de Groen PC, Gores GJ, LaRusso NF, Gunderson LL, Nagorney DM (1999) Biliary tract cancers. N Engl J Med, 341(18):1368-1378
Introduction
In the United States, an estimated 20,000 new cases of liver and biliary tract cancer are diagnosed annually. 1 Biliary tract cancer is the second most common primary hepatobiliary cancer, after hepatocellular cancer. Approximately 7500 new cases of biliary tract cancer are diagnosed per year; about 5000 of these are gallbladder cancer, and between 2000 and 3000 are bile-duct cancers. 1 Biliary tract cancers have traditionally been divided into cancers of the gallbladder, the extrahepatic bile ducts, and the ampulla of Vater, whereas intrahepatic bileduct cancers have been classified as primary liver cancers. 2 The term “cholangiocarcinoma” was originally intended to refer only to primary tumors of the intrahepatic bile ducts and was not used for tumors of the extrahepatic bile ducts. 3Most cholangiocarcinomas involve the perihilar and distal extrahepatic bile ducts.
Gallbladder cancers are more frequent in women, 8 ,cholangiocarcinomas are slightly more common in men. 9 These sex differences are probably related to the higher incidence of gallstones in women and of primary sclerosing cholangitis in men. These are known risk factors for gallbladder cancer and cholangiocarcinoma, respectively.
Risk Factors
Cholangiocarcinoma, both intrahepatic and extrahepatic, is a well-known complication of primary sclerosing cholangitis. Although lifetime risks in excess of 30 percent have been reported among patients with primary sclerosing cholangitis, most studies mention lifetime risks of about 10 percent. 26-28 The time from the diagnosis of primary sclerosing cholangitis to the development of cholangiocarcinoma ranges from 1 year to more than 25 years, although at least one third of cases of cholangiocarcinoma are diagnosed within 2 years after the diagnosis of primary sclerosing cholangitis. 27,29 Patients who have ulcerative colitis in the absence of symptomatic primary sclerosing cholangitis or who have long-standing intraductal gallstone disease also have an increased risk. 27,30 Other, rarer conditions associated with the development of cholangiocarcinoma include bile-duct adenoma, multiple biliary papillomatosis, choledochal cysts, Caroli’s disease (cystic dilatation of intrahepatic bile ducts), and exposure to the radiopaque medium thorium dioxide (Thorotrast). 30
In Southeast Asia, infestation with the parasites Opisthorchis viverrini (in Thailand, Laos, and Malaysia) 31 or
Clonorchis sinensis (in Japan, Korea, and Vietnam) 32 is associated with an increase by a factor of 25 to 50 in the risk of cholangiocarcinoma. Case–control studies have shown an increased risk associated with smoking. 33,34
Diagnosis
The most common presenting symptoms of biliary tract cancer are caused by bile-duct obstruction and include jaundice, clay-colored stools, cola-colored urine, and pruritus. 7,46In general, pain, fatigue, malaise, and weight loss occur in advanced disease.
Positron-emission tomography (PET) permits the metabolism of bileduct epithelial cells to be assessed in vivo by means of a glucose analogue, [18F]fluoro-2-deoxy-D-glucose.82,83 Cholangiocarcinoma cells have a high glucose uptake. Both glucose and [18F]fluoro-2-deoxy-D-glucose are phosphorylated, but [18F]fluoro-2-deoxy-D-glucose is not further metabolized. As a result, cholangiocarcinoma cells accumulate [18F]fluoro-2-deoxy-D-glucose, causing “hot spots” on scanning. In addition, hepatocytes have high glucose-6- phosphatase activity and rapidly turn over [18F]fluoro-2-deoxy-D-glucose, thereby further increasing the signal-to-background ratio. Several small studies have documented the ability of PET to detect cholangiocarcinomas as small as 1 cm in diameter. 82,83
Other Palliative Treatments
The median survival varies from 6 to 12 months, with most centers reporting no patients surviving at 5 years.46,96,104,110
Introduction
In the United States, an estimated 20,000 new cases of liver and biliary tract cancer are diagnosed annually. 1 Biliary tract cancer is the second most common primary hepatobiliary cancer, after hepatocellular cancer. Approximately 7500 new cases of biliary tract cancer are diagnosed per year; about 5000 of these are gallbladder cancer, and between 2000 and 3000 are bile-duct cancers. 1 Biliary tract cancers have traditionally been divided into cancers of the gallbladder, the extrahepatic bile ducts, and the ampulla of Vater, whereas intrahepatic bileduct cancers have been classified as primary liver cancers. 2 The term “cholangiocarcinoma” was originally intended to refer only to primary tumors of the intrahepatic bile ducts and was not used for tumors of the extrahepatic bile ducts. 3Most cholangiocarcinomas involve the perihilar and distal extrahepatic bile ducts.
Gallbladder cancers are more frequent in women, 8 ,cholangiocarcinomas are slightly more common in men. 9 These sex differences are probably related to the higher incidence of gallstones in women and of primary sclerosing cholangitis in men. These are known risk factors for gallbladder cancer and cholangiocarcinoma, respectively.
Risk Factors
Cholangiocarcinoma, both intrahepatic and extrahepatic, is a well-known complication of primary sclerosing cholangitis. Although lifetime risks in excess of 30 percent have been reported among patients with primary sclerosing cholangitis, most studies mention lifetime risks of about 10 percent. 26-28 The time from the diagnosis of primary sclerosing cholangitis to the development of cholangiocarcinoma ranges from 1 year to more than 25 years, although at least one third of cases of cholangiocarcinoma are diagnosed within 2 years after the diagnosis of primary sclerosing cholangitis. 27,29 Patients who have ulcerative colitis in the absence of symptomatic primary sclerosing cholangitis or who have long-standing intraductal gallstone disease also have an increased risk. 27,30 Other, rarer conditions associated with the development of cholangiocarcinoma include bile-duct adenoma, multiple biliary papillomatosis, choledochal cysts, Caroli’s disease (cystic dilatation of intrahepatic bile ducts), and exposure to the radiopaque medium thorium dioxide (Thorotrast). 30
In Southeast Asia, infestation with the parasites Opisthorchis viverrini (in Thailand, Laos, and Malaysia) 31 or
Clonorchis sinensis (in Japan, Korea, and Vietnam) 32 is associated with an increase by a factor of 25 to 50 in the risk of cholangiocarcinoma. Case–control studies have shown an increased risk associated with smoking. 33,34
Diagnosis
The most common presenting symptoms of biliary tract cancer are caused by bile-duct obstruction and include jaundice, clay-colored stools, cola-colored urine, and pruritus. 7,46In general, pain, fatigue, malaise, and weight loss occur in advanced disease.
Positron-emission tomography (PET) permits the metabolism of bileduct epithelial cells to be assessed in vivo by means of a glucose analogue, [18F]fluoro-2-deoxy-D-glucose.82,83 Cholangiocarcinoma cells have a high glucose uptake. Both glucose and [18F]fluoro-2-deoxy-D-glucose are phosphorylated, but [18F]fluoro-2-deoxy-D-glucose is not further metabolized. As a result, cholangiocarcinoma cells accumulate [18F]fluoro-2-deoxy-D-glucose, causing “hot spots” on scanning. In addition, hepatocytes have high glucose-6- phosphatase activity and rapidly turn over [18F]fluoro-2-deoxy-D-glucose, thereby further increasing the signal-to-background ratio. Several small studies have documented the ability of PET to detect cholangiocarcinomas as small as 1 cm in diameter. 82,83
Other Palliative Treatments
The median survival varies from 6 to 12 months, with most centers reporting no patients surviving at 5 years.46,96,104,110
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