESULTS
  Pancreaticoduodenectomy was curative in all the patients. Overall and disease-free survival rates were significantly better for G1/G2 tumours (p = 0.006 and p = 0.004, respectively). Although frequent, lymph node metastases did not influenced both overall (p = 0.760) and disease-free survival rates (p = 0.745). No significant differences of survival were observed in patients with ENETS stage I/II disease, as compared to ENETS stage III disease (p = 0.169 and p = 0.137, respectively). No differences were observed according to UICC staging system (p = 0.073 and p = 0.177, respectively). Tumours that are less than 2 cm or limited to the ampulla appear to have a better prognosis.
  
  
  CONCLUSIONS
  The WHO 2010 classification appear to accurately predict patient prognosis, while the ENETS or UICC staging systems have a limited value (especially in regard to lymph node metastases). Radical surgery (i.e. pancreaticoduodenectomy with lymphadenectomy) should be the standard approach in most patients with NET of the ampulla of Vater because this procedure removes all the potential tumour-bearing tissue.
authors.affiliation: Department of Surgery, Center of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Fundeni Street no 258, Bucharest, Romania. traian.dumitrascu@srchirurgie.ro
authors.forename: Traian
authors.initials: T
authors.lastname: Dumitrascu
authors.forename: Simona
authors.initials: S
authors.lastname: Dima
authors.forename: Vlad
authors.initials: V
authors.lastname: Herlea
authors.forename: Victor
authors.initials: V
authors.lastname: Tomulescu
authors.forename: Mihnea
authors.initials: M
authors.lastname: Ionescu
authors.forename: Irinel
authors.initials: I
authors.lastname: Popescu
mesh_terms: D000328:Adult; D000368:Aged; D014670:Ampulla of Vater / Q000473:pathology* / Q000601:surgery; D000704:Analysis of Variance; D003138:Common Bile Duct Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D053208:Kaplan-Meier Estimate; D008198:Lymph Nodes / Q000473:pathology*; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D018358:Neuroendocrine Tumors / Q000401:mortality / Q000473:pathology* / Q000601:surgery*; D016577:Pancreaticoduodenectomy / Q000379:methods; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012494:Sampling Studies; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed / Q000379:methods