Klatskin tumor commonly known as hilar cholangiocarcinoma (cholangiocarcinoma) is characterized by the cancer of the biliary tree majorly involving the junctions of right and left hepatic ducts.
This cancerous disorder was firstly introduced in 1965 by Gerald Klatskin, who reported 15 cases of this disease.
The major underlying cause of this disease has not been defined yet but several pathological conditions may lay the bases for the development of this disorder. One of these conditions is Primary sclerosing cholangitis which is considered responsible for approximately 40% of the cholangiocarcinoma cases. Other condition may include;
- Congenital biliary cystic diseases, such as choledochal cysts or Caroli’s disease (responsible for 25% of cholangiocarcinoma cases)
- Clonorchis Sinensis
- Opisthorchis viverrini
The population of both genders; male and female have equal vulnerability for this disease and it is generally accounted for 2% of all the cancerous cases.
klatskin Tumor Types
Generally, there are two types of classifications that have been used widely among the concerned medical professionals including;
- TNM classification: This classification is developed to describe the quantity and spread rate of the cancerous cells in the body.
- Bismuth classification: This classification is generally based on the localization of tumors in the extra-hepatic biliary tree.
(size of the tumor and its degree of spread into the adjacent cells)
(Spread of cancer to the neighboring lymphatic nodes)
(Metastasis: the spread of cancer to other body parts)
|Stage II||T2a, T2b||N0||M0|
|Stage IIIb||T1, T2, T3||N1||M0|
|Stage IVa||T4||N0, N1||M0|
|Stage IVb||T1-T4||N0, N1||M1|
- Tis – carcinoma in situ
- T1 – the tumor is in the muscular layer of biliary duct
- T2a – tumor penetrates to lipid tissue
- T2b – tumor penetrates to liver parenchyma
- T3 – tumor penetrates to the left or right portal vein or left or right hepatic artery
- T4 – tumor penetrates to portal vein, or left and right portal vein, or common hepatic artery
|Bismuth I||It involves all the tumors of the common hepatic duct that do not reach the junction.|
|Bismuth II||It involves all the tumors of common hepatic duct, that do approach the junction|
|Bismuth III||It involves all the tumors of the common hepatic duct and left or right hepatic duct|
|Bismuth IV||It involves all the tumors of common hepatic duct and left and right hepatic duct.|
klatskin Tumor Treatment
There is much-suggested treatment option by the professionals but the choice mainly depends upon the location and extent of the tumors. However the major goal of the treatment is to eliminate the biliary blockage ultimately leading to the eradication of the disease. The most common treatment option may involve;
Chemotherapy: Those tumors which fall under the category of non-resectable tumors are suggested for the chemotherapy. Combination therapy of Gemcitabine along cisplatin is considered very effective for these particular patients.
Radiotherapy: Those tumors which fall under the category of non-resectable tumors are suggested for the radiotherapy.
Surgical therapy: Complete removal of the tumor is carried out under this category and tends to improve the prognosis among patients ultimately resulting in an improved quality of life.
Palliative therapy: It is generally based on the hepatic ducts patency involving the drainage of the bile
- internal drainage involves the insertion of a metallic stent via ERCP
- external drainage involves the technique of PTD called percutaneous transhepatic drainage.
klatskin Tumor Prognosis
The prognosis of this disease is highly dependent upon the phase at which the tumor is diagnosed as well the condition of this tumor whether it is allowing the resection (surgical therapy) or not. As these tumors are majorly diagnosed in their late stages and worsen the prognosis among patients. Those tumors which can be resected after surgical procedures may have 5 years survival rate ranging from 25% to 30%. Meanwhile those tumors which do not allow the resection due to their worst condition limits the survival rate to 0%.
Most positive outcomes are obtained from the surgical methods involving the resection of the tumors but professionals suggest the palliative drainage for non-resected tumors extending the survival rate to 2-8 months. The basic goal of palliative care is to enhance the patient’s quality of life and this includes the management of cholestasis and cholangitis which indirectly results in the prolongation of the survival rate.
klatskin Tumor Life Expectancy
Living with a genetic and rare disease can be stressful and hard which ultimately affects the daily routines of the patients as well as their families. These diseases impose drastic effects on the quality of life among patients.
It is the responsibility of the concerned medical professionals to facilitate these patients with every possible opportunity to make their life easy and to improve their quality of life.