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Resection for biliary cancer


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 11 ). Three distinct macroscopic subtypes of cholangiocarcinoma are well described: sclerosing, nodular, and papillary ( ).

Sclerosing tumors cause an annular thickening of the bile duct, often with diffuse infiltration and fibrosis of the periductal tissues ( Figs. 10.1 and 10.2 ). 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.

Fig. 10.1., 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 magnetic resonance cholangiopancreatography reconstruction shows an infiltrating tumor involving the hepatic duct confluence (arrow ). The right and left intrahepatic ducts, which appear white, are dilated.

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. 10.2 ) is a characteristic feature. The bulk of the tumor may be mobile within the bile duct.

Fig. 10.2., 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 ).

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 11 ) and intrahepatic cholangiocarcinoma (IHC) by hepatic resection (see Chapters 2 to 6 ). 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 ( ). 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.

Atrophy

Portal venous inflow and bile flow are important in the maintenance of liver cell size and mass ( ). 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. 10.3 and 10.4 ).

Fig. 10.4., 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.

Fig. 10.3., Cross-sectional magnetic resonance cholangiopancreatography of a patient with hilar cholangiocarcinoma. The tumor involves the right and 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.

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. 10.1 ).

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

  • Duplex ultrasonography ( Fig. 10.5 ) is a highly accurate predictor of vascular (particularly portal vein) involvement and of lobar atrophy, level of biliary obstruction, and hepatic parenchymal involvement ( ).

    Fig. 10.5., 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.

  • 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. 10.1 , 10.3 , and 10.6 ), the presence of nodal or distant metastases, and the presence of lobar atrophy (see Fig. 10.1 , 10.3 , 10.6 , and 10.7 ) ( ).

    Fig. 10.6., 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, Magnetic resonance cholangiopancreatography 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.

    Fig. 10.7., Cross-sectional Magnetic resonance cholangiopancreatography 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. 10.8 ), and idiopathic benign focal stenosis (malignant masquerade) ( ).

Fig. 10.8., 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 ( ). Characterization of tumor extent should consider 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 10.1 ) 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 ( , ).

Table 10.1.
Proposed Preoperative T Staging of Hilar Cholangiocarcinoma
From Jarnagin WR, Fong Y, DeMatteo RP, Gonen M, Burke EC, Bodniewicz J, et al. Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma. Ann Surg 2001;234:507-519.
Stage Criteria
T1 Tumor involving biliary confluence ± unilateral extension to two biliary radicles *
T2 Tumor involving biliary confluence ± unilateral extension to two biliary radicles
And ipsilateral portal vein involvement ± ipsilateral hepatic lobar atrophy
T3 Tumor involving biliary confluence ++ bilateral extension to two biliary radicles
Or unilateral extension to two biliary radicles with contralateral portal vein involvement
Or unilateral extension to two biliary radicles with contralateral hepatic lobar atrophy
Or main or bilateral portal venous involvement

* Two biliary radicles = secondary biliary radicles.

Some clinicians use preoperative biliary drainage (PTBD) and portal venous embolization (PVE) extensively for hilar cholangiocarcinoma, but I use PVE techniques with reserve and seldom perform elective PTBD except in deeply jaundiced patients.

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