Biliary Tract Cancer

Presented By : Dr Ankit Lalchandani

Moderated By : Dr MP Singh
GB Cancer
• High prevalence in north India

• Incidence increases with age

• Women > men

• Risk Factor : chronic inflammation due to gall stones

• Gall stone present in 70-90% patients
• But only 0.5-3 % patients with gall stones develop GB cancer
• Others :

• Porcelain GB

• Adenomatous polyps

• S. Typhi infection

• Radon exposure

• APBDJ
• Abnormal Pancreaticobiliary Duct Junction

• Long common channel and increased tone of Sphincter of Oddi

• Reflux of pancreatic secretions into CBD chronic inflammation

• Carcinoma occur at a younger age

• Not associated with cholelithiasis

Pathogenesis

p53
Gall
Stone
Chronic
Dysplasia CIS Carcinoma
inflammation

APBDJ

K ras
• Histology
• Adenocarcinoma ( 80%)
• Small cell
• Squamous cell CA
• Lymphoma

• Morphology
• Infiltrative : diffuse growth, difficult to recognize on imaging, metastasize
early
• Papillary : Project into the lumen, less likely to metastasize , best prognosis
Clinical Presentation and diagnosis
• Symptoms

• Early : asymptomatic / mild abdominal pain, anorexia, nausea

• Advanced : weight loss, hepatomegaly, ascites

• Blood investigations

• Suggest obstructive jaundice

• Tumor markers CEA/ CA 19-9 may be elevated ( low sensitivity and specificity)
Diagnostic Imaging
• USG abdomen

• Asymmetrical wall thickening

• GB mass

• Loss of normal GB- liver interface

• CECT Abdomen
• To assess local invasion
• Vascular invasion
• Lymph node involvement
• Distant Metastasis

• MRI/MRCP
• Delineates invasion into porta hepatis
• ERCP/PTC :

• used primarily for palliation or preoperative management of obstructive

jaundice

• FNAC/ Biopsy :

• Contraindicated if imaging features suggestive of resectable disease

AJCC Staging
Surgical Management
• Macroscopically complete surgical resection with negative margin
(R0) remains the only curative treatment

T1a
• Tumors confined to lamina propria
• Incidental finding post cholecystectomy
• Simple cholecystectomy alone is definitive
( 5yr survival 97-99%, Recurrence 0.6-3.4 %)
T1b

• Tumors invade muscularis propria

• Rates of residual disease in GB fossa after simple cholecystectomy ~10%

• Rate of lymph node positivity ~15%

• Extended cholecystectomy with en bloc resection of adjacent liver

parenchyma to include segment IVb and V + Regional LN dissection

• Bile duct resection only if cystic duct margin is positive

T2

• Tumors extend through perimuscular connective

tissue

• Nodal positivity rate 39-46%

• Recommended Tx : same as T1b

• Simple cholecystectomy is done in subserosal plane,

increased risk of residual disease

• If diagnosed postcholecystectomy : Re exploration

and radical resection and re excision of all port sites
T3

• Tumors invade GB serosa and/or invade

liver or an adjacent organ

• Major hepatic resections may provide

survival advantage if disease is limited
to periportal lymph nodes

• 5 yr survival 16-39%

• As GB fossa bridges IVb and V ,

extended right hepatectomy may be
required
T4

• Tumors invade 2 or more adjacent organs or invade main portal vein/hepatic

artery
• Unresectable

Lymph Node dissection :

• Portal LN dissection recommended for T1b –T4

(porta hepatis, gastrohepatic ligament, retroduodenal)
• AHPBA recommends at least 6 LN to be dissected
• N2 disease ( celiac, retropancreatic, inter aortocaval) is unresectable
Staging Laparoscopy:
• Identifies those with unresectable disease when imaging studies are
equivocal
• High yield in T3 disease (30-50%)

Adjuvant Therapy :
• EBRT +/- 5 FU is associated with low rates of local recurrence
• Not standard recommendation
Palliation

• Goals :
• Relieve pain : Opioid analgesics
• Biliary obstruction : ERCP and stenting/ PTBD
• Bowel obstruction : Endoscopic Duodenal stenting

• Chemotherapy : Gemcitabine + cisplatin ( ABC-02 trial)

 Summary

Cholecystectomy
Extended
Chole.
Major hepatic
resection

Palliation
Cholangiocarcinoma
• Involves intrahepatic and extrahepatic biliary channels
• Incidence men > women
• Risk Factors
• Primary Sclerosing cholangitis
• Liver flukes (Opisthorchis and chlonorchis)
• Choledochal cyst
• Carolis disease
• Hepatolithiasis
• Thorotrast
• Hepatitis C
• 90% are adenocarcinomas

• Morphology :
• Sclerosing : intense desmoplastic reaction, highly invasive, low resectability
• Nodular : constriction annular lesions, low resectability
• Papillary : rare, bulky masses projecting into the lumen, cause jaundice early,
high resectability

• Location :
• Intrahepatic (10%)
• Perihilar (65%)
• Distal ( 25%)
• Bismuth classification
Clinical Presentation
• Intrahepatic
• Present with non specific symptoms
• May have Increased ALP with normal bilirubin
• Extrahepatic
• Present with painless obstructive jaundice
• Unilobar bile duct obstruction may present with unilobar atrophy with
compensatory contralateral hypertrophy
• Tumors arising at or below the bifurcation present early

• CEA/CA 19-9 have low sensitivity and specificity, not routinely used as
diagnostic tool
• May be used for surveillance among patients with PSC
 Diagnosis

• CECT
• Site and extent of the primary
• Vascular invasion
• Lymph node involvement
• Distant metastasis
• Unilobar Liver atrophy with
contralateral hypertrophy
( s/o unilobar bile duct
infiltration by tumor)
• Cholangiography
• PTC : for intrahepatic and perihilar tumors
• ERCP : For distally located tumors
• MRCP : non invasive, no ionic contrast used,
can visualize bile ducts both proximal and
distal to stricture

• Cytology
• Indicated for stricture in PSC to rule out
malignancy
• ERCP guided brush cytology  EUS guided FNA
AJCC Staging
Surgical Management
Intrahepatic Cholangiocarcinoma

• Major hepatic resection with negative margins is curative

( +/- EHBD, vascular resction)

• 5 yr survival in R0(39%) vs R1(4.7%)

• Surgery recommended only if R0 possible

• Contraindications:
• Involvement of inflow and outflow bilaterally
• Multiple intrahepatic tumors
• Metastatic disease

• Lymph node dissection

• No therapeutic value
• May help in staging and prognosis
Perihilar Cholangiocarcinoma

• R0 resection may require partial hepatectomy along with EHBD

resection
• Include resection of caudate lobe for tumors involving confluence
• Frozen section should be performed to ensure negative margins
• Secondary R0 resection vs primary R0
• Survival is equivalent
• Increased incidence of biliary fistula after additional resection
• After R0, 5 yr survival (20-40%, median 36 months) with high
recurrence rates (68% within 24 months)
• Contraindications
• Hepatic duct involvement with tumor extension bilaterally to second order radilcles
• Encasement of main portal vein
• Lobar atrophy with tumor involvement of contralateral second order biliary radicles
• Lobar atrophy with tumor involvement of contralateral portal vein branches
• Distant metastasis

• Lymph Node dissection

• Include nodes along hepatoduodenal ligament
• Inclusion of lymph nodes along common hepatic or coeliac axis is not recommended
• For accurate staging, at least seven LN recommended
Distal Cholangiocarcinoma

• Most commonly along the pancreatic portion

• High rates of lymph node (63%) and pancreatic invasion (87%)

• Resection involves pancreaticoduodenectomy and lymphadenectomy

• After R0, 5 yr survival ( 27-44%), median survival 18 months

Palliation
• Goal : Relieve biliary obstruction
• Biliary stenting
• Percutaneous : proximal tumors
• Endoscopic : Distal tumors
• Bismuth Type 1 require single stent while others may require two or
more
• Plastic stents patency (3-6 months) vs metal stents ( 8-12 mo)
• Chemotherapy : Gemicitabine + cisplatin
THANK YOU