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Cholangiocarcinoma

Cholangiocarcinoma is a cancer of the bile ducts with an incidence of 8 per million in the US. Risk factors include primary sclerosing cholangitis, liver flukes, congenital abnormalities, and chronic inflammation. Ductal cholangiocarcinoma presents with jaundice, abdominal pain, and weight loss while intrahepatic tumors often mimic liver metastases. Diagnosis relies on imaging, tumor markers like CA19-9, and biopsy. Surgery offers the only chance for cure but resection is often not possible due to late stage at presentation. Palliative options include stenting and chemotherapy though no treatment prolongs survival beyond 18 months.

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98 views

Cholangiocarcinoma

Cholangiocarcinoma is a cancer of the bile ducts with an incidence of 8 per million in the US. Risk factors include primary sclerosing cholangitis, liver flukes, congenital abnormalities, and chronic inflammation. Ductal cholangiocarcinoma presents with jaundice, abdominal pain, and weight loss while intrahepatic tumors often mimic liver metastases. Diagnosis relies on imaging, tumor markers like CA19-9, and biopsy. Surgery offers the only chance for cure but resection is often not possible due to late stage at presentation. Palliative options include stenting and chemotherapy though no treatment prolongs survival beyond 18 months.

Uploaded by

drpiratheepan
Copyright
© Attribution Non-Commercial (BY-NC)
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PDF, TXT or read online on Scribd
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NR 07-03

Cholangiocarcinoma
Gores, Gregory. “Cholangiocarcinoma: Current concepts and insights.” (2003) Hepatology 37(5): 961-9.
Kim, et al. “Clinical manifestations and diagnosis of cholangiocarcinoma.” UpToDate v11.2.
Chari et al. “Treatment of cholangiocarcinoma.” UpToDate v11.2.
Lowe et al. “Epidemiology, pathogenesis, and classification of cholangiocarcinoma.” UpToDate v11.2.
Escalante-Glorsky and Raijman. “Endoscopic methods for the diagnosis of biliary neoplasms.” UpToDate v11.2.

Key Points:
• Incidence is associated with inflammatory risk factors.
• Intrahepatic cholangiocellular carcinoma presents differently from ductal cholangiocarcinoma,
lacking obstructive symptoms / signs and mimicking hepatic metastases
• Diagnosis relies on combination of clinical scenario, imaging, tumor markers, and pathology,
given poor sensitivities and specificities of diagnostic tests.
• Surgery is the only modality with mortality benefit; stenting offers palliation.

Epidemiology
• U.S.: 8 per million, peak age 50-70
o Ductal: 2/3 (e.g., Klatskin tumors)
o Intrahepatic cholangiocellular: 1/3?
ƒ Misdiagnosed as metastatic adenocarcinomas
ƒ Associated with hepatitis C in Japan
• Risk factors:
o Primary sclerosing cholangitis (PSC): 1.5% per year, peak age 30-50, comprises 30% of
new dx cancers,
o Clonorchis sinensis and Opisthorchis viverrini
o Caroli’s disease (7%)
o Congenital choledochal cysts (up to 14%)
o Chronic intrahepatic lithiasis (2-10%): not cholelithiasis
• Mechanisms: iNOS, interleukin 6, COX-2, c-erB-2 tyrosine kinase, p53, hTERT (inflammation)

Clinical Presentation and Diagnosis


• Ductal:
o Obstruction: jaundice (90%), pruritus (66%), pale stools, dark urine
o Abdominal pain (30-50%), weight loss (30-50%), fever (20%), cholangitis
o Courvoisier’s sign (palpable gallbladder): rare
• Intrahepatic: abdominal pain, mass, anorexia, weight loss, night sweats, malaise, asymptomatic
o Jaundice is rare
• Imaging
o Ductal dilatation without stone: false negatives in PSC or cirrhosis
o Atrophy-hypertrophy complex
o MRCP
o PET [18F]2-deoxy-D-glucose: false-positives
• Laboratory tests (most studied in patients with PSC)
o Serum CA 19-9 ( > 100 U/mL): when cholangitis or cholestasis, diagnosis of malignancy
requires higher cut-off for specificity
o CEA: not sensitive or specific; possible role for combined index (CA 19-9 + CEAx40)
• ERCP / percutaneous transhepatic cholangiogram (PTC): diagnostic and therapeutic
o Biopsy and brushings: 30-70% sensitive, 90% specific
• Patients with PSC: serial CA 19-9, PET scan (MRI not useful)

Treatment
• Determined by:
o Local extent: distal > proximal
o Vascular involvement
NR 07-03

o Metastases
o Investigated by MRCP, endoscopic U/S (15-20% metastatic lymph nodes), laparoscopy
• Surgery:
o Complete resection: 40-60% 3-year survival
o Only for curative intent
o 10% operative mortality, mostly from infection – increased with stenting
o Possible benefit in post-operative hepatic function if bilirubin >3-10
o PSC: risk of hepatic decompensation
o Liver transplantation: role uncertain
• Palliative: survival <18 months
o Stenting
ƒ Only if not operative candidates
ƒ Metallic stents (longer) vs. plastic (adjustable)
o Chemotherapy / radiation: partial response rates with 5-FU and gemcitabine, but no
survival benefit
o Photodynamic therapy: hematoporphyrin derivative -> laser during ERCP

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