Surgical Management of Neuroendocrine Tumours of the Pancreas

Abstract

Neuroendocrine tumours of the pancreas (pNET) are rare, accounting for 1-2% of all pancreatic neoplasms. They develop from pancreatic islet cells and cover a wide range of heterogeneous neoplasms. While most pNETs are sporadic, some are associated with genetic syndromes. Furthermore, some pNETs are 'functioning' when there is clinical hypersecretion of metabolically active peptides, whereas others are 'non-functioning'. pNET can be diagnosed at a localised stage or a more advanced stage, including regional or distant metastasis (in 50% of cases) mainly located in the liver. While surgical resection is the cornerstone of the curative treatment of those patients, pNET management requires a multidisciplinary discussion between the oncologist, radiologist, pathologist, and surgeon. However, the scarcity of pNET patients constrains centralised management in high-volume centres to provide the best patient-tailored approach. Nonetheless, no treatment should be initiated without precise diagnosis and staging. In this review, the steps from the essential comprehensive preoperative evaluation of the best surgical approach (open versus laparoscopic, standard versus sparing parenchymal pancreatectomy, lymphadenectomy) according to pNET staging are analysed. Strategies to enhance the short- and long-term benefit/risk ratio in these particular patients are discussed.

Keywords: lymph node metastasis; lymphadenectomy; multidisciplinary team meeting; pancreatectomy; pancreatic neuroendocrine tumour; pancreatic sparing surgery.

Figure 1

Description of the various factors that should be considered planning the surgical management of patients with pNET.

Figure 2

“Diagnose”, “locate“, “stage” and ‘grade” as accurately as possible the tumor.

Figure 3

Glucagon-like peptide-1 receptor (GLP-1R) PET/CT locating a 7 mm insulinoma, latter enucleated.

Figure 4

Description of the main surgical indications for pNETs.

Figure 5

Summary of the indications of standard surgery or pancreatic sparing surgery for pNETs.