Cholangiocarcinoma, also known as bile duct cancer, is a rare and aggressive group of tumors that can develop anywhere within the bile duct system.
The bile ducts form a network of tubes connecting the liver and gallbladder to the small intestine. Their primary function is to carry bile — a digestive fluid produced by the liver — into the small bowel to help break down fats in food.
What Are the Parts of the Bile Duct System?
The system includes intrahepatic ducts (inside the liver), perihilar (hilum) region, and extrahepatic ducts (outside the liver), ending at the common bile duct which joins the pancreatic duct before entering the duodenum.
Are There Different Types of Cholangiocarcinoma?
Yes, based on location:
- Intrahepatic: Originates inside the liver.
- Perihilar (Hilar): Develops where the hepatic ducts exit the liver — most common type.
- Distal: Occurs in the lower part of the bile duct, closer to the small intestine.
Who Is More Likely to Get This Type of Cancer?
Risk factors include:
- Primary sclerosing cholangitis (PSC)
- Chronic liver disease (e.g., cirrhosis)
- Bile duct cysts (choledochal cysts)
- Liver fluke infections (common in Southeast Asia)
- Older age (most cases occur after 50)
- Smoking and alcohol use
What Are the Symptoms of Cholangiocarcinoma?
Early stages may be asymptomatic. As the tumor grows and blocks bile flow, symptoms appear:
- Jaundice (yellowing of skin and eyes)
- Itchy skin
- Dark urine and pale stools
- Abdominal pain (especially upper right side)
- Unexplained weight loss
- Nausea and loss of appetite
- Fever and chills (if infection develops)
How Is This Type of Cancer Diagnosed?
Liver Function Test
Elevated bilirubin and alkaline phosphatase suggest bile duct obstruction.
Tumour Marker Tests
CA 19-9 and CEA levels may be elevated, though not specific for diagnosis.
Imaging Tests
- Abdominal Ultrasound: First-line imaging to detect blockages.
- CT Scan: Assesses tumor size, spread, and involvement of nearby structures.
- MRCP (Magnetic Resonance Cholangiopancreatography): Detailed images of bile and pancreatic ducts without radiation.
- ERCP (Endoscopic Retrograde Cholangiopancreatography): Allows both visualization and therapeutic intervention like stent placement.
Biopsy
Tissue sample taken during ERCP, percutaneous procedure, or surgery confirms cancer diagnosis.
What Does Stage Mean?
Staging helps determine prognosis and treatment. While TNM classification exists, doctors often simplify staging for clinical decisions:
- Local: Confined to the bile duct; potentially resectable.
- Locally Advanced: Invades nearby organs, arteries, or veins; may not be removable surgically.
- Metastatic: Spread to distant sites (e.g., lungs, bones).
- Recurrent: Returns after initial treatment.
Cancers are also classified as:
- Resectable (Localized): Can be completely removed with surgery.
- Unresectable: Too widespread for complete surgical removal — includes most stage III and IV cancers.
How Is Cholangiocarcinoma Treated?
Treatment depends on cancer type, stage, and patient’s overall health.
Surgery
Only curative option when cancer is localized and resectable.
Intrahepatic Bile Duct Cancers
Partial liver resection (removal of affected lobe).
Perihilar Bile Duct Cancer
May require removal of part of the liver, bile duct, gallbladder, and sometimes a portion of the pancreas or small intestine.
Distal Bile Duct Cancers
Treated with Whipple procedure (pancreaticoduodenectomy).
Palliative Surgery for Bile Duct Cancers
When cure isn’t possible, procedures relieve symptoms:
Stent Placement
A tube placed via ERCP or percutaneously to bypass blockage and restore bile flow.
Biliary Bypass
Surgical rerouting of bile around the tumor into the small intestine.
Radiation Therapy
Uses high-energy beams to kill cancer cells. Used:
- Before surgery to shrink tumors
- After surgery to destroy residual cells
- As main treatment if surgery isn't an option
- To relieve symptoms in advanced cases
Chemotherapy
Drugs given orally or intravenously to control cancer growth:
- Before surgery to reduce tumor size
- After surgery, often combined with radiation
- For metastatic disease to slow progression
Common drugs: 5-fluorouracil (5-FU), gemcitabine, cisplatin, capecitabine, oxaliplatin — used alone or in combination.
Targeted Therapy
Newer treatments targeting specific genetic mutations found in some cholangiocarcinomas (e.g., FGFR2 fusions, IDH1 mutations). Examples: pemigatinib, ivosidenib.
Can Cholangiocarcinoma Be Cured? What Are the Survival Rates?
Survival depends on stage, treatment response, and overall health. Five-year survival rates reflect the percentage of people living at least five years post-diagnosis.
Intrahepatic Cholangiocarcinoma
- Local: 24%
- Locally Advanced: 7%
- Metastatic: 2%
Extrahepatic Cholangiocarcinoma
- Local: 15%
- Locally Advanced: 16%
- Metastatic: 2%
Early detection and multidisciplinary care improve outcomes. Research into targeted therapies and immunotherapies offers hope for improved survival in the future.