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

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Abstract

Purpose

Ampullary 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.

Methods

Clinical 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).

Results

Eighteen patients (9 M/9 F, averaging 62 years) 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 1.5 cm), and 11 (61%) patients underwent PD (median tumour size 2.4 cm). The median operation time of ampullectomy/excision was 221 min with operative blood loss of 75 ml (vs. 506 min and 425 ml in PD). The median hospital stay was 10 days 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 14 months) after an R0 PD. Disease-related survival was 93, 77 and 66% at 1, 3 and 5 years, respectively.

Conclusion

Ampullary NENs are mostly G1–G2 neoplasms. Lymph node metastases rarely occur in G1 NENs < 2 cm in size, which may be treated with ampullectomy/excision. Survival is 66% 5 years after surgery.

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Author information

Authors and Affiliations

  1. Clinica Chirurgica 1, Pancreatic and Endocrine Digestive Surgical Unit, University of Padua, Padua, Italy

    A. C. Milanetto, C. Pasquali & M. Da Broi

  2. Pathology Unit, Humanitas Research Hospital, Humanitas University, Rozzano, Milan, Italy

    T. Brambilla

  3. Pancreatic Surgery Unit, Humanitas Research Hospital, Humanitas University, Rozzano, Milan, Italy

    G. Capretti & A. Zerbi

Authors
  1. A. C. Milanetto
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  2. C. Pasquali
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  4. T. Brambilla
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  6. A. Zerbi
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Contributions

Study conception and design: Milanetto A.C., Pasquali C., Zerbi A. Acquisition of the data: Milanetto A.C., Da Broi M., Brambilla T., Capretti G. Analysis and interpretation of the data: Milanetto A.C., Da Broi M., Brambilla T., Capretti G. Drafting of the manuscript: Milanetto A.C. Critical revision of the manuscript: Pasquali C., Zerbi A.

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Correspondence to A. C. Milanetto.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The research ethics committee of Humanitas Research Hospital (Rozzano, Milan, Italy) approved the retrospective study (15/2018).

Informed consent

Informed consent was obtained from all individual participants included in the study.

Additional information

This paper was partially (series from the University of Padua) presented as an abstract at the European Pancreatic Club (EPC) Meeting in 2017.

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Milanetto, A.C., Pasquali, C., Da Broi, M. et al. Ampullary neuroendocrine neoplasms: surgical experience of a rare and challenging entity. Langenbecks Arch Surg 403, 581–589 (2018). https://doi.org/10.1007/s00423-018-1695-9

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