Neuroendocrine neoplasms (NENs) are a heterogeneous family of tumours of increasing incidence and concern. The appropriate systemic therapy to apply in advanced/metastatic setting needs to be validated after a multidisciplinary meeting, because of the many characteristics to consider, such as stage (TNM), histological grade (Grade 1 to 3), functional imaging (FDG-PET) and somatostatin-receptor imaging (SRI), primary organ of origin, hormone-induced clinical symptoms, tumour bulk and of course general condition of the patient. Here, systemic therapies, including somatostatin analogues (SSA), targeted therapies, chemotherapy and peptide receptor radioligand therapy (PRRT) and excluding loco-regional therapies such as selective internal radiation therapy, are discussed for the treatment of advanced/metastatic neuroendocrine carcinomas (NEC), neuroendocrine tumours (NET) from pancreatic origin (PanNET) or small intestinal origin (SI-NET).

(BELG J MED ONCOL 2021;15(7):351-6)