Cholangiocarcinoma

🧬 Cholangiocarcinoma is a malignant tumour of the bile ducts, often diagnosed late when curative options are limited. ⏳ Prognosis remains poor, but early detection and surgical resection offer the best survival outcomes.

📖 About Cholangiocarcinoma

• Arises from biliary epithelial cells (95% are adenocarcinomas).
• Accounts for ~3% of all gastrointestinal cancers, but incidence is increasing globally.
• Prevalence: 0.5–1.2 cases per 100,000 annually; more common in men (M:F ≈ 1.5:1).
• Peak incidence: 7th decade of life.

🧾 Aetiology

• Two growth patterns:
  • Nodular infiltrating type → mass lesion, often intrahepatic.
  • Diffusely infiltrating type → spreads along bile ducts, harder to detect early.

⚠️ Risk Factors

• 🔑 Primary Sclerosing Cholangitis (PSC) → risk ↑ 1,500-fold, especially with UC.
• 🦠 Parasitic infections (SE Asia): Opisthorchis viverrini, Clonorchis sinensis, Schistosoma japonicum.
• 🪨 Hepatolithiasis, chronic bile stasis, choledochal cysts, Caroli’s disease.
• 🦠 Viral hepatitis (C > B), HIV, cirrhosis.
• 🧪 Chemical exposures: thorotrast, nitrosamines, asbestos, dioxins.
• 🩺 Post-surgical changes: biliary–enteric anastomosis, chronic typhoid carriage.
• Metabolic & lifestyle: diabetes, obesity, smoking, oral contraceptives, isoniazid use.
• Age >65 years (65% of cases present here).

📍 Types

• Intrahepatic (iCCA) → mass-forming, periductal infiltrating, intraductal types.
• Extrahepatic (eCCA):
  • Perihilar (Klatskin tumour) → most common, at bile duct bifurcation.
  • Distal → further along the bile duct, often mimics pancreatic cancer.

🩺 Clinical Features

• Classically: painless obstructive jaundice (dark urine, pale stools, pruritus).
• Weight loss, anorexia, fatigue.
• ± Abdominal pain, hepatomegaly, palpable mass, Courvoisier’s sign (rare).

🔍 Investigations

• Bloods: Cholestatic LFTs (↑ ALP, GGT, bilirubin), ± deranged clotting.
• Tumour markers: CA 19-9 (often raised, not specific), CEA, CA-125.
• Imaging:
  • USS → initial test (biliary dilatation).
  • CT/MRI → staging and extent.
  • MRCP → ductal anatomy, pre-surgical planning.
• Histology: Biopsy or brush cytology at ERCP/EBUS for tissue confirmation.

💊 Management

• 🎯 Surgical resection → only curative option; offers 25–30% 5-year survival if resectable.
• 🧬 Chemotherapy (e.g. gemcitabine + cisplatin) → standard for advanced disease; modest OS benefit.
• 📈 Adjuvant chemotherapy (esp. intrahepatic cases) → may improve outcomes post-surgery.
• 🔧 Palliation: ERCP stent to relieve jaundice and pruritus; PTBD if ERCP not possible.
• 🫀 Liver transplantation → possible in highly selected cases (mainly perihilar disease, strict protocols).
• Supportive: nutrition, pain control, MDT input, discussion of prognosis and palliative care.

🌟 Key Clinical Pearls

🔑 Always suspect cholangiocarcinoma in an older patient with painless jaundice. ⚖️ Differentiate from pancreatic cancer (similar presentation but different surgical approach). 🧩 In PSC patients, new dominant stricture or rising CA19-9 should prompt urgent evaluation.

📚 References

• NORD: Cholangiocarcinoma Review
• BSG & EASL Guidelines on the Management of Cholangiocarcinoma (2021)

Cases — Cholangiocarcinoma (Bile Duct Cancer) 💛

• Case 1 — Perihilar (Klatskin Tumour) 🧬: A 66-year-old man presents with progressive jaundice, dark urine, and pale stools. Exam: palpable non-tender gallbladder (Courvoisier’s sign). LFTs: cholestatic pattern (↑ALP, ↑bilirubin). MRCP: stricture at hepatic duct confluence. Diagnosis: Perihilar cholangiocarcinoma. Management: Biliary stenting for jaundice, surgical resection if operable; palliative chemotherapy if advanced.
• Case 2 — Intrahepatic Cholangiocarcinoma 🩺: A 58-year-old woman with a history of primary sclerosing cholangitis (PSC) develops fatigue, weight loss, and right upper quadrant discomfort. Exam: hepatomegaly. Imaging: intrahepatic mass lesion. CA19-9 elevated. Diagnosis: Intrahepatic cholangiocarcinoma complicating PSC. Management: Surgical resection if localised; consider liver transplant in selected PSC patients; systemic therapy for advanced disease.
• Case 3 — Distal Extrahepatic Cholangiocarcinoma 🚻: A 70-year-old man presents with obstructive jaundice and pruritus. Endoscopic ultrasound shows a distal bile duct stricture near the ampulla. ERCP: brush cytology positive for adenocarcinoma. Diagnosis: Distal extrahepatic cholangiocarcinoma. Management: Whipple procedure (pancreaticoduodenectomy) if fit and resectable; palliative stent if unresectable.

Teaching Commentary 🧠

Cholangiocarcinoma = malignant tumour of bile duct epithelium. - Types: intrahepatic, perihilar (Klatskin), distal extrahepatic. - Risk factors: PSC (esp. with IBD), liver flukes (SE Asia), biliary cysts, chronic biliary inflammation. - Presentation: cholestatic jaundice, pruritus, RUQ pain, weight loss. - Investigations: LFTs (↑ALP, ↑bilirubin), CA19-9, MRCP/ERCP with cytology. - Management: Surgical resection or transplant (only curative option); palliative stenting/chemo otherwise. ⚠️ Prognosis often poor as diagnosis is late.