CHOLANGIOCARCINOMA
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Diagnosis and management of various types of cholangiocarcinoma
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CHOLANGIOCARCINOMA
- 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
- 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
- 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…!
Editor's Notes
- Diabetes, obesity, alcohol, tobacco
- predicts resectability, likelihood of metastatic disease, and survival
- Diffusely infiltrating vs superficially spreading type
- Fisher’s Mastery of surgery vvv Severe portal hypertension precluding surgery is the only absolute contraindication to PVE. Also, in cases where tumor obstructs the portal system in the liver to be resected, PVE is not necessary as portal flow is already redirected to the FLR.70,71 Relative contraindications include uncorrectable coagulopathy, renal failure, and extrahepatic metastasis. Two-stage hepatectomy has expanded the patients with bilobar hepatic disease burden eligible for PVE and potential curative resection as will be further detailed in the following discussion; however, diffuse hepatic disease burden remains a contraindication to PVE.
- A right-sided hepatectomy is generally recommended for perihilar cholangiocarcinomas because the procedure satisfies the nontouch (en bloc) resection and wide, tumor-free margins, which leads to a favorable local control and prognosis