Ductal adenocarcinoma is the most common primary malignant disease of the pancreas and periampullary region. It accounts for more than 75% of all nonendocrine tumors arising in this region, which includes the pancreas, the ampulla of Vater, the distal common bile duct, and the perivaterian duodenum. Pancreatic adenocarcinoma is the most common of the periampullary neoplasms. In the United States, the annual incidence of pancreatic cancer is 9 cases per 100,000 population; it ranks eleventh among all cancers and ninth among nondermatologic cancers. It remains the most lethal cancer, with an overall 5-year survival rate of less than 3% and a death-to-incidence ratio of approximately 0.99. Because more than 28,000 persons in the United States are expected to die of pancreatic cancer in 2000, it is the fifth leading cause of f1l cancer deaths in this country. This chapter largely focuses on pancreatic adenocarcinoma.
Ampullary, distal common bile duct, and duodenal adenocarcinomas are less common than pancreatic tumors. They account for 15 to 20% of all periampullary malignant diseases. Arising in the same region, they are associated with similar clinical presentations and management algorithms, but these tumors have different survival rates. These entities are discussed briefly in this chapter. EPIDEMIOLOGY AND RISK FACTORS Demographic Patterns
The risk factors for pancreatic cancer have been well studied and are summarized in Table 5-1 . From 1920 to 1978, the incidence rates of pancreatic cancer in the United States increased nearly threefold, and they subsequently have plateaued. In nearly all European countries, a similar increase has been observed, with incidence rates that continue to rise. A portion of this apparent increase may have resulted from misclassification of pancreatic cancers as other cancers, particularly gastric cancer, early in the 1900s. However, several subsequent analyses have indicated that part of the increase is real, despite confounding factors.r2l r3l r4l
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