- Cholangiocarcinoma is a rare tumour of the biliary tree
- Accounts for about 1000 deaths per year in United Kingdom
- Arises from the epithelium of the biliary tract
- 25% are intrahepatic
- Often presents late with irresectable disease
- Cure rates are low and median survival is less than 12 months
- Neoadjuvant and adjuvant therapies have not improved survival
Risk factors
- Age
- Primary sclerosing cholangitis
- Choledocholithiasis
- Biliary papillomatosis
- Choledochal cysts
- Thorotrast
- Liver flukes (Clonorchis sinesis)
Clinical features
- Most patients present with obstructive jaundice
- Pain and fever are uncommon
- Late presentation is associated with fatigue, malaise and weight
loss
- Some are found incidentally when imaging is performed for other
reasons
Diagnosis
- Liver function tests show an obstructive picture
- CA19.9 and CA125 may be raised
- Diagnosis can be confirmed by CT or MRI
- ERCP can be both diagnostic and therapeutic
- Specimens can be obtained for cytology / histology
- A biliary stent can be inserted
Management
- Surgery offers the only chance of cure
- Tumour is resected and biliary reconstruction is performed
- Aim is for resection with tumour free margins
- Determinants of resectability are:
- Extent of tumour
- Vascular invasion
- Hepatic lobar atrophy
- Metastatic disease
- Liver transplantation is controversial due to high recurrence rates
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Khan S A, Thomas H C, Davidson B R et al.
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Lazaridis K N, Gores D J. Cholangiocarcinoma.
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Nagomey D M, Kendrick M L. Hepatic resection in the
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