Makindo Medical Notes.com

Disease is often well established at diagnosis limiting treatment options

About

• Cholangiocarcinoma is a malignant neoplasm originating from biliary epithelial cells.
• The incidence and mortality of this cancer are rising in the world.
• It has a prevalence of 0.5-1.2/100.000 people and is Commoner in men than women

Aetiology

• 95% are Adenocarcinoma nature originating from the epithelial lining
• Either infiltrating nodular or diffusely infiltrating type.

Multiple risk factors

• Obesity
• Hepatolithiasis
• Bile stasis-associated cholangitis
• Hepatitis B and C (C>B)
• HIV
• Parasitic infections (endemic in southeastern Asia)x 14-27-fold increase in CC risk) leading to chronic inflammation
  • Opisthorchis viverrini
  • Clonorchis sinensis,
  • Shistosoma Japonica
• Diabetes mellitus
• Smoking
• Advanced age (65% is above 65 years of age),
• Post-biliary surgery
• Biliary-enteric anastomosis
• Chronic inflammatory diseases
• Chronic typhoid carriers (x6 )
• Cryptosporidiosis cases
• Hepatic cirrhosis,
• Congenital causes (choledochal cysts, Caroli's disease, congenital hepatic fibrosis)
• Chemical agents (thorotrast, dioxin, nitrosamines, asbestos)
• Drugs oral contraceptives, isoniazide)
• Primary Sclerosing Cholangitis x 1500 risk associated with advanced age
• Ulcerative colitis

Types

• Intrahepatic CC
  • Mass form
  • Periductal-infiltrative type
  • Intraductal type
• Extrahepatic
  • Perihilar CC (bifurcation of the main duct)
  • Distal CC.

Clinical

• Obstructive jaundice, often painless jaundice
• Some have abdominal pain, fatigue
• Dark urine, pale stools, weight loss

Investigations

• FBC, U&E, LFT are elevated ALP
• Elevated (CA) 19-9, carcinoembryonic antigen, CA-125;
• Abdominal CT/MRI
• MR angiography, and cholangiography are used for evaluation.

Management

• The rate of 5-year survival is about 5-10% including newly-diagnosed cases and 5-year chance of survival following potential surgery is 25-30%
• Surgery is the mainstay of treatment for early stage patients.
• Chemotherapy may have a positive effect on overall survival of patients following resection of intrahepatic cholangiocarcinoma.
• ERCP may be used to insert a stent into a blocked bile duct to relieve jaundice.
• Liver transplant is indicated in a small subset of patients.

References

• Cholangiocarcinoma Review