Pancreatic neuroendocrine tumors (PNETs) are on the increase. Functional tumors including gastrinoma and insulinoma cause well described clinical syndromes. Non-functional tumors are found incidentally or by direct tumor effects. A third category of tumor secretes hormone(s) at a subclinical level without producing a syndrome. When metastatic PNETs may be indolent for several years but progression is inevitable. In this chapter recent advances in the pathophysiology, diagnosis, and management of these tumors are reviewed and placed in historical context. Tumor markers remain essential in the diagnosis and follow-up of these patients. Major clinical advances have occurred in pathology/classification/staging, imaging (68 Gallium DOTATE PET), the development of additional somatostatin analogues, cytotoxic chemotherapy, targeted therapies (e.g. tyrosine kinase inhibitor sunitinib and mTOR inhibitor everolimus), other modalities (e.g. peptide receptor radiotherapy), and quality of life assessment. These are very hopeful times for patients who have these tumors and their physicians. Issues to be considered when choosing among the plethora of effective treatment options include toxicity and cost, effects on quality of life, and the age and overall health of the patient. Treatment should be coordinated by an experienced multidisciplinary team. Many unanswered questions remain including the optimal treatment sequencing. For complete coverage of this and related aspects of Endocrinology, please visit our FREE web-book, www.endotext.org.
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