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{{Cholangiocarcinoma}}
{{Cholangiocarcinoma}}
{{CMG}}
{{CMG}}; {{AE}}{{F.K}} [mailto:fkahe@bidmc.harvard.edu] [mailto:fkahe@bidmc.harvard.edu], {{PSK}}


==Overview==
==Overview==
'''Cholangiocarcinoma''' is a [[cancer]] of the [[bile duct]]s, which drain [[bile]] from the [[liver]] into the [[small intestine]]. It is a relatively rare cancer, with an annual [[incidence (epidemiology)|incidence]] of 1&ndash;2 cases per 100,000 in the Western world,<ref name="Landis">{{cite journal |author=Landis S, Murray T, Bolden S, Wingo P |title=Cancer statistics, 1998 |journal=CA Cancer J Clin |volume=48 |issue=1 |pages=6–29 |year= |id=PMID 9449931}}</ref> but rates of cholangiocarcinoma have been rising worldwide over the past several decades.<ref name="rising">{{cite journal |author=Patel T |title=Worldwide trends in mortality from biliary tract malignancies |journal=BMC Cancer |volume=2 |issue= |pages=10 |year= |id=PMID 11991810}}</ref> Risk factors for cholangiocarcinoma include [[primary sclerosing cholangitis]] (an inflammatory disease of the bile ducts), congenital liver malformations, infection with the parasitic [[liver fluke]]s ''[[Opisthorchis viverrini]]'' or ''[[Clonorchis sinensis]]'', and exposure to [[Thorotrast]] ([[thorium dioxide]]), a chemical previously used in [[medical imaging]]. The symptoms of cholangiocarcinoma include [[jaundice]], [[weight loss]], and sometimes [[pruritis|generalized itching]]. The disease is diagnosed through a combination of [[blood test]]s, imaging, [[endoscopy]], and sometimes surgical exploration.
Cholangiocarcinoma refers to the malignant tumor of bile ducts. The [[epithelial cell]] lining the [[bile ducts]] are called [[cholangiocytes]]. The malignant transformsation of [[cholangiocytes]] leads to cholangiocarcinoma. [[Malignant]] [[transformation]] of [[cholangiocytes]] into cholangiocarcinoma include [[hyperplasia]], [[metaplasia]] and dysplasia. Biliary intraepithelial [[neoplasia]] is believed to be the initial lesion of cholangiocarcinoma, particularly in patients with [[hepatolithiasis]] in [[bile ducts]]. On the basis of location, cholangiocarcinoma may be classified into extrahepatic cholangiocarcinoma, perihilar cholangiocarcinoma, and intrahepatic cholangiocarcinoma. Cholangiocarcinoma must be differentiated from other diseases that cause [[jaundice]], [[abdominal pain]], [[weight loss]], and [[fatigue]], such as [[gallbladder cancer]], [[hepatocellular carcinoma]], [[pancreatic cancer]], [[cholecystitis]], and choledochitis. Common complications of cholangiocarcinoma include [[infection]], [[liver failure]], and [[Metastasis|tumor metastasis]]. Prognosis is generally poor, and the survival rate of patients with cholangiocarcinoma mainly depends on extent of the [[tumor]] and resectability. The common symptoms of cholangiocarcinoma include [[jaundice]], [[pruritis]], [[abdominal pain]], [[weight loss]]. Common physical examination findings of cholangiocarcinoma include [[jaundice]], icteric sclera, [[hepatomegaly]], right upper quadrant mass, and [[Gall bladder|palpable gall bladder]]. The mainstay of treatment for cholangiocarcinoma is surgical resection. Surgical resection of tumors with negative margins is the best option for all subtypes of cholangiocarcinoma. Chemotherapy is indicated for unresectable cholangiocarcinoma as [[palliation|palliative chemotherapy]]. Chemotherapy agents used to treat cholangiocarcinoma include [[5-fluorouracil]], [[gemcitabine]], [[irinotecan]], [[cisplatin]], or [[doxorubicin]].


Surgery is the only potentially curative treatment, but most patients have advanced and inoperable disease at the time of diagnosis. After surgery, [[adjuvant#oncology|adjuvant]] [[chemotherapy]] or [[radiation therapy]] may be given to increase the chances of cure. Patients with advanced and inoperable cholangiocarcinoma are generally treated with chemotherapy and [[palliative care]] measures. Areas of ongoing [[medical research]] in cholangiocarcinoma include the use of newer [[targeted therapy|targeted therapies]] (such as [[erlotinib]]) and the use of [[photodynamic therapy]].
==Classification==
Cholangiocarcinoma may be classified according to location of the tumor into three subtypes such as extrahepatic bile duct cancer, perihilar bile duct cancer, and intrahepatic [[bile duct]] cancer. Perihilar cholangiocarcinoma may be classified according to Bismuth-Corlette classification into five subtypes based on the extent of ductal infiltration.
 
==Pathophysiology==
The [[epithelial cell]] lining the [[bile ducts]] are called [[cholangiocytes]]. The malignant transformsation of [[cholangiocytes]] leads to cholangiocarcinoma. [[Malignant]] [[transformation]] of [[cholangiocytes]] into cholangiocarcinoma include [[hyperplasia]], [[metaplasia]] and dysplasia. Biliary intraepithelial [[neoplasia]] is believed to be the initial lesion of cholangiocarcinoma, particularly in patients with [[hepatolithiasis]] in [[bile ducts]].
 
==Causes==
There are no established causes for cholangiocarcinoma.
==Differential Diagnosis==
Cholangiocarcinoma must be differentiated from other diseases that cause [[jaundice]], [[abdominal pain]], [[weight loss]], and [[fatigue]], such as [[gallbladder cancer]], [[hepatocellular carcinoma]], [[pancreatic cancer]], [[cholecystitis]], and choledochitis.
 
==Epidemiology and Demographics==
The [[incidence]] of cholangiocarcinoma is approximately 1-2 per 100,000 individuals in the United States. The [[prevalence]] of cholangiocarcinoma is approximately 0.01% to 0.46% per 100,000 individuals. Patients of all age groups may develop cholangiocarcinoma. Cholangiocarcinoma is more common in males than in females.
 
==Risk Factors==
Common risk factors in the development of cholangiocarcinoma are chronic inflammatory conditions of bile duct, [[liver fluke]] infections, [[choledochal cysts]], toxins, and viral infections.
==Screening==
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cholangiocarcinoma.
 
==Natural history, Complications and Prognosis==
Common complications of cholangiocarcinoma include [[infection]], [[liver failure]], and [[Metastasis|tumor metastasis]]. Prognosis is generally poor, and the survival rate of patients with cholangiocarcinoma mainly depends on extent of the tumor and resectability. Even with resection, prognosis is poor with 5-year survival of only 10-44%. The presence of [[primary sclerosing cholangitis]] is associated with a particularly poor prognosis among patients with cholangiocarcinoma.


==Diagnosis==
==Diagnosis==
Cholangiocarcinoma is definitively diagnosed from tissue, i.e. it is proven by biopsy or examination of the tissue excised at surgery. It may be suspected in a patient with [[jaundice#post-hepatic|obstructive jaundice]]. Considering it as the working-diagnosis may be challenging in patients with primary sclerosing cholangitis (PSC); such patients are at high risk of developing cholangiocarcinoma, but the symptoms may be difficult to distinguish from those of PSC. Furthermore, in patients with PSC, such diagnostic clues as a visible mass on imaging or biliary ductal dilatation may not be evident.
===Staging===
The staging of cholangiocarcinoma varies depending on whether the tumor is primarily intrahepatic (ICC), hilar/perihilar (Klatskin), or extrahepatic. Current staging classifications of intrahepatic cholangiocarcinoma include UICC system, Okabayashi system, and AJCC system. Current staging classifications of perihilar cholangiocarcinoma include MSKCC system and AJCC system.


===History and Symptoms===
The common symptoms of cholangiocarcinoma include [[jaundice]], [[pruritis]], [[abdominal pain]], [[weight loss]].
===Physical Examination===
Common physical examination findings of cholangiocarcinoma include [[jaundice]], icteric sclera, [[hepatomegaly]], right upper quadrant mass, and [[Gall bladder|palpable gall bladder]].
===Laboratory Findings===
Laboratory tests for cholangiocarcinoma include [[aspartate aminotransferase]] (AST) and [[alanine aminotransferase]] (ALT), [[prothrombin time]], [[albumin]] and total protein, [[bilirubin]], [[lactate dehydrogenase|L-Lactate dehydrogenase]] and [[alkaline phosphatase]].
===CT===
On [[Computed tomography|CT scan]], cholangiocarcinoma is characterized by homogenously low in attenuation and demonstrate heterogenous minor peripheral enhancement with gradual enhancement centrally. Capsular retraction may be observed. [[Bile ducts]] distal to the mass are typically dilated.
===MRI===
On [[Magnetic resonance imaging|MRI]], cholangiocarcinoma is characterized by either isointense or hypointense relative to the normal liver on T1 and mildly to markedly hyperintense on T2. Moderate to incomplete enhancement is observed on contrast [[MRI]].
===Abdominal Ultrasound===
On abdominal [[ultrasound]], cholangiocarcinoma is characterized by obstruction and dilation of [[bile ducts]]. Mass-forming intrahepatic cholangiocarcinoma is characterized by homogeneous mass of intermediate echogenicity with a peripheral hypoechoic halo of compressed liver. Periductal infiltrating intrahepatic cholangiocarcinoma is characterized by capsular retraction. Intraductal cholangiocarcinoma is characterized by alterations in duct caliber, usually duct ectasia with or without a visible mass.
===Other Imaging Findings===
Other imaging studies for cholangiocarcinoma include [[endoscopic retrograde cholangiopancreatography]], [[endoscopic ultrasound]], [[percutaneous transhepatic cholangiography]], and [[MRCP]].
===Other Diagnostic Studies===
No additional tests are recommended for the diagnosis of cholangiocarcinoma.
==Treatment==
==Treatment==
Cholangiocarcinoma is considered curable only by [[resection|surgical removal]]. Without surgery, it is a rapidly fatal disease with 5-year survival rates of less than 5%.<ref>{{cite journal |author=Farley D, Weaver A, Nagorney D |title="Natural history" of unresected cholangiocarcinoma: patient outcome after noncurative intervention |journal=Mayo Clin Proc |volume=70 |issue=5 |pages=425-9 |year=1995 |pmid=7537346}}</ref> Often, the operability of the tumor can only be assessed at the time of surgery;<ref>{{cite journal |author=Su C, Tsay S, Wu C, Shyr Y, King K, Lee C, Lui W, Liu T, P'eng F |title=Factors influencing postoperative morbidity, mortality, and survival after resection for hilar cholangiocarcinoma |journal=Ann Surg |volume=223 |issue=4 |pages=384-94 |year=1996 |pmid=8633917}}</ref> therefore, most patients undergo exploratory surgery unless there is a clear-cut indication that the tumor is inoperable.<ref name="feldman"/>
===Medical Therapy===
 
[[Chemotherapy]] is indicated for unresectable cholangiocarcinoma as [[palliation|palliative chemotherapy]]. [[Chemotherapy]] agents used to treat cholangiocarcinoma include [[5-fluorouracil]], [[gemcitabine]], [[irinotecan]], [[cisplatin]], or [[doxorubicin]].
Adjuvant therapy followed by [[liver transplantation]] may have a role in treatment of certain unresectable cases.<ref> Heimbach JK, Gores GJ, Haddock MG, et al, Predictors of disease recurrence following neoadjuvant chemoradiotherapy and liver transplantation for unresectable perihilar cholangiocarcinoma, Transplantation. 2006 Dec 27;82(12):1703-7.</ref>
===Surgery===
The mainstay of treatment for cholangiocarcinoma is surgical resection. Surgical resection of tumors with negative margins is the best option for all subtypes of cholangiocarcinoma.
===Prevention===
Effective measures for the primary prevention of cholangiocarcinoma [[hepatitis B]] [[vaccination]], weight reduction, avoiding excess alcohol use.


==References==
==References==
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Latest revision as of 14:45, 13 February 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Farima Kahe M.D. [2] [3] [4], Suveenkrishna Pothuru, M.B,B.S. [5]

Overview

Cholangiocarcinoma refers to the malignant tumor of bile ducts. The epithelial cell lining the bile ducts are called cholangiocytes. The malignant transformsation of cholangiocytes leads to cholangiocarcinoma. Malignant transformation of cholangiocytes into cholangiocarcinoma include hyperplasia, metaplasia and dysplasia. Biliary intraepithelial neoplasia is believed to be the initial lesion of cholangiocarcinoma, particularly in patients with hepatolithiasis in bile ducts. On the basis of location, cholangiocarcinoma may be classified into extrahepatic cholangiocarcinoma, perihilar cholangiocarcinoma, and intrahepatic cholangiocarcinoma. Cholangiocarcinoma must be differentiated from other diseases that cause jaundice, abdominal pain, weight loss, and fatigue, such as gallbladder cancer, hepatocellular carcinoma, pancreatic cancer, cholecystitis, and choledochitis. Common complications of cholangiocarcinoma include infection, liver failure, and tumor metastasis. Prognosis is generally poor, and the survival rate of patients with cholangiocarcinoma mainly depends on extent of the tumor and resectability. The common symptoms of cholangiocarcinoma include jaundice, pruritis, abdominal pain, weight loss. Common physical examination findings of cholangiocarcinoma include jaundice, icteric sclera, hepatomegaly, right upper quadrant mass, and palpable gall bladder. The mainstay of treatment for cholangiocarcinoma is surgical resection. Surgical resection of tumors with negative margins is the best option for all subtypes of cholangiocarcinoma. Chemotherapy is indicated for unresectable cholangiocarcinoma as palliative chemotherapy. Chemotherapy agents used to treat cholangiocarcinoma include 5-fluorouracil, gemcitabine, irinotecan, cisplatin, or doxorubicin.

Classification

Cholangiocarcinoma may be classified according to location of the tumor into three subtypes such as extrahepatic bile duct cancer, perihilar bile duct cancer, and intrahepatic bile duct cancer. Perihilar cholangiocarcinoma may be classified according to Bismuth-Corlette classification into five subtypes based on the extent of ductal infiltration.

Pathophysiology

The epithelial cell lining the bile ducts are called cholangiocytes. The malignant transformsation of cholangiocytes leads to cholangiocarcinoma. Malignant transformation of cholangiocytes into cholangiocarcinoma include hyperplasia, metaplasia and dysplasia. Biliary intraepithelial neoplasia is believed to be the initial lesion of cholangiocarcinoma, particularly in patients with hepatolithiasis in bile ducts.

Causes

There are no established causes for cholangiocarcinoma.

Differential Diagnosis

Cholangiocarcinoma must be differentiated from other diseases that cause jaundice, abdominal pain, weight loss, and fatigue, such as gallbladder cancer, hepatocellular carcinoma, pancreatic cancer, cholecystitis, and choledochitis.

Epidemiology and Demographics

The incidence of cholangiocarcinoma is approximately 1-2 per 100,000 individuals in the United States. The prevalence of cholangiocarcinoma is approximately 0.01% to 0.46% per 100,000 individuals. Patients of all age groups may develop cholangiocarcinoma. Cholangiocarcinoma is more common in males than in females.

Risk Factors

Common risk factors in the development of cholangiocarcinoma are chronic inflammatory conditions of bile duct, liver fluke infections, choledochal cysts, toxins, and viral infections.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for cholangiocarcinoma.

Natural history, Complications and Prognosis

Common complications of cholangiocarcinoma include infection, liver failure, and tumor metastasis. Prognosis is generally poor, and the survival rate of patients with cholangiocarcinoma mainly depends on extent of the tumor and resectability. Even with resection, prognosis is poor with 5-year survival of only 10-44%. The presence of primary sclerosing cholangitis is associated with a particularly poor prognosis among patients with cholangiocarcinoma.

Diagnosis

Staging

The staging of cholangiocarcinoma varies depending on whether the tumor is primarily intrahepatic (ICC), hilar/perihilar (Klatskin), or extrahepatic. Current staging classifications of intrahepatic cholangiocarcinoma include UICC system, Okabayashi system, and AJCC system. Current staging classifications of perihilar cholangiocarcinoma include MSKCC system and AJCC system.

History and Symptoms

The common symptoms of cholangiocarcinoma include jaundice, pruritis, abdominal pain, weight loss.

Physical Examination

Common physical examination findings of cholangiocarcinoma include jaundice, icteric sclera, hepatomegaly, right upper quadrant mass, and palpable gall bladder.

Laboratory Findings

Laboratory tests for cholangiocarcinoma include aspartate aminotransferase (AST) and alanine aminotransferase (ALT), prothrombin time, albumin and total protein, bilirubin, L-Lactate dehydrogenase and alkaline phosphatase.

CT

On CT scan, cholangiocarcinoma is characterized by homogenously low in attenuation and demonstrate heterogenous minor peripheral enhancement with gradual enhancement centrally. Capsular retraction may be observed. Bile ducts distal to the mass are typically dilated.

MRI

On MRI, cholangiocarcinoma is characterized by either isointense or hypointense relative to the normal liver on T1 and mildly to markedly hyperintense on T2. Moderate to incomplete enhancement is observed on contrast MRI.

Abdominal Ultrasound

On abdominal ultrasound, cholangiocarcinoma is characterized by obstruction and dilation of bile ducts. Mass-forming intrahepatic cholangiocarcinoma is characterized by homogeneous mass of intermediate echogenicity with a peripheral hypoechoic halo of compressed liver. Periductal infiltrating intrahepatic cholangiocarcinoma is characterized by capsular retraction. Intraductal cholangiocarcinoma is characterized by alterations in duct caliber, usually duct ectasia with or without a visible mass.

Other Imaging Findings

Other imaging studies for cholangiocarcinoma include endoscopic retrograde cholangiopancreatography, endoscopic ultrasound, percutaneous transhepatic cholangiography, and MRCP.

Other Diagnostic Studies

No additional tests are recommended for the diagnosis of cholangiocarcinoma.

Treatment

Medical Therapy

Chemotherapy is indicated for unresectable cholangiocarcinoma as palliative chemotherapy. Chemotherapy agents used to treat cholangiocarcinoma include 5-fluorouracil, gemcitabine, irinotecan, cisplatin, or doxorubicin.

Surgery

The mainstay of treatment for cholangiocarcinoma is surgical resection. Surgical resection of tumors with negative margins is the best option for all subtypes of cholangiocarcinoma.

Prevention

Effective measures for the primary prevention of cholangiocarcinoma hepatitis B vaccination, weight reduction, avoiding excess alcohol use.

References

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