CANCER OF BILE DUCT (CHOLANGIOCARCINOMA)


( CANCER OF BILE DUCT (CHOLANGIOCARCINOMA

Introduction
 cancer of bile duct cholangiocarcinomaCancer 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

 
Incidence

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
 Pathology

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
Signs
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
 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
 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
 
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
 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
 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
Treatment palliative to relieve symptoms as potent analgesia for pain surgery not prefer in these cases

Prognosis
Cholangiocarcinoma has poor prognosis and patient may die from hepatic failure or cholangitis

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