Optimising therapeutic options for patients with advanced pancreatic neuroendocrine tumours

Pancreatic neuroendocrine tumours (pNETs) are a rare form of pancreatic cancer. Several therapeutic options exist for pNETS; however, there is no algorithm to determine the optimum sequence of therapies. Approved treatments for pNETs include somatostatin analogues (SSAs), streptozocin-based chemotherapy and targeted therapies such as everolimus and sunitinib. Unapproved therapies include systemicpeptide receptor-targeted radiotherapy (PRRT), temozolomide-based chemotherapy, liver resection, liver transplantation, hepatic artery embolisation with or without chemotherapy and selective internal radiation therapy (SIRT). An individualised approach to the treatment of pNETs is described. Firstly, it is necessary to decide whether it is appropriate to treat at all. For those with symptoms, it is necessary to define the treatment goal: symptomatic or oncological control. Symptoms may direct treatment decisions; for example in patients with hypogycaemia, everolimus would be the most effective therapy. In high-volume disease where tumour reduction is the highest priority, streptozocin-based chemotherapy would be a more appropriate choice. For patients with disease progression and a moderate-to-high tumour volume, targeted therapy is the preferred choice. Following the failure of first-line therapies, second-line options include other targeted agents and cytotoxic chemotherapy. PRRT is recommended only after failure of prior therapy. Treatment decisions of pNETs should be made in a patient-oriented manner and on a case-by-case basis.