Pancreatic endocrine tumors (PETs) can either be functional, and associated with a specific clinical syndrome due to the hormone excess, or nonfunctional. Because all PETs, except insulinoma, are malignant in more than 50% of cases. PETs require treatment directed at both the tumor and the hormone excess state. This review focuses on advances reported within the past year. The following areas are covered: insights into the clinical aspects and genetic basis of PETs associated with various inherited diseases; advances in tumor localization; recent insights into tumor biological behavior, pathology, and possible genetic alterations associated with malignancy; recent insights or advances in the diagnosis or clinical course of individual syndromes; and advances in both surgical and medical treatment of localized and advanced disease.
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