Dr Deepak Varma Gastro Surgeon Cochin

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CCC or cholangiocarcinoma is a malignancy that develops in the biliary duct system. It originates in the extrahepatic bile ducts and liver and at the ampulla of Vater. Cholangiocarcinomas develop in the intrahepatic, extrahepatic and distal extrahepatic regions. They grow very slowly in the biliary epithelium, infiltrating the walls of the ducts and spreading across the tissue planes. Hilar cholangiocarcinoma or Klatskin tumor is a cause for obstructive jaundice. This is a slow growing tumor which causes blockade of bile flow leading to jaundice.

Causes And Risks

  • Chronic infections liver flukes
  • IBD or inflammatory bowel disease
  • Exposure to chemicals
  • Congenital diseases of the biliary tree
  • Bile duct adenomas
  • Alpha1-antitrypsin deficiency
  • Biliary papillomatosis
  • Chronic bilary inflammation
  • Primary sclerosing cholangitis
  • Ulcerative colitis

Signs And Symptoms

Jaundice very common manifestation and best noticed under sunlight; risk of cholestasis if tumour is in the bile duct or common hepatic duct

Weight loss

Pruritus generally preceded by jaundice with the initial symptom of itching

Clay-coloured stools


Abdominal pain a dull pain noticed in the right upper quadrant


Physical examination:

  • Jaundice
  • Palpable lymphadenopathy
  • Hepatomegaly

Routine lab and blood tests:

  • Liver function tests
  • Tumour markers: carbohydrate antigen 19-9, carcinoembriogenic antigen CEA

Imaging studies:

  • CT scan: helical CT
  • Abdominal ultrasound: for biliary duct dilatation, large hilar lesions


  • Magnetic resonance cholangiography
  • Endoscopic retrograde cholangiopancreatography or ERCP: to reveal the site of obstruction with retrograde dye injection

Percutaneous transhepatic cholangiography or PTC:

  • Access to proximal lesions
  • ERCP an PTC are done for extrahepatic cholangiocarcinoma, to relieve jaundice as apart of preoperative measure or to palliate if the tumor is not removable.
  • Positron emission tomography or PET
  • Endoscopic ultrasonography


Surgical intervention is considered to treat the cancer and its blockage. Removal of part of the liver is indicated in cholangiocarcinoma affecting one portion of the liver. In some a case, the patient may consider liver transplantation. At other times, if the cancer has spread to other parts of body, Cholangiocarcinoma may be treated with stenting, PDT or photodynamic therapy or radiation therapy.

Hilar cholangiocarcinoma, which is otherwise known as Klatskin tumor, usually requires removal along with complex liver resection.

We play a key role in the identification, staging and treatment of cholangiocarcinomas. Our team consist of multidisciplinary surgeons who take part in every aspect of curative resection of tumours as well as unresectable tumours.

Authored by Dr. Deepak Varma, MBBS, MS (General Surgery)

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