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Pancreatic cancer is a relatively rare cancer type, but a major cause of cancer-related death because there are quite rare histologically proven long-time survivors of pancreatic cancer. The main reasons for the worse prognosis are: late clinical presentation, aggressive biology, and failure of surgical and systemic treatment. The aim of this introductory chapter is to provide an update on the known causes, clinical presentations, and most current management strategies of pancreatic carcinoma.
Pancreatic adenocarcinoma comprises only 3% of estimated new cancer cases each year but with 44,030 new cases and 37,660 deaths expected in 2011 is the fourth most common cause of cancer mortality . The annual incidence rate of pancreatic cancer is approximately 8/100,000 persons worldwide. Adenocarcinoma is the most frequent type of pancreatic cancer. Others are endocrine and cystic tumors, which have a different, mostly better, prognosis . There are established risk factors for developing pancreatic cancer including chronic pancreatitis, increased age, family history, smoking, and diabetes . Obesity and physical activity have been implicated in pancreatic cancer etiology.
Ninety-five percent of pancreatic cancers originate from the exocrine portion of the gland. A proposed mechanism for the development of invasive pancreatic adenocarcinoma is a stepwise progression through genetically and histologically well-defined noninvasive precursor lesions, called pancreatic intraepithelial neoplasias (PanINs). They are microscopic lesions in small (less than 5 mm) pancreatic ducts and are classified into three grades (PanIN 1–3). The understanding of molecular alterations in PanINs has provided rational candidates for the development of early detection biomarkers and therapeutic targets. Another precursor of invasive pancreatic carcinomas is pancreatic intraductal papillary mucinous neoplasia (IPMN). IPMNs belong to the increasingly diagnosed and treated group of cystic tumors. They progress from a benign intraductal tumor through increasing grades of dysplasia to invasive adenocarcinoma and therefore provide models of neoplastic pancreatic progression . Other tumor types within the pancreas are endocrine tumors and a variety of rare pancreatic tumors like acinar cell carcinoma ( Table 1.1 ) .
Pancreatic Exocrine Cancers | Pancreatic Endocrine Neuroendocrine Tumor |
---|---|
Pancreatic ductal adenocarcinoma | Nonfunctional islet cell tumor insulinoma |
Intraductal papillary mucinous neoplasia (IPMN) | Gastrinoma |
Mucinous cystadenocarcinoma | Glucagonoma |
Adenosquamous carcinoma | Somatostatinoma |
Solid pseudopapillary tumors | Vasoactive intestinal peptide releasing tumor (VIPoma) |
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