What is Sclerosing Cholangiocarcinoma of the Biliary Tract?
Sclerosing cholangiocarcinoma of the biliary tract is the most common type of bile duct cancer in patients who have primary sclerosing cholangitis. It is a deadly cancer of the bile duct occurring in 2-8% of patients and is the leading cause of death among them.
Most primary sclerosing cholangitis associated with cholangiocarcinoma develops within the first few years after being diagnosed with primary sclerosing cholangitis (chronic inflammation, scarring, and blockage of the bile ducts or tubes).
What is Primary Sclerosing Cholangitis?
Primary sclerosing cholangitis is a chronic liver disease characterised by inflammation and damage to the bile ducts inside and outside the liver over time.
What is the Biliary Tree?
The biliary tree or system consists of the liver, gallbladder, and bile ducts. This system makes, stores and secretes bile juice. Bile juice breaks down fats into fatty acids, thus aids in the digestion of fats.
Disease Causes
There is no well-known cause, but genetic changes in the genes and DNA of certain individuals may play a role, primary sclerosing cholangitis (chronic inflammation, scarring, and obstruction of the bile ducts), and increasing age are also strongly associated risk factors with the development of sclerosing cholangiocarcinoma of the biliary tract.
Sclerosing cholangiocarcinoma of the biliary tract is also associated with chronic inflammation of the gallbladder, chronic inflammation of the biliary tree, gallstones, and bile duct stones.
Signs and Symptoms
The patient may suffer from the following signs and symptoms:
- Repeated pain in the abdomen
- Yellowish discoloration of the skin and the eyes (Jaundice)
- Constant body itching
- Nausea
- Vomiting
- Fever
- Pain in the upper right corner of the abdomen
- Unexplained weight loss
- Loss of appetite
Diagnosis
The following diagnostic techniques and procedures can be used to make a diagnosis:
- Complete blood count (CBC)
- Blood coagulation profile
- BUN ( blood urea and nitrogen)
- Serum creatinine
- Liver function tests (LFT’s)
- Amylase and lipase values
- Imaging techniques like CT-scan, MRI, PET-scan, X-Ray, and USG to detect the site and size of the tumour.
- Cholangioscopy (USG of the gallbladder and biliary tree)
- Cholangiography
- MRCP (Magnetic resonance cholangiopancreatography)
- Serum bilirubin total, serum bilirubin conjugated and unconjugated
- Preoperative biopsy
Treatment
Surgical removal of the tumour is the most common and effective treatment option. In moderately advanced cases, the standard surgery is surgical removal of the bile duct with or without pancreas resection (surgical removal of the pancreas).Chemotherapy and radiation therapy are the treatment options in the advanced metastatic cases of sclerosing cholangiocarcinoma of the biliary tract.
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