Pediatric hepatic angiosarcoma has a poor prognosis, and in this case, despite aggressive treatment including liver transplantation, lung metastases occurred at 14 months.
May warrant intensified post-transplant surveillance in pediatric hepatic angiosarcoma; leaves open the role of transplantation in this rare disease.
Pediatric hepatic angiosarcoma (PHAS) is a rare tumor, which usually presents as a rapid enlargement of the liver. To date, surgery, chemotherapy, and radiotherapy have not improved the poor prognosis of PHAS with only three survivors reported. The histology of PHAS is distinct from adult angiosarcoma, because PHAS displays hypercellular whorls of sarcomatous cells, or "kaposiform" spindle cells, in addition to the general features of angiosarcoma. We report a case of PHAS that was treated with vascular ablation, chemotherapy, and liver transplantation. Lung metastases occurred 14 months posttransplant.
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Dimashkieh et al. (2004) studied this question.
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