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Liver and intrahepatic bile ducts - tumor

Other malignancies


Reviewers: Deepali Jain, M.D. (see Reviewers page)
Revised: 9 January 2013, last major update February 2012
Copyright: (c) 2004-2013, PathologyOutlines.com, Inc.


● Rare (10-30 annual cases in US), 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 1950's, detect its α particle emissions via autoradiography), vinyl chloride
● 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
● Non-operative biopsy may cause severe bleeding and death
● Most patients die within 6 months from hepatic failure or intraabdominal bleeding
● Metastasizes widely, often to lung (vinyl chloride cases usually don't have distant metastases)

Case reports

● 71 year old man with metastatic hepatic epithelioid angiosarcoma (Arch Pathol Lab Med 2001;125:968)

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

Vascular lesion with dense fibrosis

White-tan mass with red-brown foci and several blood-filled spaces

Micro 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

Micro images

Various images

FNA and H&E

FNA, AE1 / AE3, pleomorphic epithelial cells in sinusoidal pattern and CD31

Positive stains

● CD34, CD31, factor VIII related antigen and Ulex europaeus lectin type 1 (may not be present in poorly vasoformative areas)
● Also Prox-1 (reflect the lymphatic endothelial phenotype), Claudin-5

Negative stains

● Keratin (but positive in 12-35%)

Molecular description

● 50% of vinyl chloride associated cases have A:T to T:A transversion in p53

Electron microscopy description

● Weibel-Palade bodies

Differential diagnosis

Epithelioid hemangioendothelioma: less atypia, less mitotic activity, less necrosis, often prominent fibrous and hyalinized stroma
Fibrosarcoma: very rare; not particularly vascular; CD31 negative
Hepatocellular carcinoma: atypical hepatocytes, normal endothelial cells
Kaposiís sarcoma: HIV+, have extrahepatic nodules, portal distribution and lack angiosarcomatous foci
Peliosis hepatis
Reactive bile ductule proliferation, reactive lymphoid hyperplasia: reactive, lack atypical endothelial lining cells

End of Liver and intrahepatic bile ducts - tumor > Other malignancies > Angiosarcoma

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