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Pediatric Hepatic Angiosarcoma: Case Report and Review of the Literature

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Pediatric and Developmental Pathology

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.

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ACKNOWLEDGMENTS

We thank Louis P. Dehner, M.D., at the Washington University Medical Center, for his expert opinion on this case, and Henry Giles, M.D., and Michael Doherty, M.D., at the University of Mississippi Medical Center, for providing image pictures. We also thank Chris Woods and Jay Card for their technical assistance.

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Correspondence to Margaret H. Collins.

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Dimashkieh, H., Mo, J., Wyatt-Ashmead, J. et al. Pediatric Hepatic Angiosarcoma: Case Report and Review of the Literature. Pediatr Dev Pathol 7, 527–532 (2004). https://doi.org/10.1007/s10024-004-4041-x

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  • DOI: https://doi.org/10.1007/s10024-004-4041-x

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