Ch. 54 – Biliary System

1582 Section X Abdomen
Survival
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Figure 54-34 Survival following surgical resection for T2 gallbladder
cancer. Patients undergoing radical resection (triangles) are
compared with patients undergoing simple cholecystectomy
(circles) (P > .05). (From Fong Y, Jarnigan W, Blumgart LH:
Gallbladder cancer: Comparison of patients presenting initially
for defi nitive operation with those presenting after prior noncurative
intervention. Ann Surg 232:557-569, 2000.)
in 10-year survival has been demonstrated after simple
cholecystectomy (100%) and extended cholecystectomy
(75%) among patients with T1b gallbladder cancer. Invasion
into the subserosa (T2) increases the risk for regional
lymph node metastases to between 33% and 50%. Fiveyear
survival in patients with T2 tumors is improved following
extended cholecystectomy with lymphadenectomy
and liver resection (59%-61%) compared with simple
cholecystectomy (17%-19%) 43 (Fig. 54-34). Several groups
have recently reported 5-year overall survival rates for
resected patients with stages IIA and IIB gallbladder
cancer of 28% to 63% and 19% to 25%, respectively.
However, most patients with gallbladder cancer have
advanced unresectable disease at the time of presentation.
As a result, fewer than 15% of all patients with
gallbladder cancer are alive after 5 years. The median
survival for stage IV patients at the time of presentation
is only 1 to 3 months.
Bile Duct Cancer
Cholangiocarcinoma is an uncommon tumor that may
occur anywhere along the intrahepatic or extrahepatic
biliary tree. These tumors are located most commonly at
the hepatic duct bifurcation (60%-80% of cases). Less
commonly, tumors originate in the distal common bile
duct or in the intrahepatic bile ducts. Most cholangiocarcinomas
present with jaundice, and the diagnosis
of cholangiocarcinoma should be considered in every
patient with obstructive jaundice. When possible, surgical
resection does offer a chance for long-term disease-free
survival. Many patients, however, will be candidates only
for palliative bypass or operative or nonoperative intubation
aimed to provide biliary drainage and prevent cholangitis
and hepatic failure.
Incidence
The reported incidence of cholangiocarcinoma in the
United States is 1 or 2 cases per 100,000 population.
Incidence data from the American Cancer Society are
diffi cult to interpret because intrahepatic bile duct cancers
are included with primary liver cancers, whereas extrahepatic
biliary cancers are in a separate category that
includes gallbladder cancer. In the United States, an estimated
17,550 primary liver cancers will be diagnosed in
2005. Data from the National Cancer Institute Surveillance,
Epidemiology, and End Results (SEER) program
suggest that about 15% of these (2600 cases) will be
intrahepatic cholangiocarcinomas. About 7000 cases of
extrahepatic bile duct cancer are diagnosed annually in
the United States, two thirds of which are gallbladder
cancers. Thus, 2000 to 3000 cases per year are extrahepatic
cholangiocarcinomas.
For unclear reasons, the incidence of intrahepatic cholangiocarcinoma
has been rising over the past 2 decades
in Europe and North America, Asia, Japan, and Australia,
whereas rates of extrahepatic cholangiocarcinoma are
declining internationally. The rising rates of intrahepatic
cholangiocarcinoma have not been associated with an
increase in the proportion of early-stage or smaller
lesions. Furthermore, incidence rates do not appear to
have reached a plateau.
Risk Factors
A number of diseases have been linked to cholangiocarcinoma,
including primary sclerosing cholangitis, choledochal
cysts, and hepatolithiasis. Characteristics common
to these diseases include bile duct stones, biliary stasis,
and infection. Bile duct cancers in patients with primary
sclerosing cholangitis are most often extrahepatic, commonly
occur near the hepatic duct bifurcation, and are
diffi cult to differentiate from the multiple, benign strictures
associated with this disease. As a general rule, the
incidence of biliary tract cancers increases with age; the
typical patient with cholangiocarcinoma is between 50
and 70 years of age. However, patients with PSC and
those with choledochal cysts present nearly 2 decades
earlier. In contrast to gallbladder cancer, for which female
gender predominates, the incidence of cholangiocarcinoma
is slightly higher in men. This probably refl ects the
higher incidence of PSC in men. Hepatolithiasis is also a
defi nite risk factor for cholangiocarcinoma, which will
develop in 5% to 10% of patients with intrahepatic stones.
Hepatitis B and C are also now recognized as risk factors
in the development of intrahepatic cholangiocarcinoma.
At least two genetic disorders are associated with an
increased risk for cholangiocarcinoma: the inherited
“cancer family” syndrome termed Lynch syndrome II, and
a rare inherited disorder called multiple biliary papillomatosis;
the latter condition is characterized by multiple
adenomatous polyps in the bile ducts, and repeated
episodes of abdominal pain, jaundice, and acute
cholangitis.
Prior biliary-enteric anastomosis may also increase
the future risk for cholangiocarcinoma. Five percent of
patients in a large Italian series developed cholangiocar-