cholangiocarinoma Flashcards
what is a cholangiocarcinoma and where do they commonly occur?
cancer of the biliary system
can occur anywhere in the biliary tree, but usually in the extrahepatic biliary system
most common side is the bifurcation of R and L hepatic ducts (called Klatskin tumours)
what are the features of cholangiocarcinomas?
slow growing
invade locally and metastases to local lymph nodes before spreading distally
most are adenocarcinomas arising from cholangiocytes
the remaining are SCC and sarcomas, lymphomas and small cell cancers
what are the risk factors for cholangiocarciomas?
- primary schelrosing cholangitis
- UC
- infective (liver flukes, HIV, hepatitis virus)
- Toxins
- congenital
- alcohol excess
- DM
what are the clinical features of cholangiocarcinomas?
generally asymptomatic until late stage
symptoms
- post hepatic jaundice
- pruritus
- pale stools
- dark urine
- RUQ pain
- early satiety
- weight loss
- anorexia
- malaise
- cachexia
what is courvoisiers law?
in the presence of jaundice, an enlarged gallbladder is unlikely to be due to gallstones; rather carcinoma of the pancreas or the lower biliary tree is more likely.
This may be explained by the observation that the gallbladder with stones is usually chronically fibrosed and so, incapable of enlargement.
what are the differentials for cholangiocarcinomas?
include the causes of post hepatic jaundice
- gallstones
- bile duct strictures
- choledochal cysts
- external compression from extra biliary tumour
- benign biliary tumours
- pancreatic tumours
- primary biliary cirrhosis
- primary sclerosing cholangitis
what lab tests are done for cholangiocarcinomas?
biochemical investigations for obstructive jaundice (raised bilirubin, ALP and ɣGT).
tumour markers CEA and CA19-9 may also be raised
what radiological investigations can be done for cholangiocarcinomas?
- USS initially to confirm obstructive cause
- MRCP is the optimal for imaging and diagnosis
- ERCP to show site of obstruction and to obtain samples for cytology/histology
- stage via CT imaging and locate distant metastases
how is a cholangiocarinoma managed surgically ?
definitive cure is complete surgical resection, however around 1/2 have recurrence within 5yrs
mainly inoperable
intrahepatic or klatskin tumours need a partial hepatectomy and reconstruction of the biliary tree
distal common duct tumours need pancreaticoduodenectomy (whipples procedure)
radiotherapy may be used as adjunct or neoadjunct
how is cholangiocarcinoma managed palliatively?
- stenting with ERCP to relieve obstructive symptoms (however metal sends need replacing every 3-4 months)
- surgery if stent isn’t relieving
- medical includes pallative radiotherapy. Chemotherapy can be used in combination to slow tumour growth
what are the complications of cholangiocarcinomas?
- biliary tract sepsis
- biliary cirrhosis
long term survival is generally poor and treatment should be aimed at good symptom relieving management
