No abstract

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.

Results of this study suggest that histopathologic findings may correlate with clinical outcome in cases of necrotizing fasciitis. Because the histopathologic scheme is based on results of commonly available stains, it could be easily adopted for use in other institutions that could further evaluate its usefulness as a prognostic tool.