Investigational agents for pancreatic neuroendocrine tumors: what clinical progress have we seen in the last 5 years?

Introduction Pancreatic neuroendocrine tumors (pNETs), a group of endocrine tumors that arise in the pancreas from the hormonal cells of the islets of Langerhans, are among the most common gastroenteropancreatic neuroendocrine tumors. The incidence of pNETs has increased in recent years to about 1.5 per 100,000 population and prognosis correlates with histologic grade.This review aims to provide an overview of the newly approved or currently developing drugs over the past five years that have shown a significant improvement in prognosis for patients affected by this rare disease.