Cholangiocarcinoma Flashcards
What is cholangiocarcinoma?
Cholangiocarcinoma refers to cancer of the biliary system. It can occur at any site along the biliary tree, although predominantly arises in the extrahepatic biliary system.
Where does cholangiocarcinoma commonfly affect?
The most common site for bile duct cancers is at the bifurcation of the right and left hepatic ducts (termed Klatskin tumours).
They are typically slow-growing tumours that invade locally and metastases to local lymph nodes, before spreading distally to the peritoneal cavity, lung, and liver.
Why is there higher incidence of cholangiocarcinoma in South-East Asia?
There is a higher incidence in South-East Asia due to the association with chronic endemic parasitic infections from liver flukes.
Briefly describe the histology of cholangiocarcinomas
Histologically, 95% of cholangiocarcinomas are adenocarcinomas arising from cholangiocytes within the biliary tree.
The remaining majority are squamous cell carcinomas, with other rarer types of bile duct cancers include sarcomas, lymphomas, and small cell cancers.
What are the risk factors for cholangiocarcinoma?
The main risk factors for cholangiocarcinoma include:
- Primary sclerosing cholangitis (lifetime risk of 10-20%)
- Ulcerative colitis
- Infective (Liver flukes, HIV, hepatitis virus)
- Toxins (Chemicals in rubber and aircraft industry)
- Congenital (Caroli’s disease, choledochal cyst)
- Alcohol excess
- Diabetes mellitus
Briefly describe the anatomy of the biliary tree
What are the clinical features of cholangiocarcinoma?
Cholangiocarcinoma (both intra- and extrahepatic) is generally asymptomatic until a late stage in the disease.
Symptoms include post-hepatic jaundice and pruritus, with pale stools and dark urine. Other less common features include RUQ pain, early satiety, weight loss, anorexia and malaise.
On examination, jaundice and cachexia are often evident. Courvoisier’s law can be applied in clinical assessment.
What is Courvoisier’s law?
Courvoisier’s law states that in the presence of jaundice and an enlarged or palpable gallbladder, malignancy of the biliary tree or pancreas should be strongly suspected as the cause is unlikely to be gallstones. This sign may be present only if the obstructing tumour is distal to the cystic duct.
What investigations should be ordered for cholangiocarcinoma?
Note: laboratory investigations
Biochemical investigations will confirm an obstructive jaundice (elevated bilirubin, ALP and ɣGT). The tumour markers CEA and CA19-9 may also be elevated.
What investigations should be ordered for cholangiocarcinoma?
Note: imaging
Ultrasound scanning may be used initially to confirm an obstructive cause. MRCP is the optimal imaging for diagnosing cholangiocarcinoma; ERCP may be used to demonstrate the site of obstruction and also has the ability to obtain samples for cytology/histology.
Staging of the disease is best done via CT imaging; it is superior to MRI scanning for locating distant metastases and can further evaluate the level of biliary obstruction. Angiography may be used in pre-operative planning to image the hepatic arteries or portal vein.
Briefly describe the surgical managment for cholangiocarcinoma
The definitive cure for cholangiocarcinoma is complete surgical resection. However, the majority of patients have inoperable disease at the time of presentation, with only 10-15% suitable for surgical intervention.
Intrahepatic or Klatskin tumours require a partial hepatectomy and reconstruction of the biliary tree. Patients with distal common duct tumours require a pancreaticoduodenectomy (termed a Whipple’s procedure).
Radiotherapy may be used in some cases as adjunct or neoadjunct therapy. There is limited evidence at present for the use of post-operative chemotherapy.
Briefly describe the palliative management of cholangiocarcinoma
Most cases will end up only having palliative management. The palliative treatment options in cholangiocarcinoma include:
- Stenting
- Surgery
- Medical
What is the role of stenting in cholangiocarcinoma treatment?
Note: palliative
ERCP may be used to stent the bile duct and relieve obstructive symptoms.
Self-expanding metal stents offer the best outcome but are prone to occlusion and may need replacing frequently (every 3-4 months).
What is the role of surgery in cholangiocarcinoma treatment?
Note: palliative
Surgical bypass procedures may be required if the obstruction cannot be relieved by stenting.
What is the role of medical treatment in cholangiocarcinoma?
Note: palliative
Palliative radiotherapy may be used to prolong survival.
A combination of chemotherapy agents (e.g. cisplatin and gemcitabine) can also be used to slow tumour growth, however when used without radiotherapy, no significant survival benefit is seen.
