CHOLANGIOCARCINOMA

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CHOLANGIOCARCINOMA

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:

INFLAMMATION GENETIC OR
THEORY MOLECULAR THEORY
INFLAMMATION THEORY

Inflamm
Chronic
atory iNOS Nitrosati DNA Carcinog
Inflamm
Cytokine active ve stress damage enesis
ation
s
Ac Incr
CCA
eas
tiv
INFLAMMATION THEORY
e
dev
at elo
exp
IL- pm
e ress
ent
6 ST ion
incr
of
Increase Cell
Proliferation
AT MC
eas
e
3 L1
CHOLESTASIS

Bile Acti ERBB2


Tumor
Formation
vate
Aci EGF Invasiveness
HGF
d R
and Tumor
Metastasis
GENETIC OR MOLECULAR THEORY

MUTATION OF p53

K-ras SMAD4
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

iCCA pCCA dCCA Klatskin Tumors

Imaging Imaging Imaging

Imaging Bismuth-Corlette system


Tumor marker Tumor 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


changing attitude good compliance toward therapy
PROGNOSIS
Prognosis
remains poor
Intrahepatic cholangiocarcinoma Extrahepatic cholangiocarcinoma
Stage 5-year relative survival Stage 5-year relative survival
Localized 15% Localized 30%
Regional 6% Regional 24%
Distant 2% Distant 2%
Source: American Cancer Society

Factors that affecting patient survival have been impeded by:


• lack of disease-specific symptoms in early stages
• surgical resection not being an appropriate treatment option
• high rates of tumor recurrence following surgical resection

Good 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
b. Chemotherapy or radiotherapy
Treatment using chemotherapy or radiotherapy was found to prolong survival of ICC
patients with unresectable tumors; ex. gemcitabine-based adjuvant chemotherapy
c. Distal 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 U⁄mL) • no relapse are associated
• less aggressive tumor conditions
• lower bilirubin levels
• highly differentiated tumor
• early stages of TNM staging
• no intraoperative blood transfusion
Prognosis
Poor 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

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