Fact-sheet: Hepatic angiosarcoma


Updated on 11/15/2024 at 5:15 PM

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Definition

Rare hepatic vascular tumor (2% of primary malignant liver tumors), arising from endothelial cells of hepatic blood vessels

Exposure to carcinogenic factors in 25% of cases:

  • vinyl chloride monomer (VCM)
  • inorganic arsenic
  • thorium dioxide (Thorotrast)
  • anabolic steroids, radiation, estrogens,
  • oral contraceptives

Clinical features

Annual incidence: 0.01-0.025/100,000
7 to 16 new cases/year in France
Men > 60 years

When it becomes symptomatic, it may present with:

  • Hepatomegaly
  • Ascites
  • Right upper quadrant pain
  • Hemoperitoneum
  • Splenomegaly
  • Heart failure (due to arteriovenous shunts)
  • Early metastases (spleen, lung, bone)

Rapid clinical course with early metastatic spread: spleen ++, peritoneum, bone, lungs
Poor prognosis, mean survival of 6 months

Laboratory findings

Laboratory findings: normal alpha-fetoprotein, hemolytic anemia, thrombocytopenia, DIC
Histological diagnosis is difficult, liver biopsy is theoretically contraindicated:

  • Well-defined, non-encapsulated nodules
  • Malignant endothelial cells along the sinusoids,
    portal branches, and hepatic venules
  • Intratumoral vascular cavities

Ultrasound

Ultrasound may reveal:

  • Dominant tumor: hyperechoic, heterogeneous, often ≥ 5 cm.
  • Possible secondary lesions.

CT

Nonspecific imaging findings

  • Nodular lesion(s) or infiltrative mass
  • Calcifications may be present
  • Progressive enhancement (vascular lakes)
  • Enhancement beginning in the arterial phase, sometimes nodular, followed by progressive centrifugal enhancement in the portal and delayed phases, or centripetal enhancement
  • Possible spontaneously hyperdense areas of hemorrhagic origin
  • Possible perfusion heterogeneity outside the macroscopic tumor areas
  • Hemoperitoneum in case of spontaneous rupture

MRI

• Lesions: Nodular and multifocal, hypointense on T1, hyperintense on T2, heterogeneous.

• Enhancement: Persistent early peripheral enhancement, with possible focal annular and/or central enhancement. Predominantly centripetal pattern.

• Intratumoral T1 hyperintensities: Suggestive of hemorrhage.

• Dominant lesion: Heterogeneous or septated appearance. Possible fluid-fluid levels.

• Possible hypovascular nodules.

Differential diagnosis

  • Hepatic hemangioma
  • Other malignant liver tumors (Hepatocellular carcinoma, liver metastases)
  • Hepatic adenoma