Ch. 54 – Biliary System

1586 Section X Abdomen
leads to high local recurrence rates due to early
involvement of the confl uence of the hepatic
ducts and the caudate lobe branches. The addition
of a modifi ed hepatic resection has improved
resectability rates. However, curative resections are
still possible in less than half of patients, and most
do not achieve long-term disease control. Surgical
treatment depends on the Bismuth-Corlette
classifi cation (see Fig. 54-35). For type I and II
lesions, the procedure is en bloc resection of the
extrahepatic bile ducts and gallbladder with 5- to
10-mm bile duct margins, and regional
lymphadenectomy with Roux-en-Y
hepaticojejunostomy. In addition to the above
operations, type II tumors may require hepatic
lobectomy. Because type II and III lesions often
involve the ducts of the caudate lobe, many
surgeons recommend routine caudate lobectomy.
Type III and IV tumors are amenable to
potentially curative resection in centers with
expertise in these procedures. Aggressive
techniques such as hepatectomy and portal vein
resection to achieve negative margins are now
routine in specialized centers.
Substantial progress has been made in curative resection
for perihilar cholangiocarcinomas. At least some of
this progress has been attributed to the routine use of
partial hepatectomy. The rate of margin-negative resections
is consistently more than 75% when partial hepatectomy
(including resection of the caudate lobe) is
added to the bile duct resection. This aggressive approach
has resulted in 5-year survival rates above 50% in some
series. However, these improvements have been accompanied
by higher surgical mortality rates (8%-10% versus
2%-4%). The major prognostic factors are margin status
and tumor stage. In addition to location, stage, and status
of the resection margins, other factors infl uence outcome
after resection.
Medical Therapy
Numerous reports have suggested that radiation therapy
improves survival for patients with cholangiocarcinoma,
especially when resection is impossible. External-beam
radiotherapy has been delivered using a variety of innovative
techniques, including intraoperative radiotherapy
and brachytherapy with iridium-192 through percutaneous
or endoscopic stents. However, no prospective, randomized
trials have been reported, and a well-controlled,
but not randomized, trial reported no benefi t for postoperative
adjuvant radiation. A survival benefi t for postoperative
radiation therapy may be limited to patients with
local extension into the liver parenchyma and microscopic
residual disease following resection. Chemotherapy
has also not been shown to improve survival in
patients with either resected or unresected cholangiocarcinoma.
Given the potential radiosensitization effect of
5-fl uorouracil or gemcitabine, the combination of radiation
and chemotherapy may be more effective than either
agent alone. As with gallbladder cancer, the role of adju-
vant chemoradiation needs to be tested in patients with
cholangiocarcinoma. Finally, photodynamic therapy is
emerging as an important palliative option for patients
with unresectable cholangiocarcinoma, although it is not
widely available.
Outcomes
Long-term survival in patients with cholangiocarcinoma
is highly dependent on the stage of disease at presentation
and on whether the patient is treated by a palliative
procedure or complete tumor resection. The rate of
margin-negative resections has consistently been reported
above 75% when partial hepatectomy including resection
of the caudate lobe is added to the biliary resection. 49,50
This aggressive approach has increased 5-year survival
rates to above 50% in some series. 47,49 However, the perioperative
mortality rates accompanying these more
extensive resections are slightly higher than those accompanying
local excision only (8%-10% versus 2%-4%). 49,51,52
Patients with resectable distal bile duct cancer have the
highest rate of resection. Those with resectable distal bile
duct cancer have a median survival of 32 to 38 months
and a 5-year survival rate of 28% to 45%. Even with
multimodality adjuvant therapy, median survival for unresectable
intrahepatic tumors has been only 6 to 7 months.
Similarly, median survival for patients with unresectable
perihilar tumors varies between 5 and 8 months.
The use of liver transplantation for the treatment of
cholangiocarcinoma is controversial and should be
reserved for select patients as a part of research protocols.
As more effective adjuvant and neoadjuvant protocols
are developed, transplantation may be a more useful
treatment for this disease. As indicated previously, this is
suggested by early reports from the Mayo Clinic in which
survival after neoadjuvant chemoradiation and liver transplantation
was signifi cantly improved over resection
alone for stage I and II hilar cholangiocarcinoma. 40
Metastatic and Other Tumors
Hepatocellular carcinoma and liver metastases can cause
obstructive jaundice by direct extension into the perihilar
bile ducts. Hepatocellular and metastatic colorectal carcinoma
have also both been reported to “embolize” into
the biliary tree. This rare phenomenon occurs when
tumor cells are shed into the biliary tract and implant
distally, leading to biliary obstruction when the tumor
embolus increases in size. Hepatic cystadenomas and
cystadenocarcinomas arise from the biliary epithelium,
and these tumors or the mucin they produce may also
cause bile duct obstruction.
Primary and secondary hepatic tumors can also
produce biliary obstruction by metastasizing to hilar or
pericholedochal lymph nodes. Hepatocellular carcinoma,
colorectal carcinoma, and pancreatic carcinoma are the
most common primary sites associated with biliary tract
obstruction from lymph node metastases, although nodal
metastases from a number of tumors including breast and
ovarian cancer have been reported to cause bile duct
obstruction. Lymphoma can also result in biliary obstruc-