Makindo Medical Notes

🌿 Cholangiocarcinoma is a rare but aggressive adenocarcinoma of the bile ducts. Although uncommon in the UK, it is more prevalent in regions such as France and Southeast Asia (due to liver fluke infections). Late presentation, frequent biliary obstruction, and limited surgical resectability contribute to its poor prognosis.

📖 About

• Represents ~3% of all GI cancers but accounts for ~15% of primary liver cancers.
• Median age at diagnosis: >60 years.
• Prognosis is generally poor due to advanced stage at presentation.

⚠️ Aetiology / Risk Factors

• 🔄 Primary Sclerosing Cholangitis (PSC): Strongest risk factor in Western countries (often linked with ulcerative colitis).
• 🐛 Liver flukes: Opisthorchis viverrini, Clonorchis sinensis (endemic in SE Asia).
• 🪣 Choledochal cysts: Congenital biliary dilatation → chronic stasis + inflammation.
• 🦠 Hepatitis B & C: Chronic viral hepatitis increases cholangiocarcinoma risk.
• 📈 Age: More common >60 years.
• 🌍 Geography: SE Asia (high incidence) vs rare in UK.

🧭 Types of Cholangiocarcinoma

• Intrahepatic: Arises from smaller bile ducts within the liver.
• Perihilar (Klatskin tumour): Most common type; located at the hepatic duct bifurcation.
• Distal extrahepatic: Arises in bile ducts closer to the duodenum/pancreas.

🔬 Pathology

• Histology: Almost always adenocarcinomas.
• 📌 Distribution: ~50% at hepatic duct confluence, ~50% in CBD/cystic duct.
• Tumours spread locally to the liver, porta hepatis, and adjacent vasculature.

🩺 Clinical Features

• 🟡 Painless jaundice: Most common presenting feature (obstructive pattern).
• RUQ abdominal pain, often vague or dull.
• Weight loss, anorexia, malaise.
• Fever with chills → suggests superimposed cholangitis.
• Pruritus due to bile salt deposition in skin.
• Pale stools + dark urine → classic obstructive jaundice features.

🔍 Investigations

• Ultrasound: Biliary dilatation; may show mass.
• CT / MRI + MRCP: Defines anatomy, obstruction, and resectability.
• ERCP / PTC: Diagnostic + therapeutic (stenting); brush cytology for biopsy.
• Tumour markers: CA 19-9, CEA – supportive, not diagnostic.
• LFTs: Obstructive picture (↑ ALP, ↑ GGT, ↑ bilirubin).

🛠️ Management

• Surgery (only curative option):
  • Intrahepatic → partial hepatectomy.
  • Perihilar → bile duct resection ± extended hepatectomy.
  • Distal → pancreaticoduodenectomy (Whipple’s procedure).
• Liver transplant: Considered for selected perihilar tumours with strict criteria (often combined with neoadjuvant therapy).
• Chemotherapy: Gemcitabine + cisplatin is standard first-line in unresectable disease.
• Radiotherapy: Limited role; sometimes adjunct or palliative.
• Palliative care: Endoscopic/percutaneous stenting for biliary drainage, analgesia, nutrition support, and symptom control.

📊 Prognosis

• 5-year survival overall: <10–20%.
• Resectable disease: 25–40% 5-year survival.
• Advanced unresectable cases: Median survival <12 months.

📚 Teaching Commentary

💡 Exam pearl: – Painless jaundice + raised ALP = always think of obstructive pathology → pancreatic cancer vs cholangiocarcinoma. – PSC patients with new jaundice → suspect cholangiocarcinoma. – Liver fluke infection (SE Asia) is the classic global risk factor.

🩺 UK practice: MDT approach, often palliative due to late presentation. Resection is only possible in <30% of patients at diagnosis.