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Chapter 62: Biliary Neoplasms 1003

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this, during a second operation for incidentally discovered gall-bladder cancer, an extended right hepatectomy along with exci-sion of the extrahepatic biliary tree and portal lymphadenec-tomy is often necessary. This resection allows adequateexposure for lymphadenectomy and greater confidence of anegative margin on the bile duct, and also permits biliaryreconstruction to only one side of the liver. The disadvantage isthat a large portion of normal liver parenchyma is sacrificed,and consequently, transient postoperative liver dysfunction iscommon. Although it may be more difficult to curatively resectdisease in patients with incidentally discovered gallbladder can-cer after laparoscopic cholecystectomy, there is no difference inoverall survival between patients with incidentally discoveredgallbladder cancer who are submitted to curative resection andthose patients who undergo initial curative resection.35

When a patient presents with T1 gallbladder cancer discov-ered after simple cholecystectomy, the pathology should bereviewed to determine if the entire gallbladder has beenremoved and if the cystic duct margin is clear of tumor. If thecystic duct margin is positive, the patient requires bile ductexcision. If all margins are negative, no further therapy is war-ranted. If the tumor is proved to be T2 or greater, the patientshould undergo reexcision to attempt complete resection if theextent of disease evaluation is negative. Patients with a knownor suspected early gallbladder carcinoma should not undergolaparoscopic cholecystectomy. Rather, open exploration andcholecystectomy should be performed.

Adjuvant Therapy

Adjuvant therapy for gallbladder cancer remains a controver-sial and unproved consideration. Very few randomized trialshave been conducted, and those that have are limited because ofthe number of patients included. Given the relative rarity ofthese malignancies in the United States, large-scale, randomizedtrials are feasible only in the context of a multi-institutional orcooperative group setting.

One recent prospective, randomized phase III trial of adju-vant chemotherapy with 5-fluorouracil and mitomycin C ver-sus surgery alone for patients with pancreaticobiliary malig-nancies having resection found that in the subset of patientswith gallbladder cancer (n � 112), the 5-year survival rate wassignificantly better in the adjuvant group (26%) versus thecontrol group (14%).46 Similarly, the 5-year disease-free sur-vival rate was 20.3% versus 11.6%, clearly favoring the adju-vantly treated group. This trial suggests that adjuvantchemotherapy may offer benefit for patients with resectedgallbladder cancer; however, replication in a larger-scale set-ting is required before definitive conclusions can be drawn.

Prognosis

The 5-year survival rate for all patients with gallbladder can-cer is less than 5% in most series, with a median survival of 6months. This is primarily because most patients present withunresectable disease. Of those patients undergoing resection,survival is dependent on depth of penetration and nodal sta-tus. Nearly 100% survival is reported after simple cholecys-tectomy for T1 disease, whereas patients with T2 and T3tumors without nodal disease have a 5-year survival greaterthan 50%.11,12,34,36 Node positivity is an ominous finding, withfew series reporting 5-year survivors.

Follow-up after Resection for Gallbladder Cancer

The most common sites of recurrence after resection of gall-bladder cancer include carcinomatosis, intrahepatic metastases,

or nodal recurrence in the retroperitoneum. Jaundice is a com-mon sign, but patients with recurrence may also present withascites caused by carcinomatosis. For most tumors, localrecurrence is found synchronously with diffuse intra-abdominalspread. Therefore, surgical treatment of recurrence has littlepotential for cure. If recurrent disease is found after resection,prognosis is exceedingly poor, with death occurring sec-ondary to biliary sepsis or liver failure within months ofdiagnosis.

The main goal of follow-up after resection of gallbladdercancer is to provide palliation for symptomatic recurrences.The main symptoms associated with recurrence requiring pal-liation are pruritus or cholangitis associated with jaundice, orbowel obstruction associated with carcinomatosis. Additionalgoals of follow-up are to detect benign complications of sur-gical treatment such as biliary stricture. When jaundice orcholangitis is the presenting symptom of possible recurrence,a nonsurgical palliative approach using percutaneous trans-hepatic cholangiogram (PTC) and stenting is usually favoredunless a benign postsurgical stricture is suspected. Because ofthe rapid growth of tumor in patients with recurrence, thehospitalization and recovery time from a surgical bypass isusually not justified for recurrences resulting in biliaryobstruction.

The routine follow-up of a patient after resection of gall-bladder cancer includes office visits every 3 months with phys-ical examination and measurement of liver function tests.Although CA19-9 may be elevated in patients with gallbladdercancer, the sensitivity and specificity are poor47 and, thus,should not be used for screening patients for recurrence.Because an asymptomatic recurrence of gallbladder cancer hasonly limited treatment options, overaggressive use of imagingstudies is not warranted. Therefore, the use of imaging studiesshould be individualized.

Issues for the Future

Clearly, improving our ability to recognize early gallbladdercancer in high-risk geographic areas would have an importantimpact on outcome in these patients. This will likely requireimprovements in understanding the sequential molecularchanges associated with gallbladder cancer. Other improve-ments in screening programs in high-risk areas, which couldresult in prophylactic cholecystectomy, would likely bebeneficial.48

BILE DUCT CARCINOMAOne of the most technically difficult surgical resections occursin patients with bile duct tumors arising in the hepatic hilus,named Klatskin tumors, or hilar cholangiocarcinoma. Bileduct cancers can arise at other sites, including within the liver(intrahepatic cholangiocarcinoma) and below the biliary bifur-cation but above the pancreas (mid–bile duct cholangiocarci-noma). Distal cholangiocarcinoma involves that portion of thebile duct within the pancreas, which requires pancreaticoduo-denectomy. The location of the tumor affects prognosis as wellas the potential for curative resection.

Resection of biliary neoplasms, particularly hilar cholangio-carcinoma, often requires radical resections and complex bil-iary reconstructions that have only recently become safe in rou-tine practice. Surgery may also offer effective palliation forthese cancers by providing biliary bypass for jaundiced patientswith unresectable tumors. Because disease is often diagnosedlate in the course and because complex operative techniquesare required for potentially curative resection, these tumorsrepresent one of the greatest challenges for definitive treatment.Adding to this is that there are no proved effective options foradjuvant treatment.

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1004 Part Two: Surgical Practice

Incidence

The overall incidence of hilar cholangiocarcinoma in theUnited States is 1.0/100,000 per year, although other geo-graphic regions such as Israel and Japan have higher rates.49

The incidence of intrahepatic cholangiocarcinoma in theUnited States is approximately 0.7/100,000 with a similarmortality. During the last 30 years, it appears that both theincidence and mortality in the United States are increasing.50

Most recently, population studies have noted that there hasbeen a trend toward a relative increased incidence of intrahep-atic cholangiocarcinoma (ICC) compared to extrahepaticcholangiocarcinoma (ECC).1,51,52 Using the Surveillance, Epi-demiology and End Results-Medicare databases, Welzel et al.51

noted HCV infection, chronic nonalcoholic liver disease andobesity, and smoking being associated only with ICC and notECC, possibly explaining the divergent trends in incidence.Cholangiocarcinomas arise slightly more often in males,9

with a male-to-female ratio of 1.3:1 and an average age of 50to 70 years.

Known risk factors for this disease include primary scleros-ing cholangitis, ulcerative colitis, choledochal cysts, and bil-iary tract infection, either with Clonorchis or in chronictyphoid carriers.53 Treating patients with cholangiocarcinomaarising from one of these underlying conditions is challeng-ing.54 Some industrial chemicals (e.g., nitrosamines, dioxin,asbestos, and polychlorinated biphenyls) have also been impli-cated in the pathogenesis of cholangiocarcinoma.49 Althoughthere has been some suggestion of an increased risk of cholan-giocarcinoma arising after transduodenal sphincteroplasty,55 itis difficult to determine if this is caused by the surgical inter-vention or the underlying disease leading to sphincteroplasty.

Pathology and Staging

Similar to gallbladder cancer, bile duct tumors tend to invadelocally. More than 95% of these tumors are adenocarcinomas.They are morphologically described as nodular, which is themost common, scirrhous, diffusely infiltrating, or papillary.Histologic subtypes include acinar, ductular, trabecular, alveo-lar, and papillary. Papillary tumors appear to have an improvedoutcome. Much less common types of bile duct tumors includecystadenocarcinomas, hemangioendotheliomas, and mucoepi-dermoid carcinomas. Perineural invasion is clearly a poorprognostic sign.56

In patients with intrahepatic cholangiocarcinoma, negativeprognostic signs include vascular invasion, multiple tumors,positive margin, large size, and lymph node metastases.57 Thesetumors can be either sclerotic, masslike lesions, or cystic lesions.

Historically, cholangiocarcinomas have been classifiedaccording to their location in the upper (60%), middle(15%–20%), or lower third (15%–20%) of the bile duct.Middle-third lesions arise between the cystic duct and thesuperior border of the duodenum. Lower-third lesions arefound below the superior border of the duodenum but abovethe ampulla. The problem with this classification is that theanatomic landmarks are somewhat arbitrary and not clinicallyuseful. Most mid–bile duct malignant obstructions are causedby gallbladder cancer. Even when it is truly a mid–bile ductcholangiocarcinoma, very few of these tumors are amenable totreatment by local excision of the bile duct. A more useful clas-sification is to divide these lesions into upper-half or lower-halftumors, based on the location of the cystic duct as it enters thecommon duct (in the case of normal anatomy). The usefulnessof this classification scheme is that it allows the surgeon todelineate whether a hepatic or pancreatic resection will berequired for clearance of tumor. The AJCC TNM staging sys-tem (seventh edition) for bile duct cancers is described inTables 62.4A and B.

Other staging systems have been created that attempt toincorporate clinically important indicators of resectability forhilar cholangiocarcinoma that are defined preoperatively,including hepatic lobe atrophy or portal vein involvement.58

With the increasing acceptance of major hepatic resection forthese tumors, this preoperative staging system attempts todefine whether there is ipsilateral involvement alone, becausetumors with bilateral extension past the primary biliary radi-cles are not resectable.

Clinical Findings and Diagnosis

The vast majority of patients with cholangiocarcinoma presentwith painless jaundice, though mild right upper quadrantpain, pruritus, anorexia, malaise, and weight loss may also bereported. Cholangitis is the presenting symptom in 10% to30% of patients. Some patients have cancer discovered onevaluation for otherwise asymptomatic elevations of alkalinephosphatase and gamma-glutamyl transferase.

Patients with intrahepatic cholangiocarcinoma are usuallyasymptomatic. Many patients are found to have a liver tumorpresent on cross-sectional imaging obtained for other reasons.Many of these patients will present to the surgeon with abiopsy showing adenocarcinoma without a known primarytumor. The standard evaluation in these patients shouldinclude tumor markers to rule out an elevated carcinoembry-onic antigen (CEA) or �-fetoprotein (AFP); upper and lowerendoscopy to evaluate for a gastrointestinal source; CT scan toassess for a primary tumor in the gastrointestinal tract or pan-creas; and, in women, a mammogram. If no site of primarydisease is found, in most patients, the diagnosis is intrahepaticcholangiocarcinoma.

Various imaging tests are available to assess patients withhilar cholangiocarcinoma. Abdominal ultrasound is noninva-sive, easily available, and inexpensive, and thus is commonlyused as a first imaging modality. It can establish the level of bil-iary obstruction and rule out cholelithiasis or choledocholithi-asis as the etiology. CT scans frequently reveal dilated intra-hepatic biliary ducts with a normal, collapsed gallbladder,and, depending on the level of the tumor, a nondilated or par-tially dilated extrahepatic biliary tree (Fig. 62.4). In addition,the presence of hilar adenopathy can be assessed. Portal veinpatency can be determined with ultrasound or helical CT. Inaddition, signs of hepatic lobar atrophy should be sought,because this is associated with a high incidence of ipsilateralportal vein involvement by tumor. MRCP offers the potentialof evaluating parenchymal, vascular, biliary, and nodalinvolvement with a single noninvasive examination.20–22 Fre-quently, it is possible to visualize the tumor itself with MRI(Figs. 62.5 and 62.6).

In most centers, direct cholangiography is used to evaluatethe extent of biliary involvement and provide palliation forjaundice. Endoscopic retrograde cholangiopancreatography(ERCP) has little role to play in high biliary obstructionbecause opacification of the proximal biliary tree is difficult.ERCP, however, can be effectively used to image more distallesions. At the time cholangiography is performed, someauthors advocate the routine preoperative placement of biliarydrainage catheters to aid in intraoperative identification of thebile ducts.59,60 Others have found a higher incidence of infec-tious complications61 and mortality,62 and a longer hospitalstay,63 after preoperative placement of biliary drainagecatheters. The difficulty in making the decision regardingpreoperative stenting is that many patients are severely symp-tomatic because of jaundice and pruritus and require pallia-tion, thus if the operation is delayed, many patients requirepalliation.

In many cases, it is difficult to obtain pathologic confirma-tion of cholangiocarcinoma except in very advanced cases,even with the use of biliary brushings and cytology obtained at

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FIGURE 62.4. Computed tomography scan in a patient with hilar cholangiocarcinoma, demonstrating dilated intrahepatic ducts in the right lobebut inability to directly visualize tumor.

AMERICAN JOINT COMMITTEE ON CANCER, 6TH EDITION, STAGING SYSTEM FORPERIHILAR BILE DUCT CARCINOMA

TABLE 62 .4A STAGING

■ STAGE ■ TUMOR ■ NODES ■ METASTASIS

0 Tis N0 M0

I T1 N0 M0

II T2a-–b N0 M0

IIIA T3 N0 M0

IIIB T1–3 N1 M0

IVA T4 N0–1 M0

IVB Any T N1–2 M0

Any T Any N M1

Definition of TNM

Primary tumor (T)

TX Primary tumor cannot be assessed

T0 No evidence of primary tumor

Tis Carcinoma in situ

T1 Tumor confined to bile duct, with extension up to the muscle layer or fibrous tissue

T2a Tumor invades beyond the wall of the bile duct to surroundng adipose tissue

T2b Tumor invades adjacent hepatic parenchyma

T3 Tumor invades unilateral branches of the portal vein or hepatic artery

T4 Tumor invades main portal vein or its branches bilaterally; or the common hepaticarter; or the second-order biliary radicals bilaterally; or unilateral second-orderbiliary radicals with contralateral portal vein or hepatic artery involvement.

Regional lymph nodes (N)

NX Regional lymph nodes cannot be assessed

N0 No regional lymph node metastasis

N1 Regional lymph node metastasis (including nodes along the cystic duct, commonbile duct, hepatic artery and portal vein)

N2 Metastasis to periaortic pericaval, superior mesentary artery, and/or celiac arterylymph nodes

Distant metastasis (M)

M0 No distant metastasis (no pathologic M0; use clinical M to complete stage group)

M1 Distant metastasis



A B

FIGURE 62.5. Coronal (A) and axial (B) magnetic resonance imaging in a patient with hilar cholangiocarcinoma. Dilated intrahepatic ducts arepresent with a soft tissue density consistent with tumor (arrows).

AMERICAN JOINT COMMITTEE ON CANCER, 6TH EDITION, STAGING SYSTEM FOR DISTAL BILE DUCT CARCINOMA

TABLE 62 .4B STAGING

■ STAGE ■ TUMOR ■ NODES ■ METASTASIS

0 Tis N0 M0

I T1 N0 M0

II T2a–b N0 M0

IIIA T3 N0 M0

IIIB T1–3 N1 M0

IVA T4 N0–1 M0

IVB Any T N1–2 M0

Any T Any N M1

DEFINITION OF TNM

Primary tumor (T)

TX Primary tumor cannot be assessed

T0 No evidence of primary tumor

Tis Carcinoma in situ

T1 Tumor confined to bile duct, with extension up to the muscle layer or fibrous tissue

T2 Tumor invades beyond the wall of the bile duct

T3 Tumor invades gallbladder, pancreas, duodenum, or other adjacent organs withoutinvolvement of the celiac axis or the superior mesenteric artery

T4 Tumor involves the celiac axis or the superior mesteteric artery

Regional lymph nodes (N)

NX Regional lymph nodes cannot be assessed

N0 No regional lymph node metastasis

N1 Regional lymph node metastasis (including nodes along the cystic duct, commonbile duct, hepatic artery and portal vein)

N2 Metastasis to periaortic pericaval, superior mesentary artery, and/or celiac arterylymph nodes

Distant metastasis (M)

M0 No distant metastasis (no pathologic M0; use clinical M to complete stage group)

M1 Distant metastasis



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FIGURE 62.6. Coronal (A) magnetic resonance imaging and magnetic resonance cholangiopancreatography (B) in a patient with hilar cholan-giocarcinoma, demonstrating dilated intrahepatic ducts narrowing at the area of obstruction.

the time of direct cholangiography. In most cases, patients areoffered surgical therapy based on clinical suspicion and radi-ographic appearance. In patients with intrahepatic cholangio-carcinoma, cross-sectional imaging with CT scan is usuallysufficient. Tumors may be masslike or may have cystic areas.

Surgery

Proximal Cholangiocarcinomas. Untreated, most patientswith bile duct cancers die within a year of diagnosis.63,64 Sur-gical excision is the treatment of choice, with no other poten-tially curative therapy. The immediate causes of death aremost commonly hepatic failure or cholangitis related to tumorgrowth and inadequate drainage of the biliary tree.65 There-fore, the objectives of management for patients with cholan-giocarcinoma include both complete removal of tumor andadequate biliary drainage. It has become clear over the lastthree decades that curative treatment for patients with tumorsinvolving the upper half of the bile duct depends on aggressiveexcision that often requires a major liver resection. Until asrecently as one decade ago, treatment of hilar cholangiocarci-nomas was associated with mortality as high as 30%.66–70

Recently, major improvements in the safety of these operationshas been demonstrated, and resection of hilar tumors nowresults in mortality of less than 10%, even when liver resec-tions are required.66,67,69,71

Assessment of Resectability and Surgical Procedure.Surgical exploration is often the only means of assessingresectability. Because of the potential morbidity of a laparo-tomy that has no therapeutic benefit, staging laparoscopy hasbeen advocated to save patients from unnecessary laparotomy.In patients with hilar cholangiocarcinoma, up to 25% willbenefit from staging laparoscopy because of detection ofoccult extrahepatic disease.24,28 Laparoscopy is a very sensitivemeans to detect peritoneal metastases or additional intrahep-atic disease through the use of laparoscopic ultrasound but isless sensitive in detecting nodal metastases or locally invasivetumors.24

Hilar cholangiocarcinoma is considered unresectablebecause of both local factors and metastatic spread. Clearly,patients with disease outside the liver, including most commonlyperitoneal and intrahepatic metastases, are not amenable tocurative resection. Local factors that make these tumors unre-sectable include invasion of the main portal vein or both the

right and left portal vein and hepatic arteries and tumorextension into second-order biliary radicals of both rightand left hepatic lobes. By contrast, tumors extending intosecond- or third-order biliary radicles on one side of the liverwithout vascular involvement can be resected with curativeoutcome.

The goals of surgical management for cholangiocarcinomasare both eradication of tumor and establishment of adequatebiliary drainage. Tumors of the biliary confluence are particu-larly difficult to treat because symptoms often appear late inthe course of disease when the lesion has already involvedadjacent structures including the portal vein or adjacenthepatic parenchyma. Complete resection, therefore, requiresbiliary and hepatic resection and often major vascular recon-struction. It is not surprising, therefore, that in the past thesurgical therapy for proximal biliary malignancies consistedmainly of biliary-enteric bypass as palliation for jaundice andcholangitis. The therapeutic approach to hilar cholangiocarci-noma was largely nihilistic, because of difficulty in delineatingthe extent of disease and the technical challenge of completeresection for such lesions.

Over the last decade, surgical approaches have becomemore aggressive, as demonstrated by the increasing number ofhepatic resections that have been performed for bile duct can-cers.66–70 Recent improvements in ultrasound, CT, MRI, andangiography have greatly facilitated preoperative diagnosesand staging of cholangiocarcinoma. This has allowed improvedpatient selection and surgical planning. The location and localextension of tumor dictates the extent of resection, with mostlesions requiring an extended right or left hepatectomy for com-plete excision. Caudate resection is often required because ofdirect extension into caudate biliary radicles or parenchyma.58,69,70

CBD excision and portal lymphadenectomy are also essentialfor tumor clearance.

Liver Transplantation for Hilar CCA and the MayoProtocol. Orthotopic liver transplantation (OLT) wasthought to hold promise for patients with hilar CC, because ofits ability to achieve adequate margins by complete hepatec-tomy. Unfortunately, when used as a single treatment modal-ity, results have been poor. Three- and 5-year survival rateshave been reported at 25% to 30%.73–75 Because of this pooroutcome, the Mayo Clinic developed a protocol combiningneoadjuvant therapy with liver transplant, based on a strategyinitially developed by the University of Nebraska. This protocoluses high-dose neoadjuvant 5-fluorouracil and brachytherapy


followed by liver transplantation.72,76 Inclusion criteriainclude: (i) locally advanced unresectable disease with eitherpositive intraluminal brush cytology, positive intraluminalbiopsy, or CA19-9 �100 in the setting of a radiographicmalignant stricture; (ii) primary sclerosing cholangitis withresectable disease, and; (iii) absence of medical contraindica-tions for OLT. Since 2003, biliary aneuploidy as demon-strated by digital image analysis (DIA)77 and fluorescent insitu hybridization have been considered equivalent to cytol-ogy.78 Exclusion criteria include: (i) extrahepatic diseaseincluding regional lymph node involvement; (ii) uncontrolledinfection; (iii) prior attempt at resection; (iv) prior treatmentwith radiation or chemotherapy; and (v) previous malignancywithin 5 years. In this protocol, patients receive externalbeam radiotherapy to a target dose of 4,500 cGy with con-comitant fluorouracil (5-FU). Following this, transcatheteriridium-192 brachytherapy with a target dose of 2,000 to3,000 cGy is administered. Thereafter, patients receive oralcapecitabine as tolerated until transplantation. It is importantthat, prior to transplantation, patients undergo a staginglaparotomy, at which time biopsy of perihilar lymph nodes aswell as any lymph nodes or nodules suspicious for tumor isperformed. Only patients with negative staging operationsremain eligible for transplantation.72

Thus, patients eligible for OLT under this protocol havelocally advanced tumors but no pathologic nodal disease. Fur-thermore, the prolonged course of neoadjuvant therapy, stag-ing laparotomy, and time on the OLT waiting list provides anopportunity to exclude patients demonstrating disease pro-gression. This highly rigorous selection bias in favor ofpatients with biologically favorable disease is reflected in theearly outcomes published from the Mayo group. In a recentreview of 71 patients enrolled in this transplant protocol, only38 underwent transplantation (38%). These patients werecompared to 26 patients (of 54 explored, 48%) who under-went successful resection. When compared to those undergo-ing resection (some with node-positive disease), patientsundergoing transplantation were younger (p �0.001) andmore likely to have inflammatory bowel disease (p �0.03) andPSC (p �0.001). It is important that only 58% of patients hadhistologically proven cancer.

In these highly disparate groups, 5-year survival was 82%after transplantation (38 patients) compared to 21% after

resection of 26 patients (p � 0.022).72 There were also fewerrecurrences in the transplant patients (13% vs. 27%), andrecurrences became apparent later after transplantation thanafter resection (mean 40 months vs. 21 months). Direct com-parisons are difficult with this study given the differencesbetween groups. At present, OLT cannot be considered a stan-dard form of therapy for hilar cholangiocarcinoma forpatients with resectable disease, but it does offer a potentialoption for patients with underlying PSC or those with unre-sectable tumors who fit the rigorous inclusion and exclusioncriteria of the protocol.

Prognosis after Resection. Results of major studies onresection of hilar cholangiocarcinoma are summarized in Table62.5. In patients amenable to curative resection, the mediansurvival is 35 months with a 5-year survival rate of 10% to30%.61,68,69,72–80 Surgical resection provides both improved sur-vival and improved quality of life.79,80 The greatest risk factorsfor recurrence include the presence of positive margins79,81 andnode-positive tumors.82

Prognosis after Resection for Intrahepatic or DistalCholangiocarcinoma. In patients with intrahepatic cholan-giocarcinoma, expected 3-year survival rates as high as 60%have been reported,24,83 with 5-year survival rates of 30% to45%.84 Patients with unresectable disease have a median sur-vival of 12 months.85,86 Thus, completely resected intrahepaticcholangiocarcinoma appears to have an improved prognosisover proximal (hilar) cholangiocarcinoma.

Most patients with distal cholangiocarcinomas are defini-tively managed with pancreaticoduodenectomy (Whipple pro-cedure) to obtain adequate clearance of tumor, because of theintrapancreatic location of the distal CBD. Patients with cholan-giocarcinomas arising in the distal bile duct have both anincreased resectability rate and improved prognosis over thosewith hilar cholangiocarcinomas.71 Patients with resectable distalbile duct cancer have a 5-year survival rate of 30% to 50%,84,87

with a decreased survival if nodes are involved with tumor.87

Surgical Treatment of Unresectable Cholangiocarci-noma. For patients with unresectable hilar cholangiocarcino-mas, significant improvement in quality of life can occur withsurgical bypass. Palliative bypass can be performed in several


RESULTS AFTER RESECTION FOR HILAR CHOLANGIOCARCINOMA

TABLE 62 .5 RESULTS

■ SURVIVAL (MONTHS)■ PERCENT ■ POSTOPERATIVE ■ 5-YEAR

■ AUTHOR ■ N RESECTED MORTALITY (%) SURVIVAL (%) ■ MEAN ■ MEDIAN

Cameron et al., 199059 96 55 2 8 18

Hadjis et al., 199070 131 21 7 12 25

Altaee et al., 199166 70 21 19 12

Baer et al., 199367 48 44 4 23 34

McMasters et al., 1997123 91 44 0 26 22

Klempnauer et al., 1997124 151 10 28 24

Nagino et al., 1999125 173 80 10 26

Nakeeb et al., 2002126 72 57 34

Jarnagin et al., 2001127 225 71 10 27 35

Rea et al., 200472 46 9 26 28

Jang et al, 2005128 48 0 48

Konstadoulakis et al., 2008129 73 81 7 35

Yubin et al., 2008130 115 100 2 22 40



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FIGURE 62.7. Surgical approach to segment III duct. A: The bridge of tissue present at the base of the liver is divided. B: Theligamentum teres is held superiorly to expose the tissue overlying the segment III duct. C: The segment III duct is exposed.D: The duct is opened in preparation for anastomosis with a Roux-en-Y jejunal limb. (Courtesy of Dr. L. H. Blumgart.)

ways. A partial excision of the left lateral segment and biliary-enteric anastomosis to the left hepatic duct (Longmire proce-dure) was used commonly in the past, but more recently surgi-cal techniques have become less complicated and do not requirehepatic parenchymal transection. One technique involves biliarydecompression through the left duct, approached through theround ligament, a segment III bypass (Fig. 62.7). Opening thebridge of tissue just beneath the ligamentum teres allows accessto the duct. In this position, a long anastomosis can be per-formed from the segment III duct to a jejunal limb because ofthe horizontal course of the duct in this location. Although lesscommonly used, the right hepatic duct can be approached atthe base of the gallbladder fossa. This is technically more diffi-cult and results in a higher rate of late bypass failure.88

Nonoperative palliative biliary decompression can beaccomplished with percutaneous or endoscopic stenting,depending on the level of obstruction. Proximal lesions are usu-ally approached percutaneously with placement of expandablestents or drainage catheters (Fig. 62.8). Internal stents result infewer electrolyte abnormalities and improvement in patientcomfort, although morbidity and mortality occurs in up to 30%of patients and stent occlusion is common.89–91 There is a signif-icant risk of cholangitis with external and internal drainage,occurring in more than 90% of patients with metallic expand-able internal stents in one series.90 Bleeding and bile leaks arealso frequent complications. More recent techniques (e.g., pho-

todynamic therapy) have been used to palliate patients with bil-iary obstruction and may hold some promise for the future.92

Because patients with unresectable disease have a shortmedian survival, those whose disease is clearly unresectable onpreoperative imaging should undergo percutaneous internal orexternal drainage. In patients who undergo exploratorysurgery and whose disease is found to be unresectable, surgicalbypass offers fewer episodes of cholangitis, with an improvedquality of life. In some series, surgical bypass for patients withunresectable disease is the only biliary drainage procedure everrequired by the patient.

In patients with unresectable distal cholangiocarcinomas,palliation can be achieved with surgical bypass, percutaneousbiliary drains, or ERCP-placed stents. The simplest and mosteffective way to relieve jaundice is usually with an ERCP stent.Although surgical bypass offers improved patency and fewerepisodes of cholangitis, the morbidity of the procedure is notwarranted in patients with these aggressive tumors.

INTRAHEPATICCHOLANGIOCARCINOMA

Patients with intrahepatic cholangiocarcinoma typically pre-sent with single liver lesions. The procedure of choice isanatomically based hepatic resection. Because these tumors


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