Editing Cholangiocarcinoma (section)

== '''Hilar''' ==

=== Presentation ===

** Obstructive painless jaundice
** Constitutional symptoms
** Isolated intrahepatic biliary dilatation

=== Imaging ===

** MRI/CT will show it
** Look for portal lymphadenopathy, metastatic disease, liver atrophy, and vascular involvement (right arterial invasion more common)

=== Workup ===

** ERCP/PTC to drain biliary system and attempt to get tissue

=== Staging ===

** Bismuth-Corlette - may be considered old, doesn't guide resectability, but best starting point to describe the tumour anatomically. Higher stage tumours are less likely to be resectable.
** TNM - pathologic, doesn't guide operative planning, but useful for prognostication
** Other staging systems which have been proposed, but not universally used:
*** Blumgart - predicts resectability, and correlates with overall survival
*** International cholangiocarcinoma group staging system - combines multiple other staging systems, but still under evaluation

=== '''Bismuth-Corlette: - to follow''' ===

=== '''Perihilar bile duct cancer TNM staging AJCC UICC 8th edition - to follow''' ===

=== '''Resectability''' ===

** '''Unresectable factors''' (based on Blumgart staging system):
*** Patient factors
**** Medically unfit for major operation
**** Cirrhosis
*** Local tumour-related factors (Blumgart T2 or T3)
**** Hepatic duct involvement up to secondary biliary radicals bilaterally (that is, proximal to both right and left CHD)
**** Encasement or occlusion of the main portal vein proximal to its bifurcation
**** Atrophy of one hepatic lobe with contralateral encasement of portal vein branch
**** Atrophy of one hepatic lobe with contralateral involvement of secondary biliary radicals
**** Unilateral tumour extension to secondary biliary radicles with contralateral vein branch encasement or occlusion
*** Metastatic disease
**** Histologically proven metastases to distant lymph node basins
**** Lung, liver or peritoneal mets

=== Treatment ===

** Surgical resection
*** Initial laparoscopy to determine resectability
*** Commonly right subcostal incision
*** Bismuth-Corlette may assist with best operation choice
**** I and II: CBD resection, cholecystectomy, and 5-10mm margin of resection. Type II lesions may also require partial hepatic resection, which commonly includes caudate lobe. Reconstruction using a Roux limb of jejunum.
**** III and IV: may involve complex resection and reconstruction of the PV, hepatic artery, or both.
*** Controversial role of routine lymph node dissection. No demonstrable benefit, but may help prognosticate and direct adjuvant therapy.
** Transplant
*** Very select patients
*** Can be granted MELD exception points to make them eligible
*** Specifically for cholangiocarcinoma in setting of severe liver disease and PSC
*** Often means neoadjuvant CTX followed LTx

=== Prognosis: ===

** 5-year survival rates as high as 59% have been reported