Mazen Hassanain
Dec 27, 2015
Bile duct CancerAverage age 60 years
Ulcerative colitis is a common associated condition
Subtypes: (1) periductal infiltrating, (2) papillary or intraductal, and (3) mass forming-nodular
Location: 85% extrahepatic
Risk FactorsDefinite risk factors
Primary sclerosing cholangitis (1% per year) Liver fluke infection (Opisthorchis viverrini) Hepatolithiasis (10%) Biliary malformation (10% choledochal cysts,
Caroli's) Thorotrast
Probable risk factors Hepatitis C Cirrhosis Toxins (dioxin, polyvinyl chloride) Biliary-enteric drainage procedures
T1: Tumor involving biliary confluence ± unilateral extension to 2° biliary radicles
T2 Tumor involving biliary confluence ± unilateral extension to 2° biliary radicles AND Ipsilateral portal vein involvement ± ipsilateral hepatic lobe atrophy
T3 Tumor involving biliary confluence + bilateral extension to 2° biliary radicles OR Unilateral extension to 2° biliary radicles
with contralateral portal vein involvement OR Unilateral extension to 2° biliary radicles
with contralateral hepatic lobe atrophy OR Main or bilateral portal venous involvement
DiagnosisBlood work
CA19-9: Its sensitivity and specificity for detection of CCA in PSC are 79% and 98%, respectively, at a cutoff value of 129 U/mL.
Imaging (US, CT, MRI/MRCP, ERCP, PTC, EUS, PET/CT)
Treatment and prognosisSurgical resection Adjuvant and neoadjuvant treatmentsMayo Protocol
The average patient with adenocarcinoma of the bile duct survives less than a year. The overall 5-year survival rate is 15%.
Following a thorough radical operation, 5-year survival is about 40%. Biliary cirrhosis
Surgery
Local lymph node metastases (N1) are not an absolute contraindication to surgical treatment, because they do not significantly influence outcomes in hilar CCA
GB cancerPredominantly in the elderly
Incidentally diagnosed at an early stage after cholecystectomy for cholelithiasis (1%)
Approximately 90% of patients have gallstones. The 20-year risk of developing cancer for
patients with gallstones is less than 0.5% for the overall population and 1.5% for high-risk groups
Risk FactorsLarger stones (3 cm) tenfold increased risk
The risk is higher in patients with symptomatic pts
Polypoid lesions, particularly in polyps >10mm
The calcified "porcelain" gallbladder (20%) selective mucosal calcification (7%)
Choledochal cysts have an increased risk of developing cancer anywhere in the biliary tree, but the incidence is highest in the gallbladder.
Other Risk FactorsAnomalous pancreatobiliary duct junction Obesity and pregnancyChronic inflammatory bowel disease Polyposis coli Mirizzi syndrome Bacterial and Salmonella infections Industrial exposure to carcinogens Familial tendency
PathologyAdenocarcinomas 90% .
Squamous cell, adenosquamous, oat cell, …
Papillary (10%), nodular, and tubular
Lymphatics are present in the subserosal layer only. Therefore cancers invading but growing through the muscular layer have minimal risk of nodal disease
40% have distant metastasis at Dx
PresentationAbdominal discomfort, right upper quadrant
pain, nausea, and vomiting.
Jaundice, weight loss, anorexia, ascites, and mass
Blood work
Imaging (UD, CT, MRI/MRCP, ERCP, PTC, PET/CT)
AJCC stagingStage 0: Carcinoma in situ Stage I: T1/2 N0 M0: invades lamina propria,
muscle layer, perimuscular connective tissue Stage II: T3 N0/1 M0 T3: perforates the
serosa and/or directly invades the liver and/or one adjacent organ
Stage III: T4: invades any main vesselStage IV: M1: distant metastases, including
metastases in lymph nodes at the pancreatic body and tail
Treatment and prognosisSurgeryAdjuvant therapyThe 5-year survival rate of all patients is less than
5%, median survival of 6 months.T1 treated with cholecystectomy 90% 5-year
survival T2 lesions treated with an extended
cholecystectomy and lymphadenectomy is over 70%
Advanced but resectable gallbladder cancer are reported to have 5-year survival rates of 20 to 50%.