ReviewsThe Molecular and Clinical Landscape of Pancreatic Neuroendocrine TumorsBatukbhai, Bhavina D.O. MD*; De Jesus-Acosta, Ana MD†Author Information From the *Department of Hematology and Oncology, Dartmouth Hitchcock Medical Center, Lebanon, NH; and †Department of Oncology, Kimmel Comprehensive Cancer Center at Johns Hopkins, Baltimore, MD. Received for publication December 12, 2017; accepted August 30, 2018. Address correspondence to: Ana De Jesus-Acosta, MD, Department of Oncology, Kimmel Comprehensive Cancer Center at Johns Hopkins Hospital, 401, North Broadway, Baltimore, MD 21231 (e-mail: [email protected]). The authors declare no conflict of interest. Pancreas: January 2019 - Volume 48 - Issue 1 - p 9-21 doi: 10.1097/MPA.0000000000001189 Buy Metrics Abstract Pancreatic neuroendocrine tumors are rare tumors of the pancreas originating from the islets of the Langerhans. These tumors comprise 1% to 3% of all newly diagnosed pancreatic cancers every year and have a unique heterogeneity in clinical presentation. Whole-genome sequencing has led to an increased understanding of the molecular biology of these tumors. In this review, we will summarize the current knowledge of the signaling pathways involved in the tumorigenesis of pancreatic neuroendocrine tumors as well as the major studies targeting these pathways at preclinical and clinical levels. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.