Abstract

Simple SummaryIn this narrative review, we update the surgical management of pancreatic neuroendocrine tumours (pNETs) and highlight key elements in view of the recent literature. These tumours are rare and suffer from a lack of data and randomized controlled trials. The pNETs management is difficult due to their heterogeneity and the risks associated with pancreatic surgery. Innovative managements such as “watch and wait” strategies, parenchymal sparing surgery and minimally invasive approach are emerging. The correct use of all these therapeutic options requires a good selection of patients but also a constant update of knowledge.Pancreatic neuroendocrine tumours (pNETs) represent 1 to 2% of all pancreatic neoplasm with an increasing incidence. They have a varied clinical, biological and radiological presentation, depending on whether they are sporadic or genetic in origin, whether they are functional or non-functional, and whether there is a single or multiple lesions. These pNETs are often diagnosed at an advanced stage with locoregional lymph nodes invasion or distant metastases. In most cases, the gold standard curative treatment is surgical resection of the pancreatic tumour, but the postoperative complications and functional consequences are not negligible. Thus, these patients should be managed in specialised high-volume centres with multidisciplinary discussion involving surgeons, oncologists, radiologists and pathologists. Innovative managements such as “watch and wait” strategies, parenchymal sparing surgery and minimally invasive approach are emerging. The correct use of all these therapeutic options requires a good selection of patients but also a constant update of knowledge. The aim of this work is to update the surgical management of pNETs and to highlight key elements in view of the recent literature.

Highlights

  • Pancreatic neuroendocrine tumours are a group of rare neoplasms with an incidence of 1–2/100,000 inhabitants/year

  • PNETs are divided into two categories depending on clinical presentation due to hypersecretion of hormones [2]: the non-functional Pancreatic neuroendocrine tumours (pNETs) (NF-pNETs) that represent 60–90% of pNETs are frequently diagnosed as incidentaloma (82%) [3] vs. the functional pNETs (F-pNETs) (Table 1)

  • This review aims to describe the main principles of surgical management of patients with pNETs, incorporating recent literature and highlighting some key points and grey areas

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Summary

Introduction

Pancreatic neuroendocrine tumours (pNETs) are a group of rare neoplasms with an incidence of 1–2/100,000 inhabitants/year. 25% pancreatic surgery is not systWemhiaptpiclea’ns dtrimaday(Seyvmenptboemdaetilcetheyripoougsl.yTcaheemfiiraswt chaesree concerns the small (≤2 cm), In(sInusliunloinm) awspaoirtr”aoedfssitttcorharaetansetegdioysnay)smoiysfmpantnpootmroompmst)oi,agoctlnioycncvGafaue1lsmidiNoiaanFte,-rpdebNsebuhEylatTvstshii,onefroEathrluewcrhodhapiinsecaghapenapsN,esvaiemriusauprnoalceelensudrov>ce9rii9ln%laenTcuem(“owuartScohcaientdy (ENETS), the US National Ctoromupbrlehs,ecnosmivea Cancer Network (NCCN), and the gastro-enteroponantchreeabteWinc eDnfieHtu-Arriossekynnbddaolrcaornminceeao(ncwfehosaluotperlrhgaysyemrddyirs.aiarIEnr)ShMfeaacO,t,h[iy5np–m1o1ko]a.sltTemshuiisrag,diecacilssioernie9iss0,%btahseerde in particular is still a nonnegligible risk of lymph node involvement for these tumours, about 10% [12], but which should be compared with the significant morbidity, mortality and functional consequences of pancreatic surgery [13]. NF-pNETs A surgical indication is established for NF-pNETs larger than >2 cm, symptomatic and/or associated with biliary or pancreatic ductal dilatation In these situations, the risk of metastases becomes significant and justifies pancreatectomy [5,15]. During and several months after surgery, patients need high doses of PPIs to efficiently control it

Insulinoma
Glucagonoma
VIPoma
NF-pNETs
Gastrinoma
Others F-pNETs
Surgical Approach
Cholecystectomy and NETs
Lymph Nodes Removal and NETs
Place of Surgery in Metastatic Disease?
Resection of the Primary pNET in Patients with Liver Metastases
Liver Transplantation
Surgery in Unresectable pNETs
Follow-up of pNETs after Resection
Findings
Conclusions
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