Ch. 54 – Biliary System

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1582 Section X Abdomen Survival 1.0 0.8 0.6 0.4 0.2 0.0 0 12 24 36 48 60 Months Figure 54-34 Survival following surgical resection for T2 gallbladder cancer. Patients undergoing radical resection (triangles) are compared with patients undergoing simple cholecystectomy (circles) (P > .05). (From Fong Y, Jarnigan W, Blumgart LH: Gallbladder cancer: Comparison of patients presenting initially for defi nitive operation with those presenting after prior noncurative intervention. Ann Surg 232:557-569, 2000.) in 10-year survival has been demonstrated after simple cholecystectomy (100%) and extended cholecystectomy (75%) among patients with T1b gallbladder cancer. Invasion into the subserosa (T2) increases the risk for regional lymph node metastases to between 33% and 50%. Fiveyear survival in patients with T2 tumors is improved following extended cholecystectomy with lymphadenectomy and liver resection (59%-61%) compared with simple cholecystectomy (17%-19%) 43 (Fig. 54-34). Several groups have recently reported 5-year overall survival rates for resected patients with stages IIA and IIB gallbladder cancer of 28% to 63% and 19% to 25%, respectively. However, most patients with gallbladder cancer have advanced unresectable disease at the time of presentation. As a result, fewer than 15% of all patients with gallbladder cancer are alive after 5 years. The median survival for stage IV patients at the time of presentation is only 1 to 3 months. Bile Duct Cancer Cholangiocarcinoma is an uncommon tumor that may occur anywhere along the intrahepatic or extrahepatic biliary tree. These tumors are located most commonly at the hepatic duct bifurcation (60%-80% of cases). Less commonly, tumors originate in the distal common bile duct or in the intrahepatic bile ducts. Most cholangiocarcinomas present with jaundice, and the diagnosis of cholangiocarcinoma should be considered in every patient with obstructive jaundice. When possible, surgical resection does offer a chance for long-term disease-free survival. Many patients, however, will be candidates only for palliative bypass or operative or nonoperative intubation aimed to provide biliary drainage and prevent cholangitis and hepatic failure. Incidence The reported incidence of cholangiocarcinoma in the United States is 1 or 2 cases per 100,000 population. Incidence data from the American Cancer Society are diffi cult to interpret because intrahepatic bile duct cancers are included with primary liver cancers, whereas extrahepatic biliary cancers are in a separate category that includes gallbladder cancer. In the United States, an estimated 17,550 primary liver cancers will be diagnosed in 2005. Data from the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) program suggest that about 15% of these (2600 cases) will be intrahepatic cholangiocarcinomas. About 7000 cases of extrahepatic bile duct cancer are diagnosed annually in the United States, two thirds of which are gallbladder cancers. Thus, 2000 to 3000 cases per year are extrahepatic cholangiocarcinomas. For unclear reasons, the incidence of intrahepatic cholangiocarcinoma has been rising over the past 2 decades in Europe and North America, Asia, Japan, and Australia, whereas rates of extrahepatic cholangiocarcinoma are declining internationally. The rising rates of intrahepatic cholangiocarcinoma have not been associated with an increase in the proportion of early-stage or smaller lesions. Furthermore, incidence rates do not appear to have reached a plateau. Risk Factors A number of diseases have been linked to cholangiocarcinoma, including primary sclerosing cholangitis, choledochal cysts, and hepatolithiasis. Characteristics common to these diseases include bile duct stones, biliary stasis, and infection. Bile duct cancers in patients with primary sclerosing cholangitis are most often extrahepatic, commonly occur near the hepatic duct bifurcation, and are diffi cult to differentiate from the multiple, benign strictures associated with this disease. As a general rule, the incidence of biliary tract cancers increases with age; the typical patient with cholangiocarcinoma is between 50 and 70 years of age. However, patients with PSC and those with choledochal cysts present nearly 2 decades earlier. In contrast to gallbladder cancer, for which female gender predominates, the incidence of cholangiocarcinoma is slightly higher in men. This probably refl ects the higher incidence of PSC in men. Hepatolithiasis is also a defi nite risk factor for cholangiocarcinoma, which will develop in 5% to 10% of patients with intrahepatic stones. Hepatitis B and C are also now recognized as risk factors in the development of intrahepatic cholangiocarcinoma. At least two genetic disorders are associated with an increased risk for cholangiocarcinoma: the inherited “cancer family” syndrome termed Lynch syndrome II, and a rare inherited disorder called multiple biliary papillomatosis; the latter condition is characterized by multiple adenomatous polyps in the bile ducts, and repeated episodes of abdominal pain, jaundice, and acute cholangitis. Prior biliary-enteric anastomosis may also increase the future risk for cholangiocarcinoma. Five percent of patients in a large Italian series developed cholangiocar-
Figure 54-35 Bismuth classifi cation of perihilar cholangiocarcinoma by anatomic extent. Type I tumors (upper, left) are confi ned to the common hepatic duct, and type II tumors (upper, right) involve the bifurcation without involvement of secondary intrahepatic ducts. Type IIIa and IIIb tumors (lower, left) extend into either the right or left secondary intrahepatic ducts, respectively. Type IV tumors (lower, right) involve the secondary intrahepatic ducts on both sides. cinoma between 11 and 18 years after a biliary-enteric anastomosis. The risk for bile duct cancer was higher after transduodenal sphincteroplasty and choledochoduodenostomy than after hepaticojejunostomy and was most strongly associated with recurrent episodes of cholangitis. Multiple other risk factors for cholangiocarcinoma have been identifi ed, including liver fl ukes, Thorotrast, industrial chemicals, dietary nitrosamines, and exposure to dioxin. Staging and Classifi cation Cholangiocarcinoma is best classifi ed anatomically into three broad groups: 1. Intrahepatic 2. Perihilar 3. Distal Intrahepatic tumors are treated like hepatocellular carcinoma with hepatectomy, when possible. The perihilar tumors make up the largest group and are managed with resection of the bile duct, preferably with hepatic resection. Distal tumors are managed in a fashion similar to other periampullary malignancies with pancreatoduodenectomy. Cancers of the hepatic duct bifurcation have also been classifi ed according to their anatomic location (Fig. 54-35). In this system, type I tumors are confi ned to the common hepatic duct, and type II tumors involve the bifurcation without involvement of secondary intrahe- Chapter 54 Biliary System 1583 Table 54-3 Current American Joint Commission on Cancer TNM Staging System for Cholangiocarcinoma Stage 0 Tis N0 M0 Stage I T1 N0 M0 Stage II T2 N0 M0 Stage III T1 or T2 N1 or N2 M0 Stage IVA T3 Any N M0 Stage IVB Any T Any N M1 Tis, carcinoma in situ; T1, tumor invades the subepithelial connective tissue; T2, tumor invades perifi bromuscular connective tissue; T3, tumor invades adjacent organs. N0, no regional lymph node metastases; N1, metastasis to hepatoduodenal ligament lymph nodes; N2, metastasis to peripancreatic, periduodenal, periportal, celiac, and/or superior mesenteric artery lymph nodes. M0, no distant metastasis; M1, distant metastasis. Adapted from Greene F, Page D, Fleming I, et al (eds): AJCC Cancer Staging Manual, 6th ed. New York, Springer-Verlag, 2002. patic ducts. Types IIIa and IIIb tumors extend into either the right or left secondary intrahepatic ducts, respectively, and type IV tumors involve the secondary intrahepatic ducts on both sides. Cholangiocarcinoma is also staged according to the tumor, node, metastasis (TNM) classifi cation of the AJCC (Table 54-3). Using this system, stage IA tumors are limited to the bile duct, whereas stage IB tumors invade periductal tissues. Stage IIA tumors are locally advanced without lymph node metastases, and stage IIB tumors have regional lymph node metastases. Stage III tumors are locally advanced and unresectable, and stage IV tumors have distant metastases. Portal vein involvement and lobar atrophy have been reported as important prognostic factors for cholangiocarcinoma and may be incorporated in the classifi cation in the future. 45 Clinical Presentation More than 90% of patients with perihilar or distal tumors present with jaundice. Patients with intrahepatic cholangiocarcinoma are rarely jaundiced until late in the course of the disease. Less common presenting clinical features include pruritus, fever, mild abdominal pain, fatigue, anorexia, and weight loss. Cholangitis is not a frequent presenting fi nding but most commonly develops after biliary manipulation. Except for jaundice, the physical examination is usually normal in patients with cholangiocarcinoma. Diagnosis and Assessment of Resectability At the time of presentation, most patients with perihilar and distal cholangiocarcinoma have a total serum bilirubin level greater than 10 mg/dL. Marked elevations in alkaline phosphatase are also routinely observed. Serum CA 19-9 may also be elevated in patients with cholangiocarcinoma, although levels may fall once biliary obstruction is relieved. The radiologic evaluation of patients with cholangiocarcinoma should delineate the overall extent of the
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Ch. 54 – Biliary System

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