Makindo Medical Notes

Cholangiocarcinoma is often diagnosed in advanced stages, which limits the available treatment options.

About Cholangiocarcinoma

• Cholangiocarcinoma is a malignant tumour that originates from the biliary epithelial cells.
• The incidence and mortality rates for this cancer are rising globally.
• It has a prevalence of 0.5-1.2 cases per 100,000 people and is more common in men than women.

Aetiology

• About 95% of cholangiocarcinomas are adenocarcinomas, originating from the epithelial lining of the bile ducts.
• There are two main types: nodular infiltrating and diffusely infiltrating types.

Risk Factors

• Obesity
• Hepatolithiasis
• Bile stasis-associated cholangitis
• Hepatitis B and C (higher risk with Hepatitis C than B)
• HIV
• Parasitic infections (endemic in Southeast Asia, associated with a 14-27-fold increase in risk):
  • Opisthorchis viverrini
  • Clonorchis sinensis
  • Schistosoma japonicum
• Diabetes mellitus
• Smoking
• Advanced age (65% of cases are in individuals older than 65 years)
• Post-biliary surgery
• Biliary-enteric anastomosis
• Chronic inflammatory diseases
• Chronic typhoid carriers (6x increased risk)
• Cryptosporidiosis infection
• Hepatic cirrhosis
• Congenital causes (choledochal cysts, Caroli's disease, congenital hepatic fibrosis)
• Chemical exposure (thorotrast, dioxin, nitrosamines, asbestos)
• Medications (e.g., oral contraceptives, isoniazid)
• Primary Sclerosing Cholangitis (increases risk 1,500-fold, associated with advanced age)
• Ulcerative colitis

Types of Cholangiocarcinoma

• Intrahepatic Cholangiocarcinoma:
  • Mass-forming type
  • Periductal-infiltrative type
  • Intraductal type
• Extrahepatic Cholangiocarcinoma:
  • Perihilar (involving the bifurcation of the main bile ducts)
  • Distal (occurring further along the bile duct)

Clinical Features

• Obstructive jaundice, often painless
• Some patients may experience abdominal pain and fatigue
• Dark urine, pale stools, and weight loss are common

Investigations

• Blood tests: FBC, U&E, and liver function tests, showing elevated alkaline phosphatase (ALP)
• Tumor markers: Elevated CA 19-9, carcinoembryonic antigen (CEA), and CA-125
• Imaging: Abdominal CT or MRI to evaluate the extent of the disease
• MR angiography and cholangiography for detailed assessment of the bile ducts and vasculature

Management

• The overall 5-year survival rate for cholangiocarcinoma is about 5-10%, but the chance of survival improves to 25-30% following potentially curative surgery.
• Surgery is the primary treatment option for early-stage patients.
• Chemotherapy may improve overall survival for patients after surgical resection, particularly in intrahepatic cholangiocarcinoma.
• Endoscopic Retrograde Cholangiopancreatography (ERCP) may be used to place a stent in a blocked bile duct to relieve jaundice.
• Liver transplantation may be an option for a small subset of patients.

References

• Cholangiocarcinoma Review