Diagnostic Ultrasound - Abdomen and Pelvis

Cholangiocarcinoma
Diagnoses: Biliary System
Choledocholithiasis
• Intra- and extrahepatic bile duct stones with ductal
dilatation
• Echogenic filling defects with posterior acoustic shadowing
• Biliary sludge of medium echoes mimic intraluminal form of
extrahepatic tumor
• May see ductal wall thickening due to superimposed
cholangitis
Recurrent Pyogenic Cholangitis
• Ductal wall thickening due to repeated and longstanding
cholangitis
• Intrahepatic biliary stones and echogenic biliary sludge
• Accompanies atrophy of involved hepatic segment &/or
biliary cirrhosis
• Present clinically with repeated episodes of acute
ascending cholangitis
Primary Sclerosing Cholangitis (PSC)
• Multiple strictures associated with beading and peripheral
pruning of bile ducts
• Bile duct wall thickening
• Risk for cholangiocarcinoma: 10% of PSC patients
Porta Hepatis Tumor
• Bulky primary tumor (e.g., hepatocellular carcinoma [HCC]),
lymph node metastasis, metastasis
• May invade or obstruct IHBDs and cause dilatation
PATHOLOGY
General Features
• Etiology
○ Biliary intraepithelial neoplasm is considered
premalignant lesion
○ Risk factors
– Primary sclerosing cholangitis
– Choledochal cyst
– Biliary papillomatosis
– Parasites (clonorchiasis, Opisthorchis)
– Recurrent pyogenic cholangitis
– Ulcerative colitis
– Chronic liver disease
– Thorotrast exposure
• Patterns of disease progression
○ Local extension along bile duct
○ Local infiltration of liver parenchyma
○ Spread to regional lymph nodes; porta hepatis,
paraceliac and peripancreatic lymph node (LN)
○ Perineural spread
○ Intraperitoneal seeding (peritoneal carcinomatosis)
○ Hematogenous spread
Gross Pathologic & Surgical Features
• Bismuth-Corlette classification of Klatskin tumor
○ I: Tumor involving CHD below confluence
○ II: Tumor involving CHD reaching confluence
○ IIIa: Tumor involving right 2nd-order branch
○ IIIb: Tumor involving left 2nd-order branch
○ IV: Tumor involving both 2nd-order branches
Microscopic Features
• Sclerosing type: Stricture, most common type, worst
prognosis
• Nodular type: Exophytic intrahepatic masses
• Papillary type: Intraluminal polypoid lesions, least common
type, best prognosis
CLINICAL ISSUES
Presentation
• Most common signs/symptoms
○ Painless obstructive jaundice, pain, palpable mass,
weight loss
• Lab data:↑ bilirubin/alkaline phosphatase,↑ CEA/CA19-9
Demographics
• Age: Peak at 6th-7th decade
• Gender: M:F = 3:2
• Epidemiology
○ More common in Asia
○ Causes 15-33% of primary hepatobiliary cancers
○ 2nd most common primary hepatic tumor (after HCC)
Natural History & Prognosis
• 5-year survival rate: 5-10%
• Resectability rate: Distal 91%, hilar 56%, peripheral 60%
Treatment
• Surgical resection (only potentially curative method) +
adjuvant chemoradiation
• Palliative treatment: Chemotherapy, radiation; laser
therapy; biliary stenting
DIAGNOSTIC CHECKLIST
Consider
• Rule out other biliary & pancreatic pathologies that can
mimic cholangiocarcinoma by obstructing extrahepatic bile
duct
Image Interpretation Pearls
• Long and rarely short focal extrahepatic biliary stricture,
irregular ductal wall, stenosis and prestenotic biliary ductal
dilatation
• Klatskin tumor: Small tumor at confluence of right/left
hepatic and proximal common hepatic ducts
SELECTED REFERENCES
1. Chung YE et al: Varying appearances of cholangiocarcinoma: radiologicpathologic
correlation. Radiographics. 29(3):683-700, 2009
2. Vilgrain V: Staging cholangiocarcinoma by imaging studies. HPB (Oxford).
10(2):106-9, 2008
3. Han JK et al: Cholangiocarcinoma: pictorial essay of CT and cholangiographic
findings. Radiographics. 22(1):173-87, 2002
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