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Periampullary cancers are composed of a group of malignant neoplasms arising in the region of the ampulla of Vater. These include mainly adenocarcinomas of the head of the pancreas, ampulla of Vater, distal bile duct (cholangiocarcinoma), and duodenum. Less commonly, acinar cell carcinomas or pancreatic endocrine neoplasms occur in the periampullary region of the pancreas. Periampullary cancers are often discussed as a group based on their similar presentation, workup, and surgical management. In addition, pancreas cancer is also discussed with this group since the natural history for both proximal and distal pancreatic lesions is similar—differing mainly in the type of resection performed.

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The first successful resection of a periampullary tumor was performed by Halsted in 1898. He described a local ampullary resection with reanastomosis of the pancreatic and bile ducts to the duodenum in a patient who presented with obstructive jaundice.1 In the early part of the 20th century, most periampullary cancers were managed by a transduodenal approach similar to that first reported by Halsted. Codivilla is often credited with performing the first en bloc resection of the head of the pancreas and duodenum for periampullary carcinoma, but this patient did not survive beyond the early postoperative period.2 The first successful two-stage pancreaticoduodenectomy was performed in Germany by Kausch in 1909.3 In 1914, Hirschel reported the first successful one-stage pancreaticoduodenectomy.4 Pancreaticoduodenectomy was not popularized until Whipple and colleagues reported three successful, two-stage, en bloc resections of the head of the pancreas and the duodenum in 1935.5 Over the next decade, a number of modifications and technical refinements were made in the procedure, including the first one-stage pancreaticoduodenectomy, reported in the United States by Trimble in 1941. The procedure was infrequently performed despite technical advances, until the 1980s because of the formidable operative morbidity, mortality, and the poor prognosis associated with periampullary cancers.

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Currently, the resection of periampullary cancer with a pancreaticoduodenectomy is performed routinely at many referral centers and carries a mortality of approximately 2%. Moreover, significant advances have been made in understanding of the pathogenesis, biology, and staging of periampullary carcinoma in the past two decades.

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Periampullary carcinomas are a major public health concern throughout the world. Pancreatic cancer is the fourth leading cause of cancer death in the United States. In 2009, there were an estimated 35,240 deaths in the United States compared to 159,390 deaths for lung cancer, 49,920 for colorectal cancer, and 40,610 for breast cancer.6 The incidence of pancreatic carcinoma rose dramatically from the 1930s until the mid-1970s, nearly doubling during this time period. Since 1973, the incidence in the United States has remained stable at about 8–9 per 100,000 of population. The incidence in Western Europe is similar to that in the United States and has also remained stable during the past three decades. In Europe, pancreatic cancer is the sixth leading cause of cancer death. In Japan, however, a dramatic increase has been observed during ...

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