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 adequate
exposure for lymphadenectomy and greater condence of a
negative margin on the bile duct, and also permits biliary
reconstruction to only one side of the liver. The disadvantage is
that a large portion of normal liver parenchyma is sacriced,
and consequently, transient postoperative liver dysfunction is
common. Although it may be more difcult to curatively resect
disease in patients with incidentally discovered gallbladder can-
cer after laparoscopic cholecystectomy, there is no difference in
overall survival between patients with incidentally discovered
gallbladder cancer who are submitted to curative resection and
those patients who undergo initial curative resection.
35
When a patient presents with T1 gallbladder cancer discov-
ered after simple cholecystectomy, the pathology should be
reviewed to determine if the entire gallbladder has been
removed and if the cystic duct margin is clear of tumor. If the
cystic duct margin is positive, the patient requires bile duct
excision. If all margins are negative, no further therapy is war-
ranted. If the tumor is proved to be T2 or greater, the patient
should undergo reexcision to attempt complete resection if the
extent of disease evaluation is negative. Patients with a known
or suspected early gallbladder carcinoma should not undergo
laparoscopic cholecystectomy. Rather, open exploration and
cholecystectomy should be performed.
Adjuvant Therapy
Adjuvant therapy for gallbladder cancer remains a controver-
sial and unproved consideration. Very few randomized trials
have been conducted, and those that have are limited because of
the number of patients included. Given the relative rarity of
these malignancies in the United States, large-scale, randomized
trials are feasible only in the context of a multi-institutional or
cooperative group setting.
One recent prospective, randomized phase III trial of adju-
vant chemotherapy with 5-uorouracil and mitomycin C ver-
sus surgery alone for patients with pancreaticobiliary malig-
nancies having resection found that in the subset of patients
with gallbladder cancer (n 112), the 5-year survival rate was
signicantly better in the adjuvant group (26%) versus the
control 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 adjuvant
chemotherapy may offer benet for patients with resected
gallbladder cancer; however, replication in a larger-scale set-
ting is required before denitive 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 6
months. This is primarily because most patients present with
unresectable 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 T3
tumors without nodal disease have a 5-year survival greater
than 50%.
11,12,34,36
Node positivity is an ominous nding, with
few 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 with
ascites caused by carcinomatosis. For most tumors, local
recurrence is found synchronously with diffuse intra-abdominal
spread. Therefore, surgical treatment of recurrence has little
potential 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 of
diagnosis.
The main goal of follow-up after resection of gallbladder
cancer is to provide palliation for symptomatic recurrences.
The main symptoms associated with recurrence requiring pal-
liation are pruritus or cholangitis associated with jaundice, or
bowel obstruction associated with carcinomatosis. Additional
goals of follow-up are to detect benign complications of sur-
gical treatment such as biliary stricture. When jaundice or
cholangitis is the presenting symptom of possible recurrence,
a nonsurgical palliative approach using percutaneous trans-
hepatic cholangiogram (PTC) and stenting is usually favored
unless a benign postsurgical stricture is suspected. Because of
the rapid growth of tumor in patients with recurrence, the
hospitalization and recovery time from a surgical bypass is
usually not justified for recurrences resulting in biliary
obstruction.
The routine follow-up of a patient after resection of gall-
bladder cancer includes ofce visits every 3 months with phys-
ical examination and measurement of liver function tests.
Although CA19-9 may be elevated in patients with gallbladder
cancer, the sensitivity and specicity are poor
47
and, thus,
should not be used for screening patients for recurrence.
Because an asymptomatic recurrence of gallbladder cancer has
only limited treatment options, overaggressive use of imaging
studies is not warranted. Therefore, the use of imaging studies
should be individualized.
Issues for the Future
Clearly, improving our ability to recognize early gallbladder
cancer in high-risk geographic areas would have an important
impact on outcome in these patients. This will likely require
improvements in understanding the sequential molecular
changes associated with gallbladder cancer. Other improve-
ments in screening programs in high-risk areas, which could
result in prophylactic cholecystectomy, would likely be
benecial.
48
BILE DUCT CARCINOMA
One of the most technically difcult surgical resections occurs
in patients with bile duct tumors arising in the hepatic hilus,
named Klatskin tumors, or hilar cholangiocarcinoma. Bile
duct cancers can arise at other sites, including within the liver
(intrahepatic cholangiocarcinoma) and below the biliary bifur-
cation but above the pancreas (midbile duct cholangiocarci-
noma). Distal cholangiocarcinoma involves that portion of the
bile duct within the pancreas, which requires pancreaticoduo-
denectomy. The location of the tumor affects prognosis as well
as 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 for
these cancers by providing biliary bypass for jaundiced patients
with unresectable tumors. Because disease is often diagnosed
late in the course and because complex operative techniques
are required for potentially curative resection, these tumors
represent one of the greatest challenges for denitive treatment.
Adding to this is that there are no proved effective options for
adjuvant treatment.
6
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1004 Part Two: Surgical Practice
Incidence
The overall incidence of hilar cholangiocarcinoma in the
United 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 the
United States is approximately 0.7/100,000 with a similar
mortality. During the last 30 years, it appears that both the
incidence and mortality in the United States are increasing.
50
Most recently, population studies have noted that there has
been a trend toward a relative increased incidence of intrahep-
atic cholangiocarcinoma (ICC) compared to extrahepatic
cholangiocarcinoma (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 and
obesity, and smoking being associated only with ICC and not
ECC, 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 50
to 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 chronic
typhoid carriers.
53
Treating patients with cholangiocarcinoma
arising 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
Although
there has been some suggestion of an increased risk of cholan-
giocarcinoma arising after transduodenal sphincteroplasty,
55
it
is difcult 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 invade
locally. More than 95% of these tumors are adenocarcinomas.
They are morphologically described as nodular, which is the
most common, scirrhous, diffusely inltrating, or papillary.
Histologic subtypes include acinar, ductular, trabecular, alveo-
lar, and papillary. Papillary tumors appear to have an improved
outcome. Much less common types of bile duct tumors include
cystadenocarcinomas, hemangioendotheliomas, and mucoepi-
dermoid carcinomas. Perineural invasion is clearly a poor
prognostic sign.
56
In patients with intrahepatic cholangiocarcinoma, negative
prognostic signs include vascular invasion, multiple tumors,
positive margin, large size, and lymph node metastases.
57
These
tumors can be either sclerotic, masslike lesions, or cystic lesions.
Historically, cholangiocarcinomas have been classified
according 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 the
superior border of the duodenum. Lower-third lesions are
found below the superior border of the duodenum but above
the ampulla. The problem with this classication is that the
anatomic landmarks are somewhat arbitrary and not clinically
useful. Most midbile duct malignant obstructions are caused
by gallbladder cancer. Even when it is truly a midbile duct
cholangiocarcinoma, very few of these tumors are amenable to
treatment by local excision of the bile duct. A more useful clas-
sication is to divide these lesions into upper-half or lower-half
tumors, based on the location of the cystic duct as it enters the
common duct (in the case of normal anatomy). The usefulness
of this classication scheme is that it allows the surgeon to
delineate whether a hepatic or pancreatic resection will be
required for clearance of tumor. The AJCC TNM staging sys-
tem (seventh edition) for bile duct cancers is described in
Tables 62.4A and B.
Other staging systems have been created that attempt to
incorporate clinically important indicators of resectability for
hilar cholangiocarcinoma that are dened preoperatively,
including hepatic lobe atrophy or portal vein involvement.
58
With the increasing acceptance of major hepatic resection for
these tumors, this preoperative staging system attempts to
dene whether there is ipsilateral involvement alone, because
tumors with bilateral extension past the primary biliary radi-
cles are not resectable.
Clinical Findings and Diagnosis
The vast majority of patients with cholangiocarcinoma present
with painless jaundice, though mild right upper quadrant
pain, pruritus, anorexia, malaise, and weight loss may also be
reported. Cholangitis is the presenting symptom in 10% to
30% of patients. Some patients have cancer discovered on
evaluation for otherwise asymptomatic elevations of alkaline
phosphatase and gamma-glutamyl transferase.
Patients with intrahepatic cholangiocarcinoma are usually
asymptomatic. Many patients are found to have a liver tumor
present on cross-sectional imaging obtained for other reasons.
Many of these patients will present to the surgeon with a
biopsy showing adenocarcinoma without a known primary
tumor. The standard evaluation in these patients should
include tumor markers to rule out an elevated carcinoembry-
onic antigen (CEA) or -fetoprotein (AFP); upper and lower
endoscopy to evaluate for a gastrointestinal source; CT scan to
assess for a primary tumor in the gastrointestinal tract or pan-
creas; and, in women, a mammogram. If no site of primary
disease is found, in most patients, the diagnosis is intrahepatic
cholangiocarcinoma.
Various imaging tests are available to assess patients with
hilar cholangiocarcinoma. Abdominal ultrasound is noninva-
sive, easily available, and inexpensive, and thus is commonly
used as a rst 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 vein
patency can be determined with ultrasound or helical CT. In
addition, signs of hepatic lobar atrophy should be sought,
because this is associated with a high incidence of ipsilateral
portal vein involvement by tumor. MRCP offers the potential
of evaluating parenchymal, vascular, biliary, and nodal
involvement with a single noninvasive examination.
2022
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 evaluate
the extent of biliary involvement and provide palliation for
jaundice. Endoscopic retrograde cholangiopancreatography
(ERCP) has little role to play in high biliary obstruction
because opacication of the proximal biliary tree is difcult.
ERCP, however, can be effectively used to image more distal
lesions. At the time cholangiography is performed, some
authors advocate the routine preoperative placement of biliary
drainage catheters to aid in intraoperative identication of the
bile ducts.
59,60
Others have found a higher incidence of infec-
tious complications
61
and mortality,
62
and a longer hospital
stay,
63
after preoperative placement of biliary drainage
catheters. The difficulty in making the decision regarding
preoperative 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 require
palliation.
In many cases, it is difcult to obtain pathologic conrma-
tion of cholangiocarcinoma except in very advanced cases,
even with the use of biliary brushings and cytology obtained at
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Chapter 62: Biliary Neoplasms 1005
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FIGURE 62.4. Computed tomography scan in a patient with hilar cholangiocarcinoma, demonstrating dilated intrahepatic ducts in the right lobe
but inability to directly visualize tumor.
AMERICAN JOINT COMMITTEE ON CANCER, 6TH EDITION, STAGING SYSTEM FOR
PERIHILAR BILE DUCT CARCINOMA
TABL E 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 T13 N1 M0
IVA T4 N01 M0
IVB Any T N12 M0
Any T Any N M1
Denition of TNM
Primary tumor (T)
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1 Tumor conned to bile duct, with extension up to the muscle layer or brous 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 hepatic
arter; or the second-order biliary radicals bilaterally; or unilateral second-order
biliary 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, common
bile duct, hepatic artery and portal vein)
N2 Metastasis to periaortic pericaval, superior mesentary artery, and/or celiac artery
lymph 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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1006 Part Two: Surgical Practice
A B
FIGURE 62.5. Coronal (A) and axial (B) magnetic resonance imaging in a patient with hilar cholangiocarcinoma. Dilated intrahepatic ducts are
present with a soft tissue density consistent with tumor (arrows).
AMERICAN JOINT COMMITTEE ON CANCER, 6TH EDITION, STAGING SYSTEM
FOR DISTAL BILE DUCT CARCINOMA
TABL E 62. 4B STAGING
STAGE TUMOR NODES METASTASIS
0 Tis N0 M0
I T1 N0 M0
II T2ab N0 M0
IIIA T3 N0 M0
IIIB T13 N1 M0
IVA T4 N01 M0
IVB Any T N12 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 conned to bile duct, with extension up to the muscle layer or brous tissue
T2 Tumor invades beyond the wall of the bile duct
T3 Tumor invades gallbladder, pancreas, duodenum, or other adjacent organs without
involvement 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, common
bile duct, hepatic artery and portal vein)
N2 Metastasis to periaortic pericaval, superior mesentary artery, and/or celiac artery
lymph 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 are
offered surgical therapy based on clinical suspicion and radi-
ographic appearance. In patients with intrahepatic cholangio-
carcinoma, cross-sectional imaging with CT scan is usually
sufcient. Tumors may be masslike or may have cystic areas.
Surgery
Proximal Cholangiocarcinomas. Untreated, most patients
with 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 are
most commonly hepatic failure or cholangitis related to tumor
growth 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 and
adequate biliary drainage. It has become clear over the last
three decades that curative treatment for patients with tumors
involving the upper half of the bile duct depends on aggressive
excision that often requires a major liver resection. Until as
recently as one decade ago, treatment of hilar cholangiocarci-
nomas was associated with mortality as high as 30%.
6670
Recently, major improvements in the safety of these operations
has been demonstrated, and resection of hilar tumors now
results 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 assessing
resectability. Because of the potential morbidity of a laparo-
tomy that has no therapeutic benet, staging laparoscopy has
been advocated to save patients from unnecessary laparotomy.
In patients with hilar cholangiocarcinoma, up to 25% will
benet from staging laparoscopy because of detection of
occult extrahepatic disease.
24,28
Laparoscopy is a very sensitive
means to detect peritoneal metastases or additional intrahep-
atic disease through the use of laparoscopic ultrasound but is
less sensitive in detecting nodal metastases or locally invasive
tumors.
24
Hilar cholangiocarcinoma is considered unresectable
because of both local factors and metastatic spread. Clearly,
patients with disease outside the liver, including most commonly
peritoneal and intrahepatic metastases, are not amenable to
curative 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 tumor
extension into second-order biliary radicals of both right
and left hepatic lobes. By contrast, tumors extending into
second- or third-order biliary radicles on one side of the liver
without vascular involvement can be resected with curative
outcome.
The goals of surgical management for cholangiocarcinomas
are both eradication of tumor and establishment of adequate
biliary drainage. Tumors of the biliary conuence are particu-
larly difcult to treat because symptoms often appear late in
the course of disease when the lesion has already involved
adjacent structures including the portal vein or adjacent
hepatic parenchyma. Complete resection, therefore, requires
biliary and hepatic resection and often major vascular recon-
struction. It is not surprising, therefore, that in the past the
surgical therapy for proximal biliary malignancies consisted
mainly of biliary-enteric bypass as palliation for jaundice and
cholangitis. The therapeutic approach to hilar cholangiocarci-
noma was largely nihilistic, because of difculty in delineating
the extent of disease and the technical challenge of complete
resection for such lesions.
Over the last decade, surgical approaches have become
more aggressive, as demonstrated by the increasing number of
hepatic resections that have been performed for bile duct can-
cers.
6670
Recent improvements in ultrasound, CT, MRI, and
angiography have greatly facilitated preoperative diagnoses
and staging of cholangiocarcinoma. This has allowed improved
patient selection and surgical planning. The location and local
extension of tumor dictates the extent of resection, with most
lesions requiring an extended right or left hepatectomy for com-
plete excision. Caudate resection is often required because of
direct extension into caudate biliary radicles or parenchyma.
58,69,70
CBD excision and portal lymphadenectomy are also essential
for tumor clearance.
Liver Transplantation for Hilar CCA and the Mayo
Protocol. Orthotopic liver transplantation (OLT) was
thought to hold promise for patients with hilar CC, because of
its 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 rates
have been reported at 25% to 30%.
7375
Because of this poor
outcome, the Mayo Clinic developed a protocol combining
neoadjuvant therapy with liver transplant, based on a strategy
initially developed by the University of Nebraska. This protocol
uses high-dose neoadjuvant 5-uorouracil and brachytherapy
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followed by liver transplantation.
72,76
Inclusion criteria
include: (i) locally advanced unresectable disease with either
positive intraluminal brush cytology, positive intraluminal
biopsy, or CA19-9 100 in the setting of a radiographic
malignant stricture; (ii) primary sclerosing cholangitis with
resectable disease, and; (iii) absence of medical contraindica-
tions for OLT. Since 2003, biliary aneuploidy as demon-
strated by digital image analysis (DIA)
77
and uorescent in
situ hybridization have been considered equivalent to cytol-
ogy.
78
Exclusion criteria include: (i) extrahepatic disease
including regional lymph node involvement; (ii) uncontrolled
infection; (iii) prior attempt at resection; (iv) prior treatment
with radiation or chemotherapy; and (v) previous malignancy
within 5 years. In this protocol, patients receive external
beam radiotherapy to a target dose of 4,500 cGy with con-
comitant uorouracil (5-FU). Following this, transcatheter
iridium-192 brachytherapy with a target dose of 2,000 to
3,000 cGy is administered. Thereafter, patients receive oral
capecitabine as tolerated until transplantation. It is important
that, prior to transplantation, patients undergo a staging
laparotomy, at which time biopsy of perihilar lymph nodes as
well as any lymph nodes or nodules suspicious for tumor is
performed. Only patients with negative staging operations
remain eligible for transplantation.
72
Thus, patients eligible for OLT under this protocol have
locally 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 an
opportunity to exclude patients demonstrating disease pro-
gression. This highly rigorous selection bias in favor of
patients with biologically favorable disease is reected in the
early outcomes published from the Mayo group. In a recent
review of 71 patients enrolled in this transplant protocol, only
38 underwent transplantation (38%). These patients were
compared to 26 patients (of 54 explored, 48%) who under-
went successful resection. When compared to those undergo-
ing resection (some with node-positive disease), patients
undergoing transplantation were younger (p 0.001) and
more likely to have inammatory bowel disease (p 0.03) and
PSC (p 0.001). It is important that only 58% of patients had
histologically 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 fewer
recurrences in the transplant patients (13% vs. 27%), and
recurrences became apparent later after transplantation than
after resection (mean 40 months vs. 21 months). Direct com-
parisons are difcult with this study given the differences
between groups. At present, OLT cannot be considered a stan-
dard form of therapy for hilar cholangiocarcinoma for
patients with resectable disease, but it does offer a potential
option for patients with underlying PSC or those with unre-
sectable tumors who t the rigorous inclusion and exclusion
criteria of the protocol.
Prognosis after Resection. Results of major studies on
resection of hilar cholangiocarcinoma are summarized in Table
62.5. In patients amenable to curative resection, the median
survival is 35 months with a 5-year survival rate of 10% to
30%.
61,68,69,7280
Surgical resection provides both improved sur-
vival and improved quality of life.
79,80
The greatest risk factors
for recurrence include the presence of positive margins
79,81
and
node-positive tumors.
82
Prognosis after Resection for Intrahepatic or Distal
Cholangiocarcinoma. 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% to
45%.
84
Patients with unresectable disease have a median sur-
vival of 12 months.
85,86
Thus, completely resected intrahepatic
cholangiocarcinoma appears to have an improved prognosis
over proximal (hilar) cholangiocarcinoma.
Most patients with distal cholangiocarcinomas are deni-
tively managed with pancreaticoduodenectomy (Whipple pro-
cedure) to obtain adequate clearance of tumor, because of the
intrapancreatic location of the distal CBD. Patients with cholan-
giocarcinomas arising in the distal bile duct have both an
increased resectability rate and improved prognosis over those
with hilar cholangiocarcinomas.
71
Patients with resectable distal
bile 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, signicant improvement in quality of life can occur with
surgical bypass. Palliative bypass can be performed in several
1008 Part Two: Surgical Practice
RESULTS AFTER RESECTION FOR HILAR CHOLANGIOCARCINOMA
TABL E 62. 5 RESULTS
SURVIVAL (MONTHS)
PERCENT POSTOPERATIVE 5-YEAR
AUTHOR N RESECTED MORTALITY (%) SURVIVAL (%) MEAN MEDIAN
Cameron et al., 1990
59
96 55 2 8 18
Hadjis et al., 1990
70
131 21 7 12 25
Altaee et al., 1991
66
70 21 19 12
Baer et al., 1993
67
48 44 4 23 34
McMasters et al., 1997
123
91 44 0 26 22
Klempnauer et al., 1997
124
151 10 28 24
Nagino et al., 1999
125
173 80 10 26
Nakeeb et al., 2002
126
72 57 34
Jarnagin et al., 2001
127
225 71 10 27 35
Rea et al., 2004
72
46 9 26 28
Jang et al, 2005
128
48 0 48
Konstadoulakis et al., 2008
129
73 81 7 35
Yubin et al., 2008
130
115 100 2 22 40
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B D
FIGURE 62.7. Surgical approach to segment III duct. A: The bridge of tissue present at the base of the liver is divided. B: The
ligamentum 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 require
hepatic parenchymal transection. One technique involves biliary
decompression through the left duct, approached through the
round ligament, a segment III bypass (Fig. 62.7). Opening the
bridge of tissue just beneath the ligamentum teres allows access
to the duct. In this position, a long anastomosis can be per-
formed from the segment III duct to a jejunal limb because of
the horizontal course of the duct in this location. Although less
commonly used, the right hepatic duct can be approached at
the base of the gallbladder fossa. This is technically more dif-
cult and results in a higher rate of late bypass failure.
88
Nonoperative palliative biliary decompression can be
accomplished with percutaneous or endoscopic stenting,
depending on the level of obstruction. Proximal lesions are usu-
ally approached percutaneously with placement of expandable
stents or drainage catheters (Fig. 62.8). Internal stents result in
fewer electrolyte abnormalities and improvement in patient
comfort, although morbidity and mortality occurs in up to 30%
of patients and stent occlusion is common.
8991
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 are
also 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 short
median survival, those whose disease is clearly unresectable on
preoperative imaging should undergo percutaneous internal or
external drainage. In patients who undergo exploratory
surgery and whose disease is found to be unresectable, surgical
bypass offers fewer episodes of cholangitis, with an improved
quality of life. In some series, surgical bypass for patients with
unresectable disease is the only biliary drainage procedure ever
required by the patient.
In patients with unresectable distal cholangiocarcinomas,
palliation can be achieved with surgical bypass, percutaneous
biliary drains, or ERCP-placed stents. The simplest and most
effective way to relieve jaundice is usually with an ERCP stent.
Although surgical bypass offers improved patency and fewer
episodes of cholangitis, the morbidity of the procedure is not
warranted in patients with these aggressive tumors.
INTRAHEPATIC
CHOLANGIOCARCINOMA
Patients with intrahepatic cholangiocarcinoma typically pre-
sent with single liver lesions. The procedure of choice is
anatomically based hepatic resection. Because these tumors
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