%0 Journal Article
%T Biology and Systemic Treatment of Advanced Gastroenteropancreatic Neuroendocrine Tumors
%A Jonathan Strosberg
%A Nicola Fazio
%A Nitya Raj
%J About the Ed Book | ASCO Educational Book
%D 2018
%R https://doi.org/10.1200/EDBK_200893
%X 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¡ªspecifically, 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 <<NET MOLECULAR BASISNET THERAPY: KEY ADVANCES...NUCLEAR MEDICINE AND PEPT...A ROLE FOR BIOMARKERS IN ...SEQUENCING TREATMENT IN N...References The World Health Organization (WHO) GEP-NEN pathologic classification is a pivotal tool to predict tumor behavior. The WHO criteria classify NENs by cell architecture (well and poorly differentiated) and proliferative index as measured by Ki-67 and/or mitotic count (grade 1, low; grade 2, intermediate; and grade 3, high).8 Well-differentiated NENs are defined as ¡°tumors¡± (NETs), whereas poorly differentiated NENs are defined as neuroendocrine ¡°carcinomas¡± (NECs). To date, the WHO classification system has been the most important prognostic tool. However, there are limitations with the classification schema particularly with respect to the grade 3 category, because more recent data demonstrate distinct patterns of response to treatment as well as divergent outcomes for grade 3 NETs compared with grade 3 NECs. In a study of both well- and poorly differentiated grade 3 GEP-NENs, poorer response rates (despite longer survival) to platinum-based
%U http://ascopubs.org/doi/full/10.1200/EDBK_200893