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- W2792636795 abstract "Neuroendocrine tumors of the pancreas (PanNET) are rare and data on their epidemiology are limited. It is estimated that they possibly account for <3% of all pancreatic tumors, and approximately 4–8% of all neuroendocrine tumors. There has been a steady rise in the incidence of PanNET over the past few decades. The PanNET have a highly variable clinical presentation, malignant potential, and prognosis. They can be functional (associated with a hormone-secreted clinical syndrome) or nonfunctional. Less than 10% of PanNET are associated with inherited disorders, including multiple endocrine neoplasia, von Hippel–Lindau syndrome, neurofibromatosis type 1, and tuberous sclerosis complex. The classification of these rare tumors has evolved gradually since the early 1960s, and over time have become relatively complex. In order to overcome this limitation, the European Neuroendocrine Tumor Society (ENETS) proposed a tumor–node–metastasis (TNM) staging system in 2006 that also included a grading system. Subsequently, the World Health Organization (WHO) introduced a classification system in 2010 that endorsed and included the proliferation-based grading system proposed by the ENETS together with the classical histological based classification. The WHO recognized the need for a separate staging system and, together with the American Joint Cancer Committee (AJCC), adopted the TNM staging system proposed in 2009 by the International Union for Cancer Control (UICC/AJCC/WHO 2010 TNM). The PanNET were thus classified based on the WHO 2010 classification system into neuroendocrine tumors (grade 1 or grade 2) or neuroendocrine carcinomas (grade 3) according to their proliferative rate substantiated by the Ki-67 labeling index. They should also be staged, using one of the two existing TNM staging systems. Further modifications in the classification system can be expected because the current grading system does not clearly distinguish between grade 2 at the extreme edge of the range and low proliferative grade 3 carcinomas; moreover, the existence of two parallel TNM staging systems that describe slightly different extents of disease while using the same TNM terminology is confusing." @default.
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- W2792636795 date "2018-02-12" @default.
- W2792636795 modified "2023-10-14" @default.
- W2792636795 title "Epidemiology and Classification of Neuroendocrine Tumors of the Pancreas" @default.
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- W2792636795 doi "https://doi.org/10.1002/9781119188421.ch121" @default.
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