Neuroendocrine tumours of the ampulla of Vater: clinico-pathological features, surgical approach and assessment of prognosis

Abstract

Background/aimsNeuroendocrine 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.MethodsSix 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).ResultsPancreaticoduodenectomy 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.ConclusionThe 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.

Major and Minor Duodenal Papilla Neuroendocrine Tumors in Type 1 Neurofibromatosis: Case Report

“…Overall survival has been shown to be modified by local invasion and tumor size (>2 cm), 66 % and 52 % of ampullary NETs, respectively; however, our case had both local invasion and a diameter of 3 cm, not modifying survival [10,11].…”

Clinicopathological features and surgical outcomes of neuroendocrine tumors of ampulla of Vater

“…Nodal involvement appears to be of lesser significance to long-term survival [5, 6, 16, 22, 23]. Because a more advanced stage does not predict a worse prognosis, the TNM and ENETS staging systems are limited in predicting prognosis.…”

Nowotwory neuroendokrynne żołądka i dwunastnicy z uwzględnieniem gastrinoma (zasady postępowania rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

“…50-70% of neuroendocrine neoplasms of the ampulla of Vater are well-differentiated NENs (G1 and G2), unlike neuroendocrine neoplasms of the gallbladder, 90-100% of which are neuroendocrine cancers (G3) [15,16].…”