This presentation highlights the important information regarding diagnosis and management of biliary tract cancers

1. Biliary Tract Cancer
Presented By : Dr Ankit Lalchandani
Moderated By : Dr MP Singh

2. 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

3. • Others :
• Porcelain GB
• Adenomatous polyps
• S. Typhi infection
• Radon exposure
• APBDJ

4. • 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

5. Pathogenesis
p53
CarcinomaCISDysplasia
Chronic
inflammation
APBDJ
Gall
Stone
K ras

6. • 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

7. 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)

8. Diagnostic Imaging
• USG abdomen
• Asymmetrical wall thickening
• GB mass
• Loss of normal GB- liver interface

9. • CECT Abdomen
• To assess local invasion
• Vascular invasion
• Lymph node involvement
• Distant Metastasis
• MRI/MRCP
• Delineates invasion into porta hepatis

10. • ERCP/PTC :
• used primarily for palliation or preoperative management of obstructive
jaundice
• FNAC/ Biopsy :
• Contraindicated if imaging features suggestive of resectable disease

11. AJCC Staging

13. 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 %)

14. 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

15. 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

16. 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

17. 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

18. 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

19. Palliation
• Goals :
• Relieve pain : Opioid analgesics
• Biliary obstruction : ERCP and stenting/ PTBD
• Bowel obstruction : Endoscopic Duodenal stenting
• Chemotherapy : Gemcitabine + cisplatin ( ABC-02 trial)

20. Summary
Cholecystectomy
Extended
Chole.
Major hepatic
resection
Palliation

21. 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

22. • 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%)

23. • Bismuth classification

24. 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

25. 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)

26. • 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

27. AJCC Staging

30. 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

31. • Contraindications:
• Involvement of inflow and outflow bilaterally
• Multiple intrahepatic tumors
• Metastatic disease
• Lymph node dissection
• No therapeutic value
• May help in staging and prognosis

32. 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)

33. • 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

34. 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

35. 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

36. THANK YOU