Biology and Systemic Treatment of Advanced Gastroenteropancreatic Neuroendocrine Tumors

Neuroendocrine neoplasms (NENs) represent a heterogeneous group of neoplasms arising from the diffuse neuroendocrine cell system. Although these tumors can originate anywhere in the body, they primarily arise in either the lung or gastroenteropancreatic (GEP) tract. For many patients, the disease can display a less aggressive biology compared with other solid tumors, growing slowly over a period even of many years. Despite their rarity, the incidence and prevalence of neuroendocrine tumors (NETs) is rising, and NETs are now the second most prevalent advanced gastrointestinal cancer after colorectal cancer.1,2 In recent years, important strides have been made in drug development, following the completion of rigorous phase III clinical trials.3-7 This burgeoning interest in NET clinical research has occurred in parallel with advances in NET translational medicine—specifically, an improvement in our understanding of the genetics through use of sequencing technology. Although few actionable alterations have been identified, sequencing can provide important information that can be used in concert with NET pathologic classification for disease prognostication and treatment decisions. In this review, we will discuss current NET classification and available drug therapies, recent advances in NET translational medicine, use of radiolabeled therapies for NETs, and best practices for sequencing the available systemic treatments. PATHOLOGY Section: ChooseTop of pageAbstractPATHOLOGY <