Hepatic angiosarcoma

Dr Henry Knipe et al.

Hepatic angiosarcoma is a rare malignancy but is still the third most common primary liver tumour. 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 tumours. Most commonly occurs in patients in their 60-70s 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. Patients can also present with spontaneous rupture and haemoperitoneum 1,5

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

Most arise spontaneously, although haemochromatosis and neurofibromatosis type 1 have also been associated.

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

Histology demonstrates varied appearance 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 haemorrhage) on both non-contrast and contrast-enhanced CT
  • nodular enhancement is common

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

  • T1/T2: heterogeneous areas of high signal reflecting mixed tumour and haemorrhage
  • T1 C+ (Gd): heterogeneous enhancement with progressive filling
  • 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 six months and survival beyond one year is rare.

Hepatobiliary pathology
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Article information

rID: 32689
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Angiosarcoma of the liver
  • Hepatic angiosarcomas
  • Kupffer-cell sarcoma
  • Angiosarcomas of the liver
  • Liver angiosarcoma
  • Liver angiosarcomas

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    Case 1a: CT
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    Case 1b: MRI
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