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Hilar Cholangiocarcinoma
in My Point of View
Facebook: Happy Friday Knight
June, 15th, 2018
General Surgical Residency Program
Thailand
• Essential Definition
• Pathogenesis
• Clinical presentation
• Diagnostic Evaluation
• Treatment
• Conclusion
Essential Definition
Nataliya Razumilava and Gregory J. Gores. Classification, Diagnosis, and Management
of Cholangiocarcinoma. Clin Gastroenterol Hepatol. 2013 Jan; 11(1): 13–e4.
• Intrahepatic CCA = tumor proximal to second-
order bile duct
• Extrahepatic CCA = tumor distal to second-
order bile duct = pCCA + dCCA
• Klatskin tumor = hilar CCA
• Hilar CCA = tumor arising at confluence of bile
duct
• Perihilar CCA = tumor arising in hilar area +
iCCA with hilar involvement
Perihilar CCA
• Clinically = tumor arising in hilar area + iCCA
with hilar involvement
• Similar treatment and prognosis
Pathogenesis
Risk Factors
• Periductal fibrosis
• Nitrosamine compound
• Primary sclerosing cholangitis
• Clonorchis sinensis
• Opisthorcis viverini
• HCV
• Cirrhosis
• Choledochal cyst
• Chronic hepatolithiasis
• Inflammatory bowel disease
Clinical Presentation
Clinical Presentation
• Jaundice
• abdominal pain
• weight loss
• Fatigue
• Pruritus
• Nausea
• dark urine
• clay colored stools
Diagnostic Evaluation
Investigation
• Imaging
– Ultrasound
– CT
– MRI
• Tumor marker
Ultrasound
• Only diffuse bile duct dilatation without
demonstrate cause of obstruction
• Maybe abrupt cutoff and nonunion of the
right and left dilated ducts at the porta
hepatis
• CCA Screening and Care Program (CASCAP)
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017
CT and MRI
• Diffuse bile duct dilatation with filling defect
at hilar area
• CT: good for assessment of vascular
involvement
• MRI is good for intraductal
cholangiocarcinoma
– Increased T2 signal
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017
Tumor Marker
• CA 19-9
• CEA
• 89% sensitivity and 86% specificity when
combined with other diagnostic modalities
Treatment
Treatment
• Staging
– Bismuth-Corlette
– AJCC
– MSKCC: Jarnagin and Blumgart T staging
• Preoperative evaluation
– Patient factor: preoperative biliary drainage
– disease factor
– Liver factor: PVE
• Principles
– Resection
– Regional lymphadenectomy
• Role of adjuvant chemotherapy
• Palliative treatment
– Biliary drainage
– chemotherapy
Staging
• Bismuth-Corlette
• AJCC
• MSKCC: Jarnagin and Blumgart T staging
• Consider longitudinal and vertical spreading
Bismuth-Corlette Classification
Kevin C. Soares1, Ihab Kamel2, David P. Cosgrove3, Joseph M. Herman4, Timothy M.
Pawlik. Hilar cholangiocarcinoma: diagnosis, treatment options, and management.
Hepatobiliary surgery and nutrition. 2014;3(1)
Hilar cholangiocarcinoma in my point of view
Hilar cholangiocarcinoma in my point of view
Hilar cholangiocarcinoma in my point of view
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017
Preoperative Evaluation
• Patient factor
• Disease factor
• Liver factor
Preoperative Evaluation: Patient Factor
• Performance status
• Cardiovascular, respiratory, and renal assess
• Complication: acute cholangitis
Preoperative Biliary Drainage
• Hepatic resection when jaundice increases risk
of postoperative complications including
hepatic failure
• Retrospective analysis showed TB > 3 mg/dL
decreases overall survival
• But routine PBD is debated. It can cause
cholangitis and no difference in morbidity and
mortality between who did and did not.
• Purposes of preoperative biliary drainage:
– Treatment of biliary sepsis
– relief of jaundice and recovery of hepatic
functional capacity
– diagnosis of lateral tumor extension
– improvement in poor nutritional intake
Preoperative Biliary Drainage
• Endoscopic biliary stent (EBS) VS
percutaneous transhepatic biliary drainage
(PTBD)
• Endoscopic nasobiliary drainage (ENBD)
Preoperative Biliary Drainage:
Which route?
ERCP
http://www.khuranagastrocare.com/biliarystent.html
EBS
http://www.khuranagastrocare.com/bi
liarystent.html
https://www.youtube.com/watch?v=vknV98Z9Aqk
ENBD
https://link.springer.com/article/10.1007
/s00534-012-0570-2
• PTBD > EBS: EBS increases risk of cholangitis
due to stent occlusion
• PTBD and ENBD can be irrigated
• ENBD is choice due to seeding when PTBD
Preoperative Biliary Drainage:
Which route?
• Future remnant lobe
• Selective > total drainage
Preoperative Biliary Drainage:
Which lobe?
Disease Factor: Criteria for
Nonresectability
• Patient factors
– Medically unfit
– Cirrhosis with portal hypertension
• Local tumor extent
– Bilateral hepatic duct involvement up to
secondary biliary radicals
– Encasement/occlusion of the main portal vein
– Encasement of portal vein branch with atrophy of
contralateral hepatic lobe
– Hepatic duct involvement up to secondary biliary
radicles with atrophy of contralateral hepatic lobe
Disease Factor: Criteria for
Nonresectability
• Distant tumor spread
– Positive lymph nodes outside the hepatoduodenal
ligament
– Metastases to liver, lung, peritoneum, or other
distant organs
Disease Factor: Criteria for
Nonresectability
Hilar cholangiocarcinoma in my point of view
Liver Factor
• Adequate future liver remnant (FLR)
• Patient with FLR <30% should undergo portal
vein embolization to induce hypertrophy of
FLR (nonembolized lobe)
• The FLR typically increases in size from 8% to
46% over 2 to 8 weeks after PVE with mean
37.9%
Principles of treatment
• Complete resection with adequate margin
• Regional lymphadenectomy
• Re-establishment of biliary-enteric drainage
Resection
• 40% to 50% of patients who undergo
exploration with curative intent are found to
have tumors that are unresectable at
laparotomy: staging laparoscopy may be used
to prevent nontherapeutic laparotomy
• Negative margin can be achieved by frozen
section
Resection
• For patients with small, Bismuth-Corlette type I
tumors, resection of the extrahepatic biliary tree
alone without concomitant liver resection may be
an option
• However, patients who have bile duct resection
alone without liver resection do have increased
rates of local recurrence and decreased survival,
and it is has been suggested that patients with
even Bismuth-Corlette type I tumors should
undergo concomitant hepatic resection
Bismuth-Corlette Classification
Kevin C. Soares1, Ihab Kamel2, David P. Cosgrove3, Joseph M. Herman4, Timothy M.
Pawlik. Hilar cholangiocarcinoma: diagnosis, treatment options, and management.
Hepatobiliary surgery and nutrition. 2014;3(1)
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Techniques
• Rt hepatectomy = resection segment 5, 6, 7, 8
• Extended Rt hepatectomy = resection
segment 5, 6, 7, 8 + segment 4 that proximal
duct is resected Rt side of umbilical portion of
Lt portal vein
• Rt trisectionectomy = Rt hepatectomy +
segment 4 Lt side
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Jarnagin et al.
Blumgart’s Surgery
of the Liver, Biliary
Tract, and
Pancreas. 6th ed.
Philadelphia:
Elsevier, 2017.
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Jarnagin et al. Blumgart’s Surgery of the
Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Caudate Lobe Resection
• Some institutes in Japan recommend to do
routine caudate lobe resection
• Since caudate lobe bile duct opens to Lt and
Rt hepatic duct at hilar area  40-98%
caudate involvement
Combined Vascular Resection
and Reconstruction
• Portal vein resection and reconstruction can
improve survival
• Hepatic artery resection and reconstruction is
in trial
Regional Lymphadenectomy
• 30-50% lymph node involvement
• Lymph node involvement further than
hepatoduodenal ligament related with very low
5-year survival: 0 – 6%
• Recommendation is to dissect LN in
hepatoduodenal ligament, retropancreatic, and
common hepatic artery
• If there is lymphadenopathy beyond
hepatoduodenal ligament  LN frozen section if
positive contraindication to curative resection
Hilar cholangiocarcinoma in my point of view
Role of Adjuvant Chemotherapy
• Not well established
NCCN. Hepatobiliary cancer
Palliative Treatment
• Biliary decompression in pruritus and
cholangitis
– Endoscopic stenting
– Transhepatic stenting
– Segment III biliary-enteric bypass when found
unresectable during laparotomy
• Chemoradiation with capecitabine or 5-FU
Conclusion
• Hilar CCA is rare disease and poor survival
• Only curative treatment is R0 surgery
• Bismuth IV is not contraindication for surgery:
PVE with trisectionectomy
• Preoperative biliary drainage is mainly
indicated in cholangitis
References
Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed.
Philadelphia: Elsevier, 2017.
Clifford S.Cho, editor. Surgical clinic of north America: Technical Aspects of
oncologic hepatic surgery. 2016;96(2):163-267.
Nataliya Razumilava and Gregory J. Gores. Classification, Diagnosis, and
Management of Cholangiocarcinoma. Clin Gastroenterol Hepatol. 2013;11(1): 13–
e4.
Kevin C. Soares1, Ihab Kamel2, David P. Cosgrove3, Joseph M. Herman4, Timothy M.
Pawlik. Hilar cholangiocarcinoma: diagnosis, treatment options, and
management. Hepatobiliary surgery and nutrition. 2014;3(1)
NCCN. Hepatobiliary cancer
References
ณรงค์ ขันตีแก้ว และคณะ. HBP surgery 2014 update and multidisciplinary approach in
hbp surgery vol2. กรุงเทพฯ: โฆสิตการพิมพ์, 2557.
ยงยุทธ ศิริวัฒนอักษร และคณะ. HPB surgery 2015: THPBS journey I: Journeys towards
surgery vol3. กรุงเทพฯ: เพนตากอน แอ็ดเวอร์ไทซิง, 2558.
ยงยุทธ ศิริวัฒนอักษร และคณะ. HPB surgery 2017 beyond frontier vol5. กรุงเทพฯ: เพนตากอน
แอ็ดเวอร์ไทซิง, 2560.
ปิยาภรณ์ อภิสารธนรักษ์. การแปลผลภาพเอกซเรย์คอมพิวเตอร์ของตับ. ฉะเชิงเทรา: ศิริวัฒนาซีเคียวริตี้พริ้นท์,
2557.

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Hilar cholangiocarcinoma in my point of view

  • 1. Hilar Cholangiocarcinoma in My Point of View Facebook: Happy Friday Knight June, 15th, 2018 General Surgical Residency Program Thailand
  • 2. • Essential Definition • Pathogenesis • Clinical presentation • Diagnostic Evaluation • Treatment • Conclusion
  • 4. Nataliya Razumilava and Gregory J. Gores. Classification, Diagnosis, and Management of Cholangiocarcinoma. Clin Gastroenterol Hepatol. 2013 Jan; 11(1): 13–e4.
  • 5. • Intrahepatic CCA = tumor proximal to second- order bile duct • Extrahepatic CCA = tumor distal to second- order bile duct = pCCA + dCCA • Klatskin tumor = hilar CCA • Hilar CCA = tumor arising at confluence of bile duct • Perihilar CCA = tumor arising in hilar area + iCCA with hilar involvement
  • 6. Perihilar CCA • Clinically = tumor arising in hilar area + iCCA with hilar involvement • Similar treatment and prognosis
  • 8. Risk Factors • Periductal fibrosis • Nitrosamine compound • Primary sclerosing cholangitis • Clonorchis sinensis • Opisthorcis viverini • HCV • Cirrhosis • Choledochal cyst • Chronic hepatolithiasis • Inflammatory bowel disease
  • 10. Clinical Presentation • Jaundice • abdominal pain • weight loss • Fatigue • Pruritus • Nausea • dark urine • clay colored stools
  • 12. Investigation • Imaging – Ultrasound – CT – MRI • Tumor marker
  • 13. Ultrasound • Only diffuse bile duct dilatation without demonstrate cause of obstruction • Maybe abrupt cutoff and nonunion of the right and left dilated ducts at the porta hepatis • CCA Screening and Care Program (CASCAP)
  • 14. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017
  • 15. CT and MRI • Diffuse bile duct dilatation with filling defect at hilar area • CT: good for assessment of vascular involvement • MRI is good for intraductal cholangiocarcinoma – Increased T2 signal
  • 16. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017
  • 17. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017
  • 18. Tumor Marker • CA 19-9 • CEA • 89% sensitivity and 86% specificity when combined with other diagnostic modalities
  • 20. Treatment • Staging – Bismuth-Corlette – AJCC – MSKCC: Jarnagin and Blumgart T staging • Preoperative evaluation – Patient factor: preoperative biliary drainage – disease factor – Liver factor: PVE • Principles – Resection – Regional lymphadenectomy • Role of adjuvant chemotherapy • Palliative treatment – Biliary drainage – chemotherapy
  • 21. Staging • Bismuth-Corlette • AJCC • MSKCC: Jarnagin and Blumgart T staging • Consider longitudinal and vertical spreading
  • 22. Bismuth-Corlette Classification Kevin C. Soares1, Ihab Kamel2, David P. Cosgrove3, Joseph M. Herman4, Timothy M. Pawlik. Hilar cholangiocarcinoma: diagnosis, treatment options, and management. Hepatobiliary surgery and nutrition. 2014;3(1)
  • 26. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017
  • 27. Preoperative Evaluation • Patient factor • Disease factor • Liver factor
  • 28. Preoperative Evaluation: Patient Factor • Performance status • Cardiovascular, respiratory, and renal assess • Complication: acute cholangitis
  • 29. Preoperative Biliary Drainage • Hepatic resection when jaundice increases risk of postoperative complications including hepatic failure • Retrospective analysis showed TB > 3 mg/dL decreases overall survival • But routine PBD is debated. It can cause cholangitis and no difference in morbidity and mortality between who did and did not.
  • 30. • Purposes of preoperative biliary drainage: – Treatment of biliary sepsis – relief of jaundice and recovery of hepatic functional capacity – diagnosis of lateral tumor extension – improvement in poor nutritional intake Preoperative Biliary Drainage
  • 31. • Endoscopic biliary stent (EBS) VS percutaneous transhepatic biliary drainage (PTBD) • Endoscopic nasobiliary drainage (ENBD) Preoperative Biliary Drainage: Which route?
  • 36. • PTBD > EBS: EBS increases risk of cholangitis due to stent occlusion • PTBD and ENBD can be irrigated • ENBD is choice due to seeding when PTBD Preoperative Biliary Drainage: Which route?
  • 37. • Future remnant lobe • Selective > total drainage Preoperative Biliary Drainage: Which lobe?
  • 38. Disease Factor: Criteria for Nonresectability • Patient factors – Medically unfit – Cirrhosis with portal hypertension
  • 39. • Local tumor extent – Bilateral hepatic duct involvement up to secondary biliary radicals – Encasement/occlusion of the main portal vein – Encasement of portal vein branch with atrophy of contralateral hepatic lobe – Hepatic duct involvement up to secondary biliary radicles with atrophy of contralateral hepatic lobe Disease Factor: Criteria for Nonresectability
  • 40. • Distant tumor spread – Positive lymph nodes outside the hepatoduodenal ligament – Metastases to liver, lung, peritoneum, or other distant organs Disease Factor: Criteria for Nonresectability
  • 42. Liver Factor • Adequate future liver remnant (FLR) • Patient with FLR <30% should undergo portal vein embolization to induce hypertrophy of FLR (nonembolized lobe) • The FLR typically increases in size from 8% to 46% over 2 to 8 weeks after PVE with mean 37.9%
  • 43. Principles of treatment • Complete resection with adequate margin • Regional lymphadenectomy • Re-establishment of biliary-enteric drainage
  • 44. Resection • 40% to 50% of patients who undergo exploration with curative intent are found to have tumors that are unresectable at laparotomy: staging laparoscopy may be used to prevent nontherapeutic laparotomy • Negative margin can be achieved by frozen section
  • 45. Resection • For patients with small, Bismuth-Corlette type I tumors, resection of the extrahepatic biliary tree alone without concomitant liver resection may be an option • However, patients who have bile duct resection alone without liver resection do have increased rates of local recurrence and decreased survival, and it is has been suggested that patients with even Bismuth-Corlette type I tumors should undergo concomitant hepatic resection
  • 46. Bismuth-Corlette Classification Kevin C. Soares1, Ihab Kamel2, David P. Cosgrove3, Joseph M. Herman4, Timothy M. Pawlik. Hilar cholangiocarcinoma: diagnosis, treatment options, and management. Hepatobiliary surgery and nutrition. 2014;3(1)
  • 47. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 48. Techniques • Rt hepatectomy = resection segment 5, 6, 7, 8 • Extended Rt hepatectomy = resection segment 5, 6, 7, 8 + segment 4 that proximal duct is resected Rt side of umbilical portion of Lt portal vein • Rt trisectionectomy = Rt hepatectomy + segment 4 Lt side
  • 49. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 50. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 51. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 52. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 53. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 54. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 55. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 56. Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017.
  • 57. Caudate Lobe Resection • Some institutes in Japan recommend to do routine caudate lobe resection • Since caudate lobe bile duct opens to Lt and Rt hepatic duct at hilar area  40-98% caudate involvement
  • 58. Combined Vascular Resection and Reconstruction • Portal vein resection and reconstruction can improve survival • Hepatic artery resection and reconstruction is in trial
  • 59. Regional Lymphadenectomy • 30-50% lymph node involvement • Lymph node involvement further than hepatoduodenal ligament related with very low 5-year survival: 0 – 6% • Recommendation is to dissect LN in hepatoduodenal ligament, retropancreatic, and common hepatic artery • If there is lymphadenopathy beyond hepatoduodenal ligament  LN frozen section if positive contraindication to curative resection
  • 61. Role of Adjuvant Chemotherapy • Not well established NCCN. Hepatobiliary cancer
  • 62. Palliative Treatment • Biliary decompression in pruritus and cholangitis – Endoscopic stenting – Transhepatic stenting – Segment III biliary-enteric bypass when found unresectable during laparotomy • Chemoradiation with capecitabine or 5-FU
  • 63. Conclusion • Hilar CCA is rare disease and poor survival • Only curative treatment is R0 surgery • Bismuth IV is not contraindication for surgery: PVE with trisectionectomy • Preoperative biliary drainage is mainly indicated in cholangitis
  • 64. References Jarnagin et al. Blumgart’s Surgery of the Liver, Biliary Tract, and Pancreas. 6th ed. Philadelphia: Elsevier, 2017. Clifford S.Cho, editor. Surgical clinic of north America: Technical Aspects of oncologic hepatic surgery. 2016;96(2):163-267. Nataliya Razumilava and Gregory J. Gores. Classification, Diagnosis, and Management of Cholangiocarcinoma. Clin Gastroenterol Hepatol. 2013;11(1): 13– e4. Kevin C. Soares1, Ihab Kamel2, David P. Cosgrove3, Joseph M. Herman4, Timothy M. Pawlik. Hilar cholangiocarcinoma: diagnosis, treatment options, and management. Hepatobiliary surgery and nutrition. 2014;3(1) NCCN. Hepatobiliary cancer
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