Editing Cholangiocarcinoma (section)

== '''Intrahepatic''' ==

=== Pathophysiology ===

** Difficult to distinguish from adenocarcinoma
** Biopsies especially would normally come back as adenocarcinoma rather than cholangiocarcinoma

=== Presentation ===

** Similar to HCC
** Generally found incidentally
** Most common symptoms are RUQ pain and weight loss
** Jaundice only if advanced or if biliary tree is occluded near hilum of the liver - but uncommon because these tumours tend to occur in peripheral liver
** Can have uni-lobar obstruction of a bile duct, leading to atrophy and contralateral hypertrophy

=== Imaging ===

** Dedicated liver MRI is best
** Low attenuating, minor peripheral enhancement, upstream biliary dilatation, capsular retraction
** Generally has persistent enhancement on delayed phases due to the fibrotic nature of cholangiocarcinoma, in contrast with the vascular nature of HCC

=== Workup ===

** Consider workup for primary, if one of the differentials of the lesion is a metastasis
*** Generally need to do scopes and CT C/A/P
** Bloods
*** Hepatitis serology
*** AFP - normal - helps with excluding HCC
*** CEA - can be high
*** CA 19-9 - can be high
*** Chromogranin A
** Liver evaluation
*** Consider biopsy/liver remnant volumes - should be >50% if patient has cirrhosis

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

=== Resectability ===

** Main determinant is adequate FLR
** Unresectable disease
*** Advanced tumour involving either inflow or outflow bilaterally
*** Multiple bilateral intrahepatic tumours
*** Distant metastatic disease
*** Relative contraindications: small localised satellite lesions, perihepatic lymphadenopathy, portal hypertension

=== Treatment ===

** Surgical
*** Only curative option is R0 resection (long-term survival in unresected patients is rare)
**** Transplant is not accepted.
*** Hilar nodes should be formally resected in intrahepatic cholangiocarcinoma - 30% positive
*** Mostly open procedures, however do laparoscopy first to assess for carcinomatosis (simple or left resections might be performed lap)
** Locoregional therapy
*** TACE/TARE, ablation, external beam radiotherapy, photodynamic therapy
*** Couldn't really recommend one over another