Hepatic angiosarcoma

Last revised by Joshua Yap on 26 May 2023

Hepatic angiosarcoma is a rare malignancy but is still the third most common primary liver tumor. They have a variable appearance on both CT and MRI, reflecting the pleomorphic histological nature. Prognosis is very poor, with survival uncommon beyond one year from diagnosis. 

Hepatic angiosarcoma was previously known as Kupffer cell sarcoma. 

Hepatic angiosarcomas account for ~1% of primary liver tumors. They most commonly occur in patients 60-70 years of age with a male predominance (M:F = 4:1) 1-3

Patients typically have non-specific abdominal symptoms and may present with an abdominal mass or hepatomegaly. They can also present with spontaneous rupture and hemoperitoneum 1,5

Hepatic angiosarcomas arise from malignant spindle cells of endothelial origin. Metastases are common at presentation, affecting lung and spleen.

Most hepatic angiosarcomas arise spontaneously, although hemochromatosis and neurofibromatosis type 1 have also been associated.

Environmental exposure to Thorotrast, arsenic, ionizing radiation, and vinyl chloride have been implicated as risk factors, but such exposure is now rare.

Histology demonstrates varied appearances of solid nodules/masses or as sinusoidal or cavernous spaces. 

Hepatic angiosarcomas most commonly present as multiple masses but can occur as a single heterogeneous mass 2,4

  • hypoattenuating masses (some may be hyperattenuating reflecting hemorrhage) on both non-contrast and contrast-enhanced CT

  • nodular enhancement is common

Findings reflect the hemorrhagic nature of angiosarcomas with fluid-fluid levels often a feature 2

  • T1/T2: heterogeneous areas of high signal reflecting mixed tumor and hemorrhage

  • T1 C+ (Gd): heterogeneous enhancement with progressive filling

The tumors are avid on FDG PET-CT 5.

It typically rapidly progresses with metastases and high recurrence rate although partial liver resection may be successful if it is confined to one lobe of the liver. 

Prognosis is poor with hepatic angiosarcomas being resistant to chemotherapy and radiation therapy. Median survival is less than 6 months and survival beyond 1 year is rare.

ADVERTISEMENT: Supporters see fewer/no ads

Cases and figures

  • Case 1a: on CT
    Drag here to reorder.
  • Case 1b: on MRI
    Drag here to reorder.
  • Case 2
    Drag here to reorder.
  • Updating… Please wait.

     Unable to process the form. Check for errors and try again.

     Thank you for updating your details.