Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors Carinne W. Anderson, MD*, Joseph J. Bennett, MD KEYWORDS Pancreatic neuroendocrine tumor Nonfunctional pancreatic neuroendocrine tumor Insulinoma Gastrinoma Glucagonoma VIPoma Somatostatinoma KEY POINTS Pancreatic neuroendocrine tumors are a rare group of neoplasms, most of which are nonfunctioning. Functional pancreatic neoplasms secrete hormones that produce unique clinical syndromes. The key management of these rare tumors is to first suspect the diagnosis; to do this, cli- nicians must be familiar with their clinical syndromes. Pancreatic neuroendocrine tumors (PNETs) are a rare group of neoplasms that arise from multipotent stem cells in the pancreatic ductal epithelium. Most PNETs are nonfunctioning, but they can secrete various hormones resulting in unique clinical syn- dromes. Clinicians must be aware of the diverse manifestations of this disease, as the key step to management of these rare tumors is to first suspect the diagnosis. In light of that, this article focuses on the clinical features of different PNETs. Surgical and medical management will not be discussed here, as they are addressed in other arti- cles in this issue. EPIDEMIOLOGY Classification PNETs are classified clinically as nonfunctional or functional, based on the properties of the hormones they secrete and their ability to produce a clinical syndrome. Nonfunctional PNETs (NF-PNETs) do not produce a clinical syndrome simply because they do not secrete hormones or because the hormones that are secreted do not The authors have nothing to disclose. Department of Surgery, Helen F. Graham Cancer Center, 4701 Ogletown-Stanton Road, S-4000, Newark, DE 19713, USA * Corresponding author. E-mail address:
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