CHOLANGIOCARCINOMA

SGD B08
INTRODUCTION
CHOLANGIOCARCINOMA
Malignancy of the epithelium of the biliary tree.
The most common biliary tract malignancy.
Affect one in every 100,000 people per year in
the United States.
Slightly more prevalent in males than females
(1.3:1.0) and usually affects patients in the fifth
to seventh decade of life.
Represents a diverse group of epithelial cancers
united by late diagnosis and poor outcomes
prognosis is considered dismal.
Treatment and long-term prognosis are
dependent upon the location of the mass.
Surgical treatment is the preferred option.
DEFINITION
CHOLANGIOCARCINOMA

Cholongiocarcinoma is an epithelial cell

malignancy merging from different areas inside
biliary tree
Based on anatomical location:

Intrahepatic
Cholangiocarcinoma(iCCA)
Perihilar
Cholangiocarcinoma(pCCA)
Distal Cholangiocarcinoma(dCCA)
ETIOLOGY
 Cholangiocarcinoma has developed without an
identifiable etiology
Risk factors are
primary sclerosing cholangitis (PSC)
Viral hepatitis (HCV & HBV)
Parasitic infections (Opisthorchis viverrini and
Clonorchis sinensis )
Cholelithiasis and choledocholithiasis
Toxins (smoking, alcohol)
Diabetes
PATHOPHYSIOLOGY
 Cholangiocarcinoma
assosiated with:

GENETIC OR
INFLAMMATION MOLECULAR
THEORY THEORY
INFLAMMATION THEORY

Chronic
Inflammator Nitrosative DNA Carcinogene
Inflammatio iNOS active
y Cytokines stress damage sis
n
INFLAMMATION THEORY
Increase
Activate
IL-6 expression
STAT3
of MCL1
CCA
developme
nt increase

Increase
Cell
Proliferation
CHOLESTASIS

Tumor
ERBB2
Formation
Bile Activate
Acid EGFR Invasivenes
HGF s and Tumor
Metastasis
GENETIC OR MOLECULAR THEORY
CODON 12

K-ras CODON 13

MUTATION
CODON 61
OF p53

TGF
SMAD4 signalling
network
SIGN & SYMPTOMS
Mostly asymptomatic.
Unspecific symptoms:
Malaise
Abdominal pain
Weight loss
Hepatomegaly
Tumor mass
Dilated gallbladder
Symptoms that can present on extrahepatic patient are:
Dark urine
Pruritis
Painless jaundice
Pale stools
DIAGNOSIS
 DIAGNOSIS

Klatskin
iCCA pCCA dCCA
Tumors

Imaging Imaging Imaging Imaging

Bismuth-
Tumor Tumor
Corlette
marker marker
system

Genetic Genetic
marker marker
DIFFERENTIAL DIAGNOSIS
 GALLBLADDER CANCER
HEPATOCELLULAR CARCINOMA
CHOLANGITIS AND
CHOLEODOCHOLITHIASIS
MANAGEMENT
Treatment Cholangiocarcinoma

Surgical
Transplantation
Resection

Photodynamic
Therapy (PDT)

Radiation
Chemotherapy
Therapy
Prevention Cholangiocarcinoma

Health Behavior Model

good
compliance
changing toward
attitude therapy

enhancing
knowledge
about disease
and risk factor
PROGNOSIS
 Prognosis
remains poor
Intrahepatic cholangiocarcinoma Extrahepatic cholangiocarcinoma
Stage 5-year relative Stage 5-year relative
survival survival
Localiz 15% Localiz 30%
ed ed
Region
Source: 6% Society
American Cancer Region 24%
al al
Factors that affecting patient survival have been impeded by:
Distant 2% Distant 2%
lack of disease-specific symptoms in early stages
surgical resection not being an appropriate treatment option
high rates of tumor recurrence following surgical resection

od prognosis associated with:

a. Surgery

modality providing the best survival prognosis for patients

offers the most beneficial curative option and outcome; R0 resection
offers the best possibility of long-term survival
 Prognosis
Chemotherapy or radiotherapy
Treatment using chemotherapy or radiotherapy was found to prolong
survival of ICC patients with unresectable tumors; ex. gemcitabine-based
adjuvant
Distal chemotherapy
or Middle Part of Bile Duct
these tumors result in complete bile duct obstruction leading to early
clinical symptoms including jaundice and cholangitis
location was not an independent prognostic factor
surgical procedure should be extended, depending on the location of
the lesion closer to liver or pancreas in other to improve the survival
d. Other factors
better liver preservation normal ALP levels
lower CA19-9 levels (<100 UmL)
no relapse are associated
less aggressive tumor conditions
lower bilirubin levels
highly differentiated tumor
early stages of TNM staging
no intraoperative blood transfusion
 Prognosis
r prognosis associated with:
tumor differentiation
tumor staging
lymph node metastasis
nerve invasion
intravascular cancer emboli
the depth and distribution of infiltration
tumor size >2 cm
successful tumor resection; positive tumor resection
margin (strongest factor)
location of tumor
presence of biliary drainage
microvascular invasion
complication