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  • • Angiosarcoma

    • Hepatoblastoma

    • Hepatic adenocarcinoma

    • Intrahepatic cholangiocarcinoma

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Epidemiology

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  • • Hepatoblastoma mainly in children

    • Frequency increased in Beckwith-Wiedemann syndrome hemihypertrophy, fetal alcohol syndrome, and parenteral nutrition

    • Angiosarcoma and sarcoma risk factors

    • -Vinyl chloride

      -Arsenic

      -Androgenic steroids

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Symptoms and Signs

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  • • Right upper quadrant pain

    • Weight loss

    • Hepatomegaly

    • Abdominal mass in childhood

    • Hepatic bruit

    • Ascites

    • Distant metastases

    • Asymptomatic

    • Intraperitoneal hemorrhage

    • Decompensated cirrhosis

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Laboratory Findings

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  • • Hyperbilirubinemia

    • Elevated transaminases

    • Elevated alkaline phosphatase

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Imaging Findings

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  • CT scan

    • -Hypervascular tumor (for angiosarcoma)

      -Multi-septate cyst for adenocarcinoma

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  • • Biopsy of limited usefulness given high degree of sampling error and risk of tumor seeding

    • Overall and planned reserve liver function

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Rule Out

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  • • Metastatic carcinoma

    • Benign neoplasms if possible

    • Other abdominal malignancy

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  • • CT scan

    • US

    • Angiography

    • Biopsy if unresectable

    • Resection for diagnosis and treatment

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When to Admit

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  • • Intrahepatic hemorrhage

    • Decompensated cirrhosis

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Surgery

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  • • Resection

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Indications

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  • • Localized tumor

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Contraindications

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  • • Poor liver function

    • Extrahepatic disease

    • Diffuse hepatic disease

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Medications

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  • • Chemotherapy for unresectable lesions

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Treatment Monitoring

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  • • CT surveillance

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Complications

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  • • Hemorrhage

    • Biliary leak

    • Abdominal infection

    • Liver failure

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Prognosis

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  • Resectable cholangiocarcinoma: 5-year survival, 15-20%

    Angiosarcoma: Very poor

    Hepatoblastoma: Overall survival 50%

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References

Endo I et al: Intrahepatic cholangiocarcinoma: rising frequency, improved survival, and determinants of survival after resection. Ann Surg 2008; 247:994.
Patel T: Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology. 2001;33:1353.  [PubMed: 11391522]

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Practice Guidelines

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