天美传媒

Abstract

Pancreatic Neuroendocrine Neoplasms (PNENs) are rare, accounting for less than 3% of all pancreatic tumors. PNENs exhibit a wide spectrum of clinical behavior that has made classification and staging difficult. While the majority of PNENs are associated with relatively good survival, there can be significant variability in outcomes based on their biological heterogeneity. PNENs share a unique genetic identity, functional behavior and clinical course. Compared with tumors of the exocrine pancreas, they are rare and show a different biological behavior and prognosis. Some PNENs are associated with symptoms of hormone secretion, with increased systemic levels of insulin, gastrin, glucagon or other hormones. More commonly, PNENs are non-functional, without hormone secretion. Surgical resection is the mainstay of therapy, particularly for localized disease that must be tailored to tumor and clinical characteristics. Surgery is indicated in patients with PNENs to alleviate systemic symptoms do to hormone over production, compressive symptoms due to local mass effect and to prevent malignant transformation or dissemination. Small, incidental PNENs are increasingly managed non-operatively. Surgery may also be indicated in some instances of metastatic disease, if all metastatic foci may be removed.

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