A 72-year-old female presented with chief complains of yellowish discolouration of skin and eyes, abdominal pain, and itching. She had a history of unintentional 6-kgs weight loss during the past 2 months.

Physical examination revealed jaundice, scratch marks from pruritis abdominal tenderness. Liver function tests revealed a high direct bilirubin level of 14.3 mg per decilitre, aspartate aminotransferase (AST) level of 55 U per litre, an alanine aminotransferase (ALT) level of 56 U per litre and an alkaline phosphatase level (ALP) of 223 U per litre.

Imaging studies were conducted. Magnetic resonance imaging (MRI) of the abdomen showed a hepatic hilar mass with vascular involvement and marked intrahepatic biliary ductal dilatation, with tapering and cut off near the hilum (arrow in the image).

A diagnosis of hilar cholangiocarcinoma or Klatskin’s tumour was made.

Percutaneous transhepatic cholangiography (PTC) with biliary drainage was performed and a sample from the hilar mass was obtained for the biopsy. Diagnosis of cholangiocarcinoma was confirmed by histopathological analysis.

Since the vasculature i.e. the hepatic artery and portal vein were involved, the tumour was deemed inoperable and unresectable. Therefore, the patient was offered supportive palliative care and biliary drainage.

Cholangiocarcinoma, in simpler terms, is the cancer of the bile ducts, and bile ducts are the tubes that connect gall bladder to the liver and to the intestines. Bile ducts have the function of carrying bile from the liver, where it is produced, to the gall bladder, where it is stored, and to the small intestines, where bile is utilised to digest food.

Cholangiocarcinoma mostly affects individuals older than 65 years of age. It is classified into three, according to the location in the bile duct with respect to the liver:

1. Intrahepatic cholangiocarcinoma: This name is given when it begins in the bile ducts within the liver, hence the name. It is the least common form (10% of all cases).
   
2. Hilar/Perihilar cholangiocarcinoma: This type, which begins in the hilum, is the most common type accounting for half of the cases of cholangiocarcinoma. It is also called Klatskin tumour.
   
3. Distal cholangiocarcinoma: When the disease begins in the bile ducts which are outside the liver, it is named distal cholangiocarcinoma.

The latter two of the above classification are grouped into extrahepatic cholangiocarcinoma because the disease begins in the bile ducts outside of the liver.

The initial most common symptom is jaundice, attributable to the blockage of the bile ducts. Other clinical features include itching, dull ache in the abdomen (particularly in the right hypochondriac region), pale and greasy stools, dark urine, unintentional weight loss and loss of appetite.

Signs on physical examination may include a palpable gallbladder, called the Courvoisier sign, mass in the abdomen, enlarged liver (hepatomegaly) or enlarged lymph nodes.

Risk factors that increase the chances of cholangiocarcinoma are chronic liver diseases, congenital bile duct anomalies, primary sclerosing cholangitis, Liver fluke infection, old age and smoking

The major challenge in the treatment of cholangiocarcinoma is that neither of the three types gives symptoms early on. It is only after the disease has spread to the surrounding structures beyond the bile ducts, that the symptoms appear and the disease is diagnosed. Moreover, the survival rate is low due to the inoperable nature of cancer. The average survival duration is approximately six months to one year.

References

Peter E Darwin, M. (2019, December 28). Cholangiocarcinoma. Retrieved from Medscape: https://emedicine.medscape.com/article/277393-overview

Rizvi S, Khan SA, Hallemeier CL, Kelley RK, Gores GJ. Cholangiocarcinoma – evolving concepts and therapeutic strategies. Nat Rev Clin Oncol. 2018 Feb. 15 (2):95-111

Banales JM, Cardinale V, Carpino G, Marzioni M, Andersen JB, Invernizzi P, Lind GE, Folseraas T, Forbes SJ, Fouassier L, Geier A, Calvisi DF, Mertens JC, Trauner M, Benedetti A, Maroni L, Vaquero J, Macias RI, Raggi C, Perugorria MJ, Gaudio E, Boberg KM, Marin JJ, Alvaro D.Expert consensus document: Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA). Nat Rev Gastroenterol Hepatol. 2016 May;13(5):261-80. DOI: 10.1038/nrgastro.2016.51. Epub 2016 Apr 20