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DR NILESH TULASKAR
Definition of Cholangiocarcinoma
Cholangiocarcinoma is a rare tumor arising from the biliary epithelium and
cholangiocarcinoma should be considered in every patient with obstructive jaundice have advanced disease at the time of diagnosis.
Surgical resection offers the only chance for cure, however, many patients
Incidence
The incidence of cholangiocarcinomas increases with
Risk Factors
Primary sclerosing cholangitis Liver flukes infestation (Opisthorchis viverrini and Clonorchis
sinensis)
Hepatitis C
Lynch syndrome II Bile duct adenoma and multiple biliary papillomatosis
Classification
This classification
correlates with the anatomic distribution and indicates the preferred treatment for each site.
Classification
Intrahepatic cholangiocarcinomas present as liver
masses.
left and right duct and usually both intra- and extrahepatic ducts. It is also known as Klatskin's tumor.
proper hepatic duct without extension to the confluens of the right and left ducts.
Bismuth-Corlette Classification
Type I
Type II
Type III
Type IV
Pathology
More than 90% of cholangiocarcinomas are
adenocarcinomas.
squamous cell carcinomas, sarcomas, small cell cancer, and lymphomas. types: sclerosing, nodular (mass-forming), papillary,
Pathology
Sclerosing tumors, which comprise over 80% of
cholangiocarcinomas, are associated with an intense desmoplastic reaction, tend to be highly invasive, and are associated with low resectability rates.
lesions and are also associated with low resectability rates. project into the bile duct lumen.
jaundice relatively early in their progression, they are associated with higher resectability rates than sclerosing or nodular tumors. It is associated with a favorable outcome
Clinical Presentation
Most CCs remain clinically silent until the advanced stage.
Pruritis (66%),
Abdominal pain,
Clay coloured stool Tumors of the bile ducts within the liver often have pain without
jaundice.
intrahepatic cholangiocarcinoma.
A palpable gallbladder (Courvoisier's sign ). Obstruction of the bile duct and biliary stasis may lead to
Diagnostic Evaluation
Laboratory Tests
Liver function tests generally reveal elevated bilirubin, alkaline phosphatase,
and -glutamyltransferase.
reveal an increased alkaline phosphatase level in the setting of normal bilirubin levels.
Cytology analysis: Obtained by brush cytology or bile duct biopsy during ERCP. Percutaneous biopsy of the primary tumor is not advised.( tumor spread)
Radiologic Evaluation
The radiologic evaluation of patients with
Radiologic Evaluation
Imaging studies are used to determine the level of
biliary obstruction and hepatic involvement and to assess for vascular invasion or metastatic disease.
Ultrasonography Computed tomography Magnetic resonance cholangiopancreatogram Endoscopic cholangiogram Percutaneous transhepatic cholangiography
Ultrasonography
Non-invasive Uses sound waves to
determine the level of biliary obstruction, hepatic involvement and overall extend of disease.
poor in the diagnosis of CC, and staging relies on other imaging modlities.
Computed tomography
Helpful in the staging, pre-op planning, and
CT.
Evaluation of intraductal spread and detection of
Cholangiography
MRCP is the best imaging modality for CC.
Provides information about tumor extend, biliary and hepatic parenchymal anatomy, and intrahepatic metastasis.
Non invasive
MRI/MRCP
Diagnosis
Endoscopic ultrasound
PET
Angiography
It shows the anatomical location of hepatic artery
Staging laparoscopy
T2=
T3=
Tumor invades the liver, gallbladder, pancreas, and/or unilateral branches of the portal vein (right or left) or hepatic artery (right or left) Tumor invades any of the following: main portal vein or its branches bilaterally, common hepatic artery, or other adjacent structures, such as the colon, stomach, duodenum, or abdominal wall Regional lymph node metastasis Distant metastasis
T4=
N1= M1=
Treatment criteria
Based on the location and extent of the tumor, distant
-surgery!
radicals
Bilateral hepatic artery involvement Encasement of the portal vein proximal to its bifurcation Atrophy of one hepatic lobe with contralateral portal
vein encasement
radical involvement
Distant metastasis
Management
Surgical excision is the only potentially curative
techniques have resulted in lower mortality and better outcome for patients undergoing aggressive surgical excision for cholangiocarcinoma.
Operative Approach
Surgical exploration should be undertaken in good-risk patients
to have either peritoneal or hepatic metastases or, more likely, locally unresectable disease. potentially resectable perihilar cholangiocarcinoma may avoid laparotomy in some patients with metastatic disease.
several operative approaches are available for palliation, including a Roux-en-Y hepaticojejunostomy to segment III or V.
Intrahepatic Cholangiocarcinoma
The goal of resection is to remove all liver parenchyma at
risk for intrahepatic metastases based on the proximal extent of the tumor.
Usually this requires a lobectomy. If both lobes of the liver
are involved with metastases, curative resection is unlikely, and other forms of therapy should be considered.
Extrahepatic spread pretends a poor prognosis and in
Intrahepatic cholangiocarcinoma
Intrahepatic cholangiocarcinoma is treated by
hepatic resection, and outcomes depend on disease stage (particularly the status of the lymph nodes) and the ability to achieve negative margins.
There is a broad range of long-term outcomes in
Distal Cholangiocarcinoma
Extrahepatic cholangiocarcinoma not involving the confluens of the right
and left main hepatic ducts involves the common hepatic duct and commonly involves the intrapancreatic portion of the duct.
extrahepatic bile duct to the level of the confluens for complete clearance of disease.
Rarely, the tumor may be confined to a small region of the duct and an
In any case, the resection should again include a complete clearance of the periportal lymph nodes: all tissue in the porta hepatis excluding the portal vein and hepatic artery.
Distal Cholangiocarcinoma
For extensive involvement of the bile duct without distant spread,
consideration can be made for en bloc combined hepatic and pancreatic resections. The morbidity of such extensive surgery is high, and the overall prognosis is poor with extensive disease.
The incidence of distal common bile duct tumors compared with
cholecystectomy, Roux-en-Y hepaticojejunostomy proximal to the tumor, and a gastrojejunostomy to prevent gastric outlet obstruction should be performed.
Median survival is expected to be between 18 and 33 months, and
Roux-en-Y Hepaticojejunostomy
Perihilar cholangiocarcinoma
For perihilar cholangiocarcinomas, bile duct resection alone leads to high
local recurrence rates due to early involvement of the confluence of the hepatic ducts and the caudate lobe branches.
Surgical treatment depends on the Bismuth-Corlette classification . For type I and II lesions, the procedure is en bloc resection of the
extrahepatic bile ducts and gallbladder with 5- to 10-mm bile duct margins, and regional lymphadenectomy with Roux-en-Y hepaticojejunostomy.
lobectomy. Because type II and III lesions often involve the ducts of the caudate lobe, many surgeons recommend routine caudate lobectomy. centers with expertise in these procedures. Aggressive techniques such as hepatectomy and portal vein resection to achieve negative margins are now routine in specialized centers.
Endoscopic
Is able to see the biliary ducts and place the biliary endoprothesis( plastic/ metallic stent)
fluorouracil alone or in combination with mitomycin C and doxorubicin, but the response rates are low
than either treatment alone for unresectable disease, but no data from randomized trials are available
Adjuvant Therapy
resection with curative intent to reduce the risk of local recurrence and potentially improve survival.
Postoperative adjuvant radiotherapy for biliary tract
cancer can be administered either by external-beam radiotherapy (EBRT), brachytherapy, intraoperative radiotherapy (IORT), or a combination of radiotherapy modalities.
EBRT is the most commonly used radiotherapy modality
Adjuvant radiotherapy
Advantages of EBRT include the widespread availability of
this modality, its noninvasive nature, and the ability to deliver a homogeneous high dose to a large volume.
Most commonly, radiotherapy is administered in a
to 50 Gy (at 1.80 Gy/d) to the tumor bed and draining lymph node basin.
Palliative treatment
Photodynamic therapy:
inject photosensitive agent to the bodyappropriate light wave to produce tumor cell death via cholangioscopy biliary decompression.
Treat the patient symptomatically( pain, ascites, anorexia, and jaundice with pruritus). Comfort is the goal! Psychosocial support also is essential.
Photodynamic treatment
PDT uses laser, or other light sources, combined with a light-
sensitive to light. Once in the body, the drug is attracted to cancer cells. When the light is directed at the area of the cancer, the drug is activated and the cancer cells are destroyed. Some healthy, normal cells in the body will also be affected by PDT, although these cells will usually heal after the treatment
but the success of living-related transplantation may rejuvenate the use of transplant for hilar and intrahepatic cholangiocarcinoma.
Complete hepatectomy provides the best chance of a complete resection
for these tumors, and may be an alternative for patients who are unresectable by conventional means.
The real possibility of achieving a cure for unresectable tumors exists
recurrence; the median time from transplantation to recurrence was 9.7 months, and the median time between recurrence and death was 2 months.
Extrahepatic nodal disease or metastases are a
contraindication to transplant.
Cholangitis
Prognosis
Poor The prognosis varies based on resectibility and
months for the first 3 years after surgery and then at longer interval of 6 months till year 5 is reasonable.
The role of CA 19-9 as surveillance is not clear, but persistently rising
clinical trials, but because of the high risk of recurrence, radiological evaluation with CT scans of the abdomen, every 6 months for 2 to 3 years after surgery may detect recurrent disease.
Summary
Cholangiocarcinoma is a highly malignant tumor and the
The mean survival for unresectable lesions is 6~12 months. Surgical resection is the only treatment modality that offer
coordinate pain management, infectious complications, biliary drain management, and terminal care.
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