Mini Review

Cholangiocarcinoma and Therapy

Goral V*
Department of Gastroenterology, Izmir University School of Medicine, Turkey

*Corresponding author: Vedat Goral, Department of Gastroenterology, Izmir University School of Medicine, Izmir, Turkey

Published: 15 Apr, 2016
Cite this article as: Goral V. Cholangiocarcinoma and Therapy. Remed Open Access. 2016; 1: 1005.


Cholangiocarcinoma is a malignant neoplasm originating from biliary epithelial cells. The incidence and mortality of disease are rising in the world. Currently, cholangiocarcinoma is accepted as stem cell disease. There are many risk factors for cholangiocarcinoma. Diagnosis of disease is easy but therapy of disease is quite difficult. Surgical resection and liver transplantation are the best therapies for the disease.

Keywords: Cholangiocarcinoma; Stemcelldisease; Pathogenesis


Cholangiocarcinoma (CC) is a malignant tumor of adenocarcinoma nature originating from the epithelial cells of bile ducts (intrahepatic, hilar and extrahepatic) [1]. It has a prevalence of 0.5-1.2/100.000 people and is more common in men than women. CC incidence is gradually increasing especially in patients with intra hepatic cholangiocarcinoma. The rate of 5-year survival is about 5-10% includingnewly-diagnosed cases and 5-year chance of survival following potential surgery is 25-30%. In metastatic cases, median survival is no longer than 8-12 month seven under a pharmaceutical or combined therapy. Japan, Chile, Eastern Asia and India are countries with highest CC incidence.
Risk factors
In CC patients especially in western countries, risk factorsare not known in 90% of the cases and chronic inflammation and biliary irritation are held responsible [2,3]. In 10% of the cases, primary sclerosing cholangitis (PSC), obesity, hepatolithiasis, bile stasis-associated cholangitis, hepatitis B and C (C>B), HIV, parasitic infections (endemic in southeastern Asia with 14-27-fold increase in CC risk) may result in this disease. Parasitic infestations increase CC risk by leading to chronic inflammation (especially opisthorchis viverrini, clonorchis sinensis, schistosoma Japonica). Besides, diabetes mellitus, smoking, advancedage (65% is above 65 years of age), post-biliarysurgery, biliaryentericanastomosis, chronic inflammatory diseases, chronic typhoid carriers (6 times higher risk) and cryptosporidiosis cases, hepatic cirrhosis, congenital causes (choledochalcysts, Caroli's disease, congenital hepatic fibrosis), chemical agents (thorotrast, dioxin, nitrosamines, asbestos), some medicines (oral contraceptives, isoniazide) with prolonged use may present risk.
Symptoms and diagnostic methods
Primary symptoms detected in these patients vary from weakness, pain, nausea-vomiting to obstructive jaundice and sepsis and there is currently no specific diagnostic laboratory blood test. AST, ALT are generally normal but may be high in acute obstruction or cholangitis. Prolonged obstruction disturbs fat-soluble vitamin absorption and PTZ is prolonged. Albumin, sedimentation, CRP and Hb values are variable. Urinary proteomic analysis, which recently came to fore ground may be beneficial in diagnosis (in differentiating from cholangitis).
Imaging methods: In CC diagnosis, the used methods are a) USG b) High resolution /spiral CT c) MRI d) Cholangiography (MRCP, ERCP, PTC) e) EUS, miniprob USG f) PET (IHCC>1 cm, 85-90%) and PET-BT g) Cholangioscopy (percutaneous, transpapillary), endomicroscopy h) histologyandcytology [4,5].
Tumor markers: CA 19-9 and CA 125 are the most commonly used tests. CA 19-9 is a weak diagnostic marker with a sensitivity of 40-70%, specificity of 50-80% and PPV of 16-40%. CA 19-9 is also increased in PSC and non-malignant obstructive events. CA-125 may be increased in 65% of these patients. Inaddition, CEA, serum total sialicacid, CYFRA 21-1, TGF-β, TUM2-PK, serotonin levels may also be studied. As new markers, it may sometimes be necessary to look at Mac-2BP, MMP-7, insulin-like growth factor 1, IL-6, trypsinogen and MUCIN-5AC levels.
Treatment is usually divided in 3 main groups: 1) endobiliary treatments 2) limited pharmaco therapy and 3) surgical treatment. Endobiliary treatments are performed in inoperable patients [6]. ERCP - guided bile drainage, plasticor metal stent placement are performed. However, this method has some complication ssuch as sepsis, tumor in growth and obstruction. —it may sometimes become necessary to repeat the procedures. Stenting with end obiliary RFA is a more reliable method. Chemotherapy may be in two forms in thes epatients: a) chemotherapy in locally advance dormetastatic cases and b) adjuvant therapy. In CC, the benefit of adjuvant therapy has not been established. Radio therapy: a) external beam radyoterapi and chemoradiation or b) local radiation techniques (intraoperative and intraductal brachy therapy) is performed. —Locoregional treatments as a) —TACE b) Radio frequency ablation c) — radioembolization may be performed in suitable CC patients. Endoscopic radiofrequency: Photofrin (porfimer sodium) is used, which is a selective method and accumulates in neoplastic tissue.


In conclusion, cholangiocellular carcinoma is a malignant disease which is suggested to be a stem cell disease triggered by inflammation, environmental and genetic factors. It originates from bile ducts and has a poor diagnosis. Future advances in the etiopathogenesis of the disease will contribute to a better understanding of the condition and its better treatment.


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