Introduction
Cancer or carcinoma of the bile duct it is also called Cholangiocarcinoma it is a rare malignant tumour of the biliary channels which arise from the epithelium and it can occur at any site along the biliary tree from common bile duct or its branches which divided into right and left hepatic ducts common site present at the hepatic duct bifurcation
According to the site of the tumor it can be divided into either distal or proximal or perihilar Cholangiocarcinoma
Common in male more than female
Age elderly patients usually above 50 years
Causes of cholangiocarcinoma
There are many diseases which considered as a risk factors for increase incidence of cholangiocarcinoma and these are
- primary sclerosing cholangitis see here
- ulcerative colitis
- stone bile duct hepatolithiasis
- biliary--enteric anastomosis
- biliary tract infections with Clonorchis
- chronic typhoid carriers
- liver flukes
- dietary factors like exposure to nitrosamines,Thorotrast, and exposure to dioxin
Over 95% of bile duct cancers are adenocarcinomas
Morphologically they are divided into
- nodular (the most common type
- scirrhous
- diffusely infiltrating
- papillary
About two-thirds of cholangiocarcinomas are located in the perihilar location
Perihilar cholangiocarcinomas, also referred to as Klatskin tumors, are further classified based on anatomic location by the Bismuth-Corlene classification
Type I
tumors are confined to the common hepatic duct
type II
tumors involve the bifurcation without involvement of the secondary intrahepatic ducts
Type IIIa and IIIb
tumors extend into the right and left secondary intrahepatic ducts
Type IV
tumors involve both the right and left secondary intrahepatic ducts
Symptoms and signs
Symptoms
- Asymptomatic patients are found to have cholangiocarcinoma which may discovered incidentally by elevated alkaline phosphatase and gamma glutamyltransferase levels
- Painless jaundice is the most common presentation
- Pruritus or itching due to presence of bile salts in the blood
- right upper quadrant pain mild pain
- anorexia, fatigue, and weight loss
- Cholangitis means inflammation of the biliary tract in the form of jaundice fever abdominal pain common presentation for more see here
Jaundice may be only signs present in the form of yellowish discolouration of the skin and sclera of the eye
Scratching markers from itching or pruritus may be present in severe cases
Investigations
Laboratory
Liver function test
may show raising of alkaline phosphatase and gamma glutamyltransferase levels and serum bilirubin but these not specific for cholangiocarcinoma and may be found in others diseases but give idea about something is wrong
Complete blood count
may showing anemia leukopenia
Urine analysis
may showing urobilinogin or bilirubin
Tumor markers
these are substance which present normally in the blood serum urine stool tears aspirated fluids which if more elevated may indicated a cancer for more details see here
Radiological studies
Abdominal ultrasongraphy
It is usually difficult to visualize the tumor itself on ultrasound but it can showing the level of obstruction and associated stone in gall bladder or bile ducts in the presence of obstructive jaundice
and determine portal vein patencyand also showing biliary dilatation
A perihilar tumor causes dilatation of the intrahepatic biliary tree, but normal or collapsed gallbladder and extrahepatic bile ducts distal to the tumor
A perihilar tumor causes dilatation of the intrahepatic biliary tree, but normal or collapsed gallbladder and extrahepatic bile ducts distal to the tumor
Distal bile duct cancer leads to dilatation of the extra- and the intrahepatic bile ducts as well as the gallbladder
CT scan abdomen
Same as the ultrasound
Transheptic cholangiography PTC
Transheptic cholangiography PTC
Showing the biliary anatomy PTC defines the proximal extent of the tumor which is the most important factor in determining resectability
ERCP is used to evaluated the distal bile duct tumors
.
For the evaluation of vascular involvement
MRI and MRCP scanning
Very excellent diagnostic studies which showing
the biliary anatomy, lymph nodes, and vascular involvement, as well as the tumor growth itself
A small incisions is done on the abdominal wall then a catheter and camera video can showing the tumor and spreading and can taken tissue biopsy
Biopsy
Tissue diagnosis may be difficult to obtain nonoperatively except in advanced cases
Percutaneous tine-needle aspiration biopsy biliary brush or scrape biopsy, and cytologic examination have a low sensitivity in detecting malignancy
Curable cases
Surgical excision is the only potentially curative treatment for cholangiocarcinoma
Indications
If they have no signs of metastasis or locally unresectable disease
Palliative treatment
Aim to relieve obstruction and symptoms
Surgical bypass operations for biliary decompression and cholecystectomy to prevent the occurrence of acute cholecystitis and this depend on site of the tumor and level of obstruction
Despite improvements in ultrasonography CT scanning
and MRI, more than one half of the patients who are explored are found to have peritoneal implants, nodal or hepatic metastasis, or locally advanced disease that precludes resection
and MRI, more than one half of the patients who are explored are found to have peritoneal implants, nodal or hepatic metastasis, or locally advanced disease that precludes resection
These by pass operations are as follow
To either segment II or III bile ducts or to the right hepatic duct can be performed in case of unresectable perihilar cbolangiocarcinoma
Perhilar tumors involving the bifurcation or proximal common hepatic duct type I or II stags with no signs of vascular involvemeat
Are treated by local tumor excision with portal lymphadenctomy, cholecystectomy, common bile duct excision, and bilateral Roux-en- Y hepaticojejunostomies
If the tumor involves the right or left hepatic duct type IIIa or IIIb stags
Are treated by right or left hepatic (liver) lobectomy and resection of the adjacent caudate lobe of the liver is required because of direct extension into caudate biliary radicals or parenchyma.
Distal bile duct tumors are more resectable
Distal bile duct tumors are more resectable
They are treated with pylorus-preserving pancreatoduodenectomy Whipple procedure
For patients with distal bile duct cancer found to be unresectable on surgical exploration
Roux-en-Y hepaticojejunostomy, cholecystectomy, and gastrojejunostomy to prevent gastric outlet obstruction should be performed
Nonoperative biliary decompression
Nonoperative biliary decompression
is performed for petieuts with unresectable disease on diagnostic evaluation
Proximal bile duct tumors
Percutaneous placement of expandable metal stents or drainage catheterss
Distal bile duct tumors
Endoscopic placement of stent
There is increase risk of cholangitis with internal and external
drainage, and stent occlusion is not uncommon
Chemotherapy
Drugs like 5-fluorouracil alone or in combination with mitamycin c and doxorubicin for unresectable tumors but the response are low
For more details see here
Radiotherapy
Has no or little role when used with chemotherapy may become more effective than used alone
For more details see here
Recurrent tumors
Recurrent tumors
Treatment palliative to relieve symptoms as potent analgesia for pain surgery not prefer in these cases
Prognosis
Prognosis
Cholangiocarcinoma has poor prognosis and patient may die from hepatic failure or cholangitis
You can see also
You can see also
- Gallbladder anatomy
- Biliary duct anatomy including the common bile duct common hepatic duct and its branches and cystic duct anatomy
- Diagnosis or investigations of biliary channels
- Gallbladder function
- Gallbladder stones causes types diagnosis and treatment
- Acute cholecystitis causes types diagnosis and treatment
- Chronic cholecystitis causes types diagnosis and treatment
- Acaclular or non calcular cholecystitis causes diagnosis and treatment
- Ascending cholangitis causes diagnosis and treatment
- Sclerosing cholangitis types causes diagnosis and treatment
- Gallbladder cancer causes diagnosis and treatment
- Bile duct cancer or cholangiocarcinoma causes diagnosis and treatment
- Bile duct cysts or choledochal cysts causes types diagnosis and treatment
- Bile ducts injury or strictures causes types diagnosis and treatment
- Problems after gall bladder removal or postcholecystectomy complications
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