Ampullary neuroendocrine neoplasms: surgical experience of a rare and challenging entity

PurposeAmpullary neuroendocrine neoplasms (NENs) account for <0.3% of gastrointestinal NENs. Surgical options include transduodenal ampullectomy/tumour excision or pancreaticoduodenectomy (PD). We report the experience of two high-volume pancreatic surgical centres of ampullary NENs.MethodsClinical records of patients who underwent surgery for ampullary NENs (January 2007-November 2017) in the study centres were retrieved retrospectively. We evaluated clinical-pathological features, post-operative outcome and follow-up (FU).ResultsEighteen patients (9 M/9 F, averaging 62years) were enrolled. All but one were non-functioning NENs; four (22%) patients presented with jaundice. Seven (39%) of the patients underwent ampullectomy/excision (median tumour size 1.5cm), and 11 (61%) patients underwent PD (median tumour size 2.4cm). The median operation time of ampullectomy/excision was 221min with operative blood loss of 75ml (vs. 506min and 425ml in PD). The median hospital stay was 10days in both groups. Overall surgical morbidity was 33%, due to four biochemical leaks, one pancreatic fistula and one abdominal haemorrhage. No reoperations were needed. The median tumour size was 1.8 (range 0.5-6.7)cm. All G2-G3 NENs were N1 (vs. 1/7 in G1 NENs). Three (17%) cases were mixed exocrine/G3 NECs. After a median FU of 45 (up to 124)months, recurrence occurred in four G3 NEC (31%) patients (median disease-free survival 14months) after an R0 PD. Disease-related survival was 93, 77 and 66% at 1, 3 and 5years, respectively.ConclusionAmpullary NENs are mostly G1-G2 neoplasms. Lymph node metastases rarely occur in G1 NENs <2cm in size, which may be treated with ampullectomy/excision. Survival is 66% 5years after surgery.