Hepatic angiosarcoma

Hepatic angiosarcoma is a rare, aggressive malignancy arising from the liver endothelium, predominantly affecting men over 60 years of age. While often idiopathic, it is associated with environmental exposures such as vinyl chloride and arsenic. Individuals present with nonspecific abdominal symptoms or acutely with tumor rupture and intraperitoneal hemorrhage. Diagnosis relies on imaging confirmed with histopathology findings. Management is often limited by advanced disease at the time of diagnosis. Surgical resection offers the best chance for survival but is only possible in a minority of cases. Liver transplantation is contraindicated due to high recurrence rates. For unresectable disease, treatment is palliative and may include transarterial embolization and chemotherapy. The overall prognosis is poor, with a median survival of approximately 6 months.

• Prevalence: 1–2% of liver tumors ^[1]
• Age: typically occurs in individuals > 60 years of age (median 57 years) ^[1]
• Sex: ♂ > ♀ (3:1) ^[2]

Epidemiological data refers to the US, unless otherwise specified.

• Environmental and iatrogenic exposures ^[1][2]
  • Vinyl chloride ^[2]
  • Thorium dioxide
  • Arsenic
  • Anabolic steroids
  • Radiation
  • Exogenous estrogens
• Idiopathic ^[1]

Hepatic angiosarcoma may manifest with nonspecific abdominal symptoms or acutely with complications. ^[1][2]

• General findings: jaundice, anemia
• Abdominal findings: pain, ascites, hepatosplenomegaly
• Constitutional symptoms: weight loss, fatigue
• Signs of complications: e.g., signs of peritonitis and/or hemorrhagic shock in cases of intraperitoneal hemorrhage

Imaging ^[2][3]

On imaging, hepatic angiosarcoma appears as a single heterogeneous mass, multiple masses, or infiltrative lesions.

• Contrast-enhanced CT
  • Lesions are typically hypoenhancing on postcontrast phases.
  • Hyperdense areas on noncontrast phases if hemorrhage is present
• MRI: heterogeneous lesions with signs of hemorrhage

Biopsy ^[1][2]

• Required for definitive diagnosis
• Laparoscopic or open biopsy is preferred. ^[1]
• Histopathologic findings are heterogeneous.
• Immunohistochemistry: positive for endothelial markers, e.g.,
  • Erythroblast transformation specific-related gene (ERG)
  • PECAM-1 (CD31)
  • CD34

• Refer patients to specialized centers with multidisciplinary expertise in sarcoma management. ^[3]
• Surgical resection, if possible, is associated with improved median overall survival. ^[1]
• Liver transplantation is contraindicated because of high recurrence rates and poor survival outcomes.
• Management of unresectable disease ^[1][2]
  • Symptom control (see "Overview of palliative medicine")
  • Transarterial embolization for tumor bleeding
  • Chemotherapy may be considered, but there are no standard approved regimens.

• Metastases ^[2]
• Tumor rupture with intraperitoneal hemorrhage
• Acute liver failure

We list the most important complications. The selection is not exhaustive.

• Median overall survival: ∼ 6 months ^[2]
• Metastases are often present at the time of diagnosis.
• High recurrence rate following resection
• Poor prognostic factors: older age, large tumor size, high Ki-67 index ^[2]