CURRENT MANAGEMENT OF BILE DUCT CANCER. HOW IT CAN BE TREATED??

Cholangiocarcinoma or bile duct cancer: how to Manage???

Cholangiocarcinoma or bile duct cancer is a cancer of bile duct. In the past it was considered as untreatable but now due to improvement in surgical techniques, it can be cured if it is diagnosed and treated in time.

Most patients are diagnosed after the age of 65, with a peak incidence occurring during the eighth decade of life. Unlike gallbladder cancer, men appear to have cholangiocarcinoma slightly more frequently than women. Known risk factors include

·      primary sclerosing cholangitis

·       choledochal cyst disease

·      chronic biliary parasitic infestation

·      and numerous chemicals, including thorotrast, asbestos, dioxin, and nitrosamines.

Bile duct cancers are usually of three types:

1.     Intrahepatic or those who arise from bile ducts insider the liver.

2.     Hilar cholangiocarcinoma or klatskin tumor which arise generally from the junction of right and left sided bile ducts.

3.     Lower bile duct cancer.

Out of these 3 types hilar cholangiocarcinoma are most common and most difficult to treat and today we will discuss mostly hilar cholangiocarcinoma

For hilar cholangiocarcinoma most commonly following classification system suggested by bismuth is used.

Type 1; Tumor below junction of right and left bile duct

Type 2: Tumor at the junction of right and left bile duct but not involvement them

Type 3 A: Tumor at the junction of right and left bile duct plus involving right

Type 3B: Tumor at the junction of right and left plus involving left

Type 4L tumor involving right and left bile duct as well as junction

Presentation or clinical features:

Symptoms associated with intrahepatic cholangiocarcinomas are nonspecific, including malaise and abdominal pain. Unlike hilar and distal cholangiocarcinomas, a minority of patients develop jaundice. Hilar and distal cholangiocarcinomas can present with nonspecific symptoms of pain, anorexia, and weight loss. Itching is a common symptom for patients with extrahepatic cholangiocarcinoma, and it typically precedes clinically apparent jaundice.

It is jaundice or the presence of abnormal liver enzymes that usually prompts medical attention.

The level of jaundice can be informative in distinguishing benign from malignant biliary obstruction; benign causes of obstructive jaundice typically produce bilirubin levels ranging from 2 to 4 mg/dL (rarely exceeding 15 mg/dL), whereas biliary obstruction from cholangiocarcinoma usually results in serum bilirubin levels greater than 10 mg/dL (with a mean level of approximately 18 mg/dL).

Diagnosis:

Apart from abovementioned clinical features diagnosis is usually achieved by imaging modalities suc as CT scan  and MRI with MRCP. Some times endoscopic ultrasound or ERCP are necessary.

Preoperative MRCP:

Treatment:

Surgical treatment is the only hope for cure in cases of cholangiocarcinoma.

ALL the cholangiocarcinoma except mentioned in following table are operable.

Criteria for Unresectability for Cholangiocarcinoma

Medical contraindication to surgical intervention

Advanced cirrhosis/portal hypertension

Bilateral second-order biliary

Main portal vein involvement

Liver Lobar atrophy with other side second-order biliary radicle involvement

 Liver Lobar atrophy with other side portal vein involvement

Advanced lymphnode involvement

Distant spread.

Surgery:

The goal of surgical therapy for cholangiocarcinoma is complete R0 resection. Complete resection has consistently proven to correlate well with survival.

Hilar Cholangiocarcinoma:

partial liver resection is often necessary in addition to extrahepatic biliary excision for complete resection of extrahepatic cholangiocarcinoma.  Review of the relevant literature suggests that the rate of negative-margin resection (no cancer remaining in the body) closely approximates the frequency with which liver resection is performed. That means that chances of completely removing the cancer increase when liver resection done along with cholangiocarcinoma.The proximity of the caudate lobe of liver to the cancer often mandates concomitant caudate lobe removal; this is particularly evident for left-sided hilar tumors, as the major caudate lobe bile ducts drain into the left hepatic bile duct.

Patients undergoing resection exhibited an overall median survival of 35 months; predictors of improved survival were

·      well-differentiated tumors (good cancer biology)

·      negative resection margin (no cancer remaining)

·      the performance of a concomitant liver resection.

The importance of obtaining negative resection margins is underscored by the observation that patients with histologically positive margins (microscopic cancer inside the body ) of resection demonstrated poor survival outcomes indistinguishable from those with locally advanced tumors undergoing operative exploration without attempted resection.

It appears that the performance of liver resection at the time of resection of hilar cholangiocarcinoma is critical for optimizing outcome.

 Indeed, the 5-year actuarial survival among those patients undergoing partial liver resection was 37%, compared with 0% for those treated with bile duct excision alone.

Liver Transplant

Orthotopic liver transplant for cholangiocarcinoma, often done for patients with underlying primary sclerosing cholangitis, has traditionally been associated with suboptimal survival outcomes. Recently, the Mayo Clinic has demonstrated promising results among a select cohort of patients undergoing neoadjuvant chemoradiation followed by cadaveric or living donor liver transplant.

Role of preoperative biliary drainage:

For optimal results and to safely perform major liver resection particularly in cases of cholangiocarcinoma preoperative biliary drainage should be done to decrease bilirubin level less than 5.

Because when bilirubin is higher than than it means that liver is not functioning properly so there are increase chances of infection and post operative liver failure in this group of patients.

Preoperative biliary drainage:

Management of cholangiocarcinoma according to bismuth classification:

Intra Hepatic+ Upper third

Cholangiocarcinomas

without vascular involvement

Type I, II, IIIA

Extended right lobe liver resection with

Caudate lobe

Type III B

Left /Extended left liver resection

preferably with caudate resection

For TYPE IV consider liver transplantation according to mayo protocol

Intra Hepatic+ Upper Third

Cholangiocarcinomas

with vascular involvement :

Liver Resection with Blood vessels resection and reconstruction (if artery/vein inseparable from tumor and achieves margin –ve resection)

Initially it was thought that if tumor involves vessels surgical treatment should not be offered but now it has been proven that with vessles involvement also surgical resection can be offered. With these approaches we can achieve 5 year survival of around 40-50% in these advanced cases also.

Details mentioned in subsequent figures.

Extended right hepatectomy

Here I am posting some pics of extended right liver resection with vascular reconstruction to give you some idea how extensive surgical approach can save life.