Hepatic Tumor, Uncommon Primary

• • Angiosarcoma

  • Hepatoblastoma

  • Hepatic adenocarcinoma

  • Intrahepatic cholangiocarcinoma

Epidemiology

• • 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

Symptoms and Signs

• • Right upper quadrant pain

  • Weight loss

  • Hepatomegaly

  • Abdominal mass in childhood

  • Hepatic bruit

  • Ascites

  • Distant metastases

  • Asymptomatic

  • Intraperitoneal hemorrhage

  • Decompensated cirrhosis

Laboratory Findings

• • Hyperbilirubinemia

  • Elevated transaminases

  • Elevated alkaline phosphatase

Imaging Findings

• • CT scan

  • -Hypervascular tumor (for angiosarcoma)

    -Multi-septate cyst for adenocarcinoma

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

  • Overall and planned reserve liver function

Rule Out

• • Metastatic carcinoma

  • Benign neoplasms if possible

  • Other abdominal malignancy

• • CT scan

  • US

  • Angiography

  • Biopsy if unresectable

  • Resection for diagnosis and treatment

When to Admit

• • Intrahepatic hemorrhage

  • Decompensated cirrhosis

Surgery

• • Resection

Indications

• • Localized tumor

Contraindications

• • Poor liver function

  • Extrahepatic disease

  • Diffuse hepatic disease

Medications

• • Chemotherapy for unresectable lesions

Treatment Monitoring

• • CT surveillance

Complications

• • Hemorrhage

  • Biliary leak

  • Abdominal infection

  • Liver failure

Prognosis

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

  • Angiosarcoma: Very poor

  • Hepatoblastoma: Overall survival 50%

References

Practice Guidelines

• • The National Comprehensive Cancer Network

  • http://www.nccn.org/