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• W2596677932 abstract "Overview The diffuse neuroendocrine system (DES) is represented by a small number of cells spread through the body, and the tumors that derive from these cells present a wide spectrum of epidemiologic, pathologic, biologic, genetic, and clinical features. Clinical and scientific investigation of the inherited multiple endocrine neoplasia (e.g., MEN‐1, MEN‐2) syndromes, and the various unique clinical syndromes secondary to secretion of specific peptides (e.g., insulinomas, glucagonomas, VIPomas) are challenging. Clinical presentation can be dramatic, nonspecific, or incidentally noted in absence of symptoms. Tumors might be small, difficult to detect tumors (<1 cm in size) or bulky hepatic metastases in a well patient. Cancer registry data show five‐year survivals for tumors of the neuroendocrine system that have not improved in the past several decades, and that remain about 30–60%. The same registry data documents a rising and unexplained incidence of these tumors, particularly the neuroendocrine tumors of the gastroenteropancreatic system (GEP‐NETs), that when coupled with the long average survival results in prevalence figures similar to cancers such as testicular, ovarian, and multiple myeloma. Recent advances in understanding of the genetics, biology, clinical features, and response to therapy are better defining subtypes of neuroendocrine tumors once clustered and studied as “carcinoids.” Revised histologic grouping and staging are informing ongoing clinical trials and are providing more informed clinical care of these diverse tumors. Recent approvals of several targeted therapies with progression‐free survival benefit suggest that the status quo is changing for tumors of the DES." @default.
• W2596677932 created "2017-03-23" @default.
• W2596677932 creator A5041297405 @default.
• W2596677932 date "2017-02-26" @default.
• W2596677932 modified "2023-09-25" @default.
• W2596677932 title "Tumors of the Diffuse Neuroendocrine and Gastroenteropancreatic System" @default.
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