Biliary Tumors

Cards (19)

  • Benign biliary tumors
    • Adenomyomatosis of the gallbladder
    • Gallbladder polyps
    • Biliary Hamartoma
  • Malignant biliary tumors
    • Cholangiocarcinoma
    • Gallbladder carcinoma
  • Risk factors for biliary tumors
    • Chronic/ recurrent cholangitis
    • Chronic/ recurrent choledocholithiasis
    • Choledochal cysts
    • Primary sclerosing cholangitis
    • Liver cirrhosis
  • Cholangiocarcinoma
    Arises from cholangiocytes in biliary tree
  • Cholangiocarcinoma is an uncommon cancer, 1-2 per 100000 in US
  • Men are more affected by cholangiocarcinoma than females, ratio of 1.5 to 1
  • Majority of cholangiocarcinoma patients are older than 65, with peak incidence in the eighth decade of life
  • Classification of cholangiocarcinoma by site of origin
    • Hilar cholangiocarcinoma (60%)
    • Distal cholangiocarcinoma (20-30%)
    • Intrahepatic cholangiocarcinoma (10%)
  • Risk factors for cholangiocarcinoma
    • Primary sclerosing cholangitis (PSC)
    • Recurrent pyogenic cholangitis (hepatolithiasis)
    • Choledocholithiasis more than cholelithiasis
    • Caroli disease / choledochal cysts (time risk of 10-15%)
    • Asian liver flukes Opisthorchis viverrini
    • Clonorchis sinensis (clonorchiasis)
    • Viral infections: HIV, hepatitis B & C, EBV
    • Toxins: polyvinyl chloride, heavy alcohol use
  • Differential diagnosis of biliary stricture
    • Malignant: Cholangiocarcinoma, Peri-ampullary malignancy: pancreatic ductal adenocarcinoma; ampulla carcinoma
    • Benign: Iatrogenic strictures, diathermy burns, clips, previous anastomosis (e.g. post liver transplant), Mirizzi syndrome, Chronic pancreatitis, Auto-immune stricture: IgG4 disease, Primary sclerosing cholangitis, HIV cholangiopathy
  • Hilar cholangiocarcinoma (Klatskin tumor)

    Clinical presentation: Early - non-specific, i.e. abdominal pain, discomfort, anorexia, weight loss and/or pruritis in 1/3. Mostly - present with jaundice or incidentally discovered abnormal LFTs. Liver may be enlarged, Gallbladder not palpable, Cholangitis rare at initial presentation.
  • Imaging for biliary tumors
    1. Delineate the level and nature of any obstructing lesion causing jaundice
    2. Determine site of lesion and length of stenosis, as well as assessing resectability in the planning of possible surgical intervention
    3. Cholangiography is essential
    4. Magnetic resonance cholangiography is least invasive
    5. CT remains an important study to assess vascular involvement and liver atrophy
  • Pre-operative evaluation and assessment of resectability for cholangiocarcinoma

    1. Is the lesion resectable?
    2. Is the future liver remnant adequate?
    3. Is the patient operable?
  • Cholangiocarcinoma-specific non-resectable criteria
    • Patient factors: Medically unfit, Liver cirrhosis, Metastatic disease: Distant lymph node basins, Lung, liver or peritoneal mets
    • Local tumor-related: Bilateral extension, Encasement/occlusion main PV, Atrophy one hepatic lobe with contralateral PV branch encasement/occlusion, Atrophy one lobe with contralateral extension to secondary biliary radicles, Unilateral extension to secondary biliary radicles with contralateral PV encasement/ occlusion
  • Palliation for cholangiocarcinoma
    1. Biliary decompression
    2. Supportive care
  • Indications for biliary decompression
    • Intractable pruritis
    • Recurrent cholangitis
    • The need for access for intraluminal radiotherapy
  • Other palliative modalities
    • Radiation therapy
    • Photodynamic therapy
    • Chemotherapy
  • Distal cholangiocarcinomas present earlier with obstructive jaundice and are usually resectable
  • Surgical resection of distal cholangiocarcinomas is by pancreaticoduodenectomy (Whipple procedure)