Pancreatic neuroendocrine tumors: Challenges in an underestimated disease

•Surgery remains the gold-standard treatment in PanNET, regardless of stage.•Radiological, metabolic, serum and endoscopic platforms participate in PanNET diagnosis.•In the metastatic setting a plethora of treatment options are used when surgery cannot be performed.•Chemotherapy and targeted therapies are commonly used in metastatic setting.•In PanNET different molecular profiles exist, suggesting what kind of targeted therapy must be used.

Pancreatic neuroendocrine tumours (PanNETs) are considered a relatively unusual oncologic entity. Due to its relative good prognosis, surgery remains the goal standard therapy not only in localized disease but also in the setting of locally or metastatic disease. Most of the patients are diagnosed in metastatic scenario, where multidisciplinary approach based on surgery, chemotherapies, liver-directed and/or molecular targeted therapies are commonly used. Owing to a deeper molecular knowledge of this disease, these targeted therapies are nowadays widely implemented, being the likely discovery of predictive biomarkers that would allow its use in other settings. This review is focused on describing the different classifications, etiology, prognostic biomarkers and multidisciplinary approaches that are typically used in PanNET.