Systemic treatment of pancreatic neuroendocrine tumors

Abstract

Pancreatic neuroendocrine tumours (pNET) are a group of heterogeneous tumours. Despite being rare, their incidence is rising. While localized lesions can be cured with surgical resection, most patients present with metastatic disease. Systemic therapy is thus integral for the management of this rare entity. Numerous advancements have been made in the past decade in the systemic treatment of unresectable, metastatic pNET. Treatment options for pNET include somatostatin analogues, cytotoxic chemotherapy, targeted agents and peptide receptor radionuclide therapy. In this Review, we present the latest evidence for these treatment options.