Biliary Tract and Gallbladder Cancer

Question 1

What percentage of cholangiocarcinoma is intrahepatic? Perihilar? Distal extra hepatic?

Answer 1

5% - intrahepatic
65% - perihilar
30% - distal extrahepatic

Question 2

What is the typical age at diagnosis of cholangiocarcinoma?

Answer 2

50-70

*earlier if PSC and biliary cuts

Question 3

What are some risk factors for cholangiocarcinoma?

Answer 3

PSC
Biliary cysts
Biliary parasitosis (Clonorchis and Opisthorchis)
Thorotrast

Question 4

What do 70-80% of patients with primary sclerosing cholangitis have?

Answer 4

IBS – ulcerative colitis

Question 5

What is the typical appearance of PSC on histology?

Answer 5

“onion skinning” around bile ducts

Question 6

Biliary cyst: Type I

Answer 6

50-85%

Exttrahepatic only

Question 7

Biliary cyst: Type IV

Answer 7

15-35%
Multiple cysts
Extrahepatic +/- intrahepatic

Question 8

Biliary cyst: Type V

Answer 8

20%
Intrahepatic only
Caroli’s disease

Question 9

What types of biliary cysts predispose to cholangiocarcinoma?

Answer 9

Types I, II and IV

Question 10

What chronic parasite infections are associated with development wolf cholangiocarcinoma?

Answer 10

Clonorchis sinesis

Opisthorchiasis

Question 11

What is it called when you get cholangiocarcinoma at the bifurcation of bile ducts?

Answer 11

Klatskin tumor – patient will have jaundice, direct bilirubin will be elevated

Question 12

The majority of cholangiocarcinomas are…

Answer 12

Adenocarcinoma (90%)

Squamous cell carcinoma and others

Question 13

How will extrahepatic cholangiocarcinoma present versus intrahepatic cholangiocarcinoma?

Answer 13

Extrahepatic – get symptoms from biliary obstruction (jaundice, abdominal pain, pruritus, weight loss, fever; cholestatic liver test elevations

Intrahepatic – less likely to be jaundiced, abdominal pain, weight loss

Question 14

What are some diagnostics available for cholangiocarcinoma?

Answer 14

Carbonic anhydrase 19-9: limited value d/t false positives and negatives
Computed tomography
MRI
Endoscopic retrograde cholangio pancreatigraphy (ERCP) –noninvasive

Question 15

What is the treatment for cholangiocarcinoma?

Answer 15

Curative treatment = surgery
Liver transplant for hilar cholangiocarcinoma
Palliative = 5-10% 5 year survival

Question 16

Curative surgeries for different locations of cholangiocarcinoma…

Answer 16

Distal = Whipple procedure
Perihilar = bile duct resection, hepatic lobectomy
Intrahepatic = hepatic resection

Question 17

What is the most common type of gall bladder cancer?

Answer 17

Adenocarcinoma – 75% of cases
Uncommon and highly fatal
Women affected 2-6 times more than men

Question 18

Risk factors for gall bladder cancer

Answer 18

Porcelain gallbladder – incomplete calcification higher risk than complete calcification
Gallbladder polyps
Cholelithiasis (70-90% of patients with gallbladder cancer have cholelithiasis)

Question 19

How does one get a porelain gallbladder?

Answer 19

Chronic cholecystitis with intramural calcification of the gallbladder wall

Question 20

What are some types of gallbladder polyps?

Answer 20

Cholesterol
Inflammatory
Adenomas
*Likelihood of malignant transformation related to size

Question 21

What is the best diagnostic tool for the gallbladder and bile ducts?

Answer 21

Ultrasound

Question 22

What are the 4 ways gallbladder cancer typically presents?

Answer 22

1. Incidental on imaging
2. Found intraoperatively during cholecystectomy for presumed benign disease
3. Found incidentally on pathologic examination (most common)
4. Symptomatic: pain, anorexia, nausea, vomiting, jaundice from biliary obstruction

Question 23

What is the treatment for gallbladder cancer?

Answer 23

Surgery is the only option for cure
Advanced stage at diagnosis – poor prognosis
Can spread to other structures it is in close proximity with