Liver & intrahepatic bile ducts

Other malignancies


Last author update: 1 February 2012
Last staff update: 8 July 2022

Copyright: 2002-2024,, Inc.

PubMed Search: Angiosarcoma liver

Deepali Jain, M.D.
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Cite this page: Jain D. Angiosarcoma. website. Accessed May 19th, 2024.
Definition / general
  • Rare (10 - 30 annual cases in U.S.) but most common hepatic primary sarcoma in adults (2% of all primary liver tumors)
  • 25 - 42% associated with exposure to androgen steroids, arsenic, Thorotrast (radiocontrast agent thorium dioxide, used through 1950s, detect its α particle emissions via autoradiography), vinyl chloride (Wikipedia: Thorotrast [Accessed 30 November 2017])
  • Patients with exposure to Thorotrast or vinyl chloride may have synchronous cholangiocarcinoma or hepatocellular carcinoma
  • Rarely associated with chemotherapy, copper sulfate, estrogens, hereditary hemochromatosis, phenelzine, radiotherapy
  • Cases with above known causes usually have latent period of 20 - 35 years, are accompanied by fibrosis or cirrhosis, have precursor conditions of hypertrophy and atypia of hepatocytes and sinusoidal lining cells but are histologically similar to idiopathic cases
Clinical features
  • 75% men, usually age 50+ years; rare in children
  • Nonoperative biopsy may cause severe bleeding and death
  • Most patients die within 6 months from hepatic failure or intra-abdominal bleeding
  • Metastasizes widely, often to lung (vinyl chloride cases usually don't have distant metastases)
Case reports
Gross description
  • Multicentric, involves right and left lobes
  • Diffusely infiltrative, hemorrhagic and gray white solid nodules with blood filled cavities
  • Thorotrast associated tumors have subcapsular hepatic and splenic deposits of yellow chalky material
Gross images

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Vascular lesion with dense fibrosis

Microscopic (histologic) description
  • Tumor composed of infiltrative, freely anastomosing vascular channels
  • Tumor cells grow along sinusoids adjacent to hepatic cords
  • Tumor cells have abundant, pale eosinophilic cytoplasm, poorly defined cell borders, are usually pleomorphic with hyperchromatic nuclei but may be only mildly atypical
  • Also variably prominent nucleoli, blood filled cavities present are lined by tumor cells that may be papillary
  • 75% have vascular invasion of portal or hepatic vein branches; frequent mitotic activity
  • Precursor stage has endothelial hypertrophy and hyperplasia
  • Also epithelioid cells with abundant cytoplasm and prominent nucleoli, bizarre tumor giant cells, fibrosarcoma-like spindle cells, cholestatic hepatocellular rosettes with bile plugs, tumor cell phagocytic activity and extramedullary hematopoiesis
  • Childhood cases: may have kaposiform areas of spindle cells with PAS+ intracytoplasmic globules; no prominent myxoid areas
  • Thorotrast exposed patients: have brown gray refractile but not birefringent granules of Thorotrast free or within macrophages
  • Also precursor stage with endothelial hypertrophy and hyperplasia
Microscopic (histologic) images

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Sinusoidal infiltration

Positive stains
Negative stains
  • Keratin (but positive in 12 - 35%)
Electron microscopy description
  • Weibel-Palade bodies
Molecular / cytogenetics description
  • 50% of vinyl chloride associated cases have A:T to T:A transversion in p53
Differential diagnosis
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