Cholangiocarcinoma

Bile duct adenocarcinoma

Cholangiocarcinoma
Synonyms	Bile duct cancer, cancer of the bile duct
Pronounce	koh-LAN-jee-oh-KAR-sih-NOH-muh
Field	Oncology
Symptoms	Abdominal pain, yellowish skin, weight loss, generalized itching, fever
Complications
Onset	70 years old
Duration
Types	Intrahepatic, perihilar, distal
Causes
Risks	Primary sclerosing cholangitis, ulcerative colitis, infection with certain liver flukes, some congenital liver malformations
Diagnosis	Confirmed by examination of the tumor under a microscope
Differential diagnosis
Prevention
Treatment	Surgical resection, chemotherapy, radiation therapy, stenting procedures, liver transplantation
Medication
Prognosis	Generally poor
Frequency	1–2 people per 100,000 per year (Western world)
Deaths

Cholangiocarcinoma, colloquially known as bile duct cancer, is a rare but serious form of cancer that arises in the bile ducts. These ducts are a series of intricate channels responsible for transporting bile – a fluid aiding in the digestion and absorption of fats – from the liver to the gallbladder and intestines.

Anatomy: Bile Ducts

The bile duct system consists of a series of small tubes that transport bile from the liver to the gallbladder and then into the small intestine. This complex network comprises intrahepatic bile ducts (within the liver), extrahepatic bile ducts (outside the liver), and the common bile duct.

Types of Cholangiocarcinoma

Cholangiocarcinomas are classified based on their location in the bile duct system:

• Intrahepatic Cholangiocarcinoma: These originate in the small bile duct branches inside the liver.
• Perihilar (or Klatskin tumor) Cholangiocarcinoma: These form at the hilum, where the left and right hepatic ducts have joined and are leaving the liver.
• Distal Cholangiocarcinoma: These occur further down the bile duct, closer to the small intestine.

Risk Factors

Several conditions are linked to an increased risk of developing cholangiocarcinoma:

• Primary sclerosing cholangitis: An inflammatory disease of the bile ducts that can lead to scarring and narrowing.
• Ulcerative colitis: A chronic condition characterized by inflammation of the large intestine (colon and rectum).
• Cirrhosis: Scarring of the liver due to various causes such as alcoholism or chronic hepatitis.
• Hepatitis B and Hepatitis C: Viral infections that can cause chronic liver disease and cirrhosis.
• Parasitic infection: Particularly infection with liver flukes which are common in certain parts of Asia.
• Congenital liver malformations: Such as Caroli's syndrome and choledochal cysts.

Signs and Symptoms

The clinical presentation of cholangiocarcinoma can be quite variable, and symptoms typically do not appear until the disease has progressed significantly. Common symptoms may include:

• Jaundice (yellowing of the skin and eyes)
• Abdominal pain, particularly in the upper right quadrant
• Unexplained weight loss
• Loss of appetite
• Fever
• Itching
• Dark urine and pale stools

Investigations

Diagnosis of cholangiocarcinoma often involves a combination of blood tests, imaging studies, and, in some cases, biopsy. Liver function tests can detect signs of liver damage or disease, while tumor markers can help identify potential cancer. Imaging studies such as ultrasound, CT, MRI, and ERCP (endoscopic retrograde cholangiopancreatography) can help visualize the bile ducts and detect any abnormalities. A biopsy may be required to confirm the diagnosis.

Staging

Staging is an important process that determines the extent of cancer spread. This information can help guide treatment decisions and prognosis. The TNM (Tumor, Nodes, Metastasis) system is commonly used in the staging of cholangiocarcinoma.

Prognosis and Treatment

The prognosis of cholangiocarcinoma is generally poor due to late diagnosis. Treatment depends on the stage and location of the cancer and can include surgery (if the tumor is localized and the patient is fit for surgery), chemotherapy, radiation therapy, and palliative care.

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