1. Cholangiocarcinoma
Introduction and management
Prepared by
Dr Shambhavi Sharma

2. Anatomy of biliary tree

4. Introduction
• All tumors originating from bile duct epithelium
• 90% adenocarcinoma

5. Risk factors
• Chronic Inflammation of proximal biliary tree
• Cirrhosis
• HBV infection
• Choledocholithiasis, hepatolithiasis
• Choledochal cyst
• Biliary / hepatic parasitic infection – Opisthorcis viverrini, Clonorchis
sinensis
• Primary sclerosing cholangitis

6. Clinical presentation
Intrahepatic Extrahepatic
Late presentation Jaundice
Fever Signs of biliary obstruction
Weight loss Imaging suggestive of bile duct
obstruction
Abdominal pain
Incidental finding of isolated hepatic
mass

7. • Investigation for diagnosis and staging
• LFT
• USG abdomen and pelvis
• CEA/ CA 19.9
• Triple phasic CECT/MRI abdomen
• NCCT chest
• Staging laparoscopy
• AFP – intrahepatic cholangiocarcioma
• Investigation for evaluating fitness of surgery
• CBC, RFT, PT/ INR, Serum Albumin, ECG

8. Role of Ultrasonography
• Klatskin :
• Segmental dilatation and nonunion of
right and left ducts at porta hepatis
• Dilated intrahepatic ducts with a
normal-caliber extrahepatic duct
• Distal cholangiocarcinoma:
• stricture
• polypoid mass
• GB distended
• Intrahepatic cholangiocarcinoma

9. Triphasic CECT abdomen/ pelvis
1.Diagnosis :
• Arterial and portal venous phase :
• Hypoenhancing soft tissue infiltration with
Mass forming- hypovascular lesion with
peripheral rim enhancement
• delayed phase: central rim enhancement
2.Evaluation of Resectibility
Advantages :
Extent of vascular involvement
Biliary anatomy, vascular anatomy
Relation of tumor
Ductal dilatation

10. Lobar atrophy Klatskin tumor
Intrahepatic cholangiocarcinoma

11. MRCP and MRI
• Delineates extent of biliary involvement
• helpful for the resection margin
Hilar cholangiocarcinoma

12. Role of endoscopic ultrasound
• Done if CBD is dilated but no mass is seen in CT/MRI
• Can define mass or abnormal thickening
• Biopsy

13. ERCP and Brush cytology
• Preoperative biopsy usually not required in resectable
• Brush cytology : Poor sensitivity (27 -56%) and low cellular yield
Indications:
• Unresectable disease : for diagnosis
• If intervention is required
• palliative stenting
• preoperative biliary drainage

14. Role of PET SCAN
In patients with resectable disease
• Helps in identifying occult metastases, nodal involvement
detect nodular CC as small as 1 cm but is less helpful for infiltrating
tumor

15. Staging
• AJCC
• Bismuth classification for hilar carcinoma
• Blumgart staging

16. Hilar cholangiocarcinoma
• Bismuth and Corlette classification
does not include vascular encasement, LN involvement, distant metastases and liver atrophy

18. Blumgart staging
Confluence with B/L extension to 2nd order biliary radicles; or U/L extension
to 2nd order biliary radicles with contralateral PV involvement; or U/L
extension to 2nd order biliary radicles with contralateral hepatic lobar
Atrophy; or main or B/L PV involvement
Biliary confluence with U/L extension to 2nd order Biliary radicles
Confluence with U/L extension to 2nd order
biliary radicles and ipsilateral portal
Vein (PV) involvement or ipsilateral hepatic
atrophy

20. Unresectability

21. Treatment of resectable disease
Complete resection with negative margins
5 year overall survival rate
Hilar : 20-24%
Distal : 16-25%

22. Preoperative Biliary Drainage
• Controversy
• benefits vs drawback
• indications :
• cholangitis
• malnutrition
• Hilar CCA: patients with low FLR needing PVE
• Endoscopic vs percutaneous drainage

23. Principles of surgery
• Complete resection with negative margin
• Regional lymphadenectomy (porta hepatis)
Hilar cholangiocarcinoma
• Major hepatic resection
• Assessessment of Future Liver Remnant
Distal cholangiocarcinoma
• Pancreaticoduodenectomy
Mid bile duct tumors
• CBD resection with regional lymphadenectomy

24. • Extirpation of locoregional draining lymphatic basin in
hepatoduodenal ligament, cystic triangle and along hepatic artery
• LN ≥7 is adequate for prognostic staging
• Frozen section assessment of proximal and distal bile duct margins

25. • CCA microscopically infiltrate the surrounding duct beyond the extent
of a macroscopic tumor.
• Recommended resection margin
• 1cm for invasive cancer
• 2cm for non-invasive cancer
• Difficult to achieve satisfactory margin
proximally- so farthest proximal point
technically possible and frozen section

26. Future liver remnant

27. Methods to increase FLR
• Portal vein embolization
• Portal vein ligation
• Associating liver partition and portal vein ligation for staged
hepatectomy (ALPPS)

28. Preoperative portal vein embolization
• Decrease possibility of postoperative liver insufficiency/ failure
• Increase FLR mass prior to resection and minimize risk of metabolic insufficiency
• Induce slight atrophy of the hemiliver embolized with a compensatory
hypertrophy of contralateral side
• Threshold
• 20% to 30% in a healthy liver
• 30% to 40% in liver with preexisting cirrhosis/steatosis/steatohepatitis.
• Advantages
• helps to investigate capacity of liver to regenerate
• minimizes abrupt increase of portal pressure after resection -- separates this
physiologic stress in time from stress and trauma of surgical hepatectomy

29. Staging laparoscopy
• Peritoneal or liver metastasis – 20 to 40% cases at surgical exploration
• To avoid morbidity of unnecessary laparotomy

30. Hilar CCA
Types I and II:
• CBD resection, cholecystectomy,
• 5- to 10-mm margin of resection
• Resection of the bile duct and nodal tissue requires skeletonization of
hepatic artery and portal vein
• Indications of partial hepatectomy
• Unilateral second-order biliary radicle involvement
• Ipsilateral portal vein involvement is detected

31. • Type III and IV
• Complex hepatic resections
• Trisectionectomy

32. Terminologies

33. Right hepatectomy Left hepatectomy
Right trisectionectomy Left lateral sectionectomy
Left trisectionectomy

34. Reconstruction
• Resection and reconstruction of portal vein and/ or hepatic artery
may be necessary for complete resection
• Biliary reconstruction
-Roux-en-Y hepaticojejunostomy

35. Post resection status
R0 resection ; -ve regional nodes R1 resection ; +ve regional nodes R2 resection
Observe Systemic therapy As unresectable disease
Systemic therapy Fluoropyrimidine based
chemoradiation
Systemic therapy
EBRT with concurrent
fluoropyrimidine
Palliative EBRT
Best supportive care
Fluoropyrimidine chemoradiation Fluoropyrimidine based or
gemcitabine based chemotherapy
f/b Fluoropyrimydine based
chemoradiation
Fluoropyrimydine based
chemoradiation f/b
Fluoropyrimidine based or
gemcitabine cased chemotherapy

36. Principles of systemic therapy
Primary treatment for unresectable and metastatic disease
• Preferred regimen – Gemcitabine + cisplatin
Subsequent line therapy for cancers if progression
• Preferred regimen – FOLFOX
• Other recommendation – FOLFIRI; Regorafenib

37. Palliative procedure
• Palliation of symptomatic jaundice
• Intraop findings of unresectability or when distal CBD margin +ve
• biliary-enteric bypass
• Preoperative : PTBD or ERCP
• Palliation of pain :
• Narcotics
• Palliation of duodenal obstruction :
• Duodenal stenting

38. Photodynamic therapy
• For palliation of unresectable hilar CC
• Photosensitising agent porphyrin or d-aminolevulinic acid given iv--
accumulates in cancer cells
• PDT delivered intraluminally to tumor by cholangioscopy
• Generation of oxygen free radical , tumor cell death
• Improve biliary drainage and improvement in cholestasis

39. Surveillance for Ro and R1 resection
• Consider imaging every 3 to 6 months for 2 years
• Every 6-12 months for upto 5 years or as clinically indicated

40. Liver transplantation
• Hilar CCA
• Locally advanced unresectable disease
• Less than 3 cm in radial diameter
• No intra and extra hepatic metastasis and negative nodal status
• Who have no progression after a year or more of combined neoadjuvant
therapy
• PSC-associated hilar CCA.

41. Intrahepatic cholangiocarcinoma
• Can mimic hepato-cellular carcinoma or metastatic carcinoma

42. Intrahepatic cholangiocarcinoma

44. Contraindication to resection
• Lymph node involvement beyond porta hepatis
• Multifocal liver disease

45. Any queries?

46. References
• NCCN Guidelines Version 5.2021 Hepatobiliary Cancers
• Fischer’s Mastery of Surgery 7th edition
• Sabiston Textbook of Surgery First South East Asian Edition
• Shackelford's Surgery of the Alimentary Tract,8th edition
• Torres OJ, Fernandes ES, Herman P. ALPPS: past, present and future.

47. THANK YOU…!