INTRODUCTION

This chapter addresses the resection of primary malignancy of the intrahepatic and extrahepatic biliary tree. Gallbladder cancer is an important extrahepatic biliary cancer that is often discovered incidentally. Cholangiocarcinoma occurs at the biliary confluence, in the mid-duct, or in the distal duct presenting as a periampullary tumor (see also Chapter 10). Three distinct macroscopic subtypes of cholangiocarcinoma are well described: sclerosing, nodular, and papillary (Weinbren and Mutum, 1983).

Sclerosing tumors cause an annular thickening of the bile duct, often with diffuse infiltration and fibrosis of the periductal tissues (Figs. 9-1 and 9-2). Nodular tumors are characterized by a firm, irregular nodule of tumor that projects into the lumen of the duct. Longitudinal spread along the duct wall and periductal tissues is an important pathologic feature.

Figure 9-1 A, Endoscopic cholangiogram shows the typical irregular biliary stricture characteristic of sclerosing adenocarcinomas (arrow). These tumors cause a circumferential thickening of the duct wall and periductal fibrosis, which ultimately obliterates the lumen. B, Coronal MRCP reconstruction shows an infiltrating tumor involving the hepatic duct confluence (arrow). The right and left intrahepatic ducts, which appear white, are dilated.

Figure 9-2 Gross cholangiographic and microscopic appearance of a papillary cholangiocarcinoma (left, A, C, E) and a nodular-sclerosing tumor (right, B, D, F). Note the papillary tumor within the bile duct lumen (arrow in A) and the nodular-sclerosing tumor invading the hepatic parenchyma (arrow in B). Transhepatic cholangiogram of a papillary tumor (C) shows multiple filling defects that expand the duct (black arrows; the biliary drainage catheter is indicated by the white arrow). This is in contrast to the cholangiographic features of nodular-sclerosing tumors characterized by an irregular stricture that constricts the duct lumen (arrow in D); a transhepatic catheter is seen traversing the stricture. Histologic section of a papillary cholangiocarcinoma with no invasive component (asterisk in E) and an invasive nodular-sclerosing tumor associated with a desmoplastic stroma (F).

(From Jarnagin WR, et al. Papillary phenotype confers improved survival after resection of hilar cholangiocarcinoma. Ann Surg 2005;241:703-712.)

The papillary variant is soft and friable and may be associated with minimal transmural invasion. A polypoid mass that expands rather than contracts the duct (see Fig. 9-2) is a characteristic feature. The bulk of the tumor may be mobile within the bile duct.

Biliary cancer may arise within the intrahepatic biliary tree presenting as a mass or as a biliary cyst. Generally speaking, periampullary cancer is dealt with by pancreaticoduodenectomy (see Chapter 10) and intrahepatic cholangiocarcinoma by hepatic resection (see Chapters 2 to 5). Cholangiocarcinoma involving the proximal bile ducts (hilar cholangiocarcinoma) and gallbladder cancer require biliary resection, with or without a concomitant hepatic resection.

INFECTION

Bacterial contamination of the bile (bactibilia) is common in patients with hilar cholangiocarcinoma (McPherson et al., 1984). Instrumentation and previous operation significantly increase the incidence of bactibilia and the risk of postoperative infection and are associated with greater morbidity and mortality rates after surgical resection.

Portal venous inflow and bile flow are important in the maintenance of liver cell size and mass (Hadjis and Blumgart, 1987). Segmental or lobar atrophy may result from portal venous occlusion or biliary obstruction. One or both of these findings are often present in patients with hilar cholangiocarcinoma. Cross-sectional imaging shows a small, often hypoperfused lobe (Figs. 9-3 and 9-4).

Figure 9-3 Cross-sectional MRCP of a patient with hilar cholangiocarcinoma. The tumor involves the right and the left hepatic ducts. The left portal vein is occluded, resulting in severe atrophy of the left liver, which is shrunken and has dilated and crowded intrahepatic ducts. The bile ducts in this study appear white.

(From Jarnagin WR, Shoup M. Surgical management of hilar cholangiocarcinoma. Semin Liver Dis 2004;24:189-199.)

Figure 9-4 Intraoperative view showing a severely atrophic left lobe of liver, which is clearly demarcated from the right liver. The ligamentum teres is being pulled downward and to the left.

(From Jarnagin WR, Shoup M. Surgical management of hilar cholangiocarcinoma. Semin Liver Dis 2004;24:189-199.)

CHOLANGIOCARCINOMA INVOLVING THE PROXIMAL BILE DUCTS (HILAR CHOLANGIOCARCINOMA)

CLINICAL PRESENTATION AND IMAGING

The early symptoms are nonspecific. Abdominal discomfort, anorexia, weight loss, pruritus, and jaundice are the most common. Segmental obstruction may result in ipsilateral lobar atrophy without overt jaundice. Patients with papillary tumors may have a history of intermittent jaundice.

Physical Examination

Jaundice is obvious. Patients with pruritus often have skin excoriations. The liver may be enlarged and firm. The gallbladder is usually nonpalpable.

Imaging

Cholangiography shows the location of the tumor and the biliary extent of disease (see Fig. 9-1).

Computed tomography (CT) provides information regarding the level of obstruction, vascular involvement, and liver atrophy.

Duplex ultrasonography (Fig. 9-5) is a highly accurate predictor of vascular (particularly portal vein) involvement and of lobar atrophy, level of biliary obstruction, and hepatic parenchymal involvement (Hann et al., 1997).

Magnetic resonance cholangiopancreatography (MRCP) not only may identify the tumor and the level of biliary obstruction, but it also may reveal obstructed and isolated ducts, vascular involvement (see Figs. 9-1, 9-3, and 9-6), the presence of nodal or distant metastases, and the presence of lobar atrophy (see Fig. 9-1, 9-3, 9-6, and 9-7) (Schwartz et al., 1998).

Figure 9-5 A, Duplex ultrasound from a patient with hilar cholangiocarcinoma. The tumor is seen at the biliary confluence just above the portal vein (arrow). The mass is in contact with the portal vein, but there is no clear invasion or venous narrowing. A transtumoral stent also is visible. B, Transverse color Doppler ultrasound of the biliary confluence shows a papillary cholangiocarcinoma (m) extending into the right anterior (a) and posterior (p) sectoral ducts and the origin of the left duct (l). The adjacent portal vein (v) is not involved and has normal flow.

(From Hann LE, et al. Malignant hepatic hilar tumors: can ultrasonography be used as an alternative to angiography with CT arterial portography for determination of resectability? J Ultrasound Med 1996;15:37-45.)

Figure 9-6 A, T1-weighted, gadolinium-enhanced MRI of a patient with hilar cholangiocarcinoma. The bile ducts appear black. A hilar tumor is seen (T), apparently adherent to or encasing the right portal vein branch (PV) (arrowhead). The tumor has occluded the right anterior sectoral branch of the portal vein, and the anterior sector appears atrophic (indicated by black lines) with crowded, dilated ducts. The arrow points to intrahepatic metastases. B, MRCP cut through the same area of the liver. Similar findings are indicated in this image, in which the bile ducts appear white. The vascular findings seen on MRI were confirmed on duplex ultrasound. Biopsy specimens of the main tumor and the intrahepatic metastases confirmed adenocarcinoma.

Figure 9-7 Cross-sectional MRCP from a patient with hilar cholangiocarcinoma extending into the left hepatic duct and left lobe atrophy. The bile ducts appear white. The left lobe is small with dilated and crowded ducts (arrowhead). The principal caudate lobe duct, seen joining the left hepatic duct, also is dilated (arrow).

Alternative Diagnoses

The most common alternative diagnoses are gallbladder carcinoma, Mirizzi syndrome (Fig. 9-8), and idiopathic benign focal stenosis (malignant masquerade) (Hadjis et al., 1985).

Figure 9-8 Endoscopic retrograde cholangiography and cross-sectional CT of a patient with Mirizzi syndrome. A, Endoscopic retrograde cholangiography shows a characteristic large gallstone impacted in the neck of the gallbladder causing obstruction of the hepatic duct just below the biliary confluence (arrow). B, CT confirms the presence of a large gallstone (arrow). In most cases, the diagnosis of Mirizzi syndrome is not as clear-cut because the stone is not visualized.

Preoperative Evaluation

Assessment includes fitness for surgery (Burke, 1998). Characterization of tumor extent should take into account all available preoperative data related to local tumor, including the extent of tumor within the biliary tree, vascular involvement, lobar atrophy, and metastatic disease. This makes it possible to stage tumors preoperatively in a way that correlates with resectability and outcome. A proposed clinical staging scheme (Table 9-1) underscores the importance of considering portal vein involvement and liver atrophy in relation to the extent of ductal cancer spread. Ipsilateral involvement of vessels and bile ducts is usually amenable to resection, whereas contralateral involvement is not. (Jarnagin et al., 2001, 2005).

TABLE 9-1 Proposed Preoperative T Staging of Hilar Cholangiocarcinoma

STAGE	CRITERIA
T1	Tumor involving biliary confluence ± unilateral extension to 2 biliary radicles ^*
T2	Tumor involving biliary confluence ± unilateral extension to 2 biliary radicles and ipsilateral portal vein involvement ± ipsilateral hepatic lobar atrophy
T3	Tumor involving biliary confluence ++ bilateral extension to 2 biliary radicles or unilateral extension to 2 biliary radicles with contralateral portal vein involvement or unilateral extension to 2 biliary radicles with contralateral hepatic lobar atrophy or main or bilateral portal venous involvement