Key result
Primary hepatic angiosarcoma is an aggressive malignancy with a median survival of 6 months without treatment, and only 3% of patients live longer than 2 years after treatment.
Primary hepatic angiosarcoma is a rare, aggressive malignancy with very poor survival outcomes.
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Poor prognosis demands early specialist referral and novel therapies; leaves open optimal management strategies for this rare tumor.
Primary hepatic angiosarcoma is a rare entity, representing only 4% of all the angiosarcomas of different origins1 and less than 1% of all hepatic malignancies.2 Accurate diagnosis of this tumour is difficult, especially if the patient has no history of exposure to specific carcinogens including thorotrast, arsenicals and vinyl chloride monomer.3 Angiosarcoma, a subtype of soft tissue sarcoma, is an aggressive malignant disease deriving from endothelium, lymphatics or blood vessels. Some of the more common hepatic sarcomas are angiosarcoma, embryonal sarcoma, leiomyosarcoma, epithelioid hemangioendothelioma, fibrosarcoma and malignant fibrous histiocytoma.2 The survival of hepatic angiosarcoma is very poor, which is attributable to its rapid progress, high recurrence rate and resistant to traditional chemotherapy and radiotherapy.4 The survival of patients with liver angiosarcoma is very poor with median survival of 6 months without treatment; after treatment, only 3% of patients were reported to live longer than 2 years
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Coppola et al. (2015) conducted a review in Primary hepatic angiosarcoma. Primary hepatic angiosarcoma is an aggressive malignancy with a median survival of 6 months without treatment, and only 3% of patients live longer than 2 years after treatment.
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