Surgical and molecular pathology of pancreatic neoplasms
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REVIEW
Open Access
Surgical and molecular pathology of pancreatic neoplasms Wenzel M. Hackeng1, Ralph H. Hruban2, G. Johan A. Offerhaus1 and Lodewijk A. A. Brosens1*
Abstract Background: Histologic characteristics have proven to be very useful for classifying different types of tumors of the pancreas. As a result, the major tumor types in the pancreas have long been classified based on their microscopic appearance. Main body: Recent advances in whole exome sequencing, gene expression profiling, and knowledge of tumorigenic pathways have deepened our understanding of the underlying biology of pancreatic neoplasia. These advances have not only confirmed the traditional histologic classification system, but also opened new doors to early diagnosis and targeted treatment. Conclusion: This review discusses the histopathology, genetic and epigenetic alterations and potential treatment targets of the five major malignant pancreatic tumors - pancreatic ductal adenocarcinoma, pancreatic neuroendocrine tumor, solid-pseudopapillary neoplasm, acinar cell carcinoma and pancreatoblastoma. Keywords: Pancreas, Pancreatic cancer, Acinar cell carcinoma, Pancreatic neuroendocrine tumor, Solid-pseudopapillary neoplasm, Genetics, Histology, Methylation, microRNA, Sequencing
Background Malignant neoplasms of the pancreas are currently classified based on the cellular direction of differentiation (ductal, acinar or neuroendocrine) of the neoplastic cells, combined with the macroscopic appearance (solid or cystic) of the tumors. Pancreatic ductal adenocarcinoma comprises about 90 % of all malignant pancreatic neoplasms. Of all other malignant pancreatic neoplasms (pancreatic neuroendocrine tumors, solid-pseudopapillary neoplasm, acinar cell carcinoma and pancreatoblastoma), neuroendocrine tumors are the most common, comprising approximately 5 % of malignant pancreatic tumors (Table 1). Recent genetic and epigenetic characterization of these histologically distinct pancreatic tumors has increased our understanding of common genetic signatures, and has also identified tumor specific genetic alterations (Table 2). In addition to serving as diagnostic tools, some genetic alterations can be exploited as targets for therapy, opening avenues for new treatments. In this review, histology, genetics and epigenetics of malignant * Correspondence: [email protected] 1 Department of Pathology, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands Full list of author information is available at the end of the article
pancreatic tumors and potential targets for treatment are discussed. Pancreatic ductal adenocarcinoma
Infiltrating ductal adenocarcinoma, also known as pancreatic ductal adenocarcinoma (PDAC), accounts for 90 % of all malignant pancreatic neoplasms and occurs at a mean age of 66 years [1]. PDAC has a very poor prognosis with an overall 5-year survival of only 7 % [2]. At diagnosis, the majority of patients are inoperable due to locally advanced or metastatic disease. The median survival for
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