Nord Kristin M, Kandel Jessica, Lefkowitch Jay H, Lobritto Steven J, Morel Kimberly D, North Paula E, Garzon Maria C
Department of Dermatology, Columbia University, New York, New York 10032, USA.
Pediatrics. 2006 Sep;118(3):e907-13. doi: 10.1542/peds.2006-0183. Epub 2006 Jul 31.
Multiple cutaneous hemangiomas can be associated with internal hemangiomas, with the liver being the most common site. Here we report a case of a premature female neonate who presented with cardiac failure at birth and had typical-appearing infantile hemangiomas on the skin in association with vascular lesions in the liver. Her clinical presentation was felt to be consistent with cutaneous and hepatic infantile hemangiomas. After failure to respond to systemic steroids and chemotherapy, she underwent liver transplantation. Histopathologic evaluation of the liver revealed a diagnosis of type 2 infantile hepatic hemangioendothelioma (regarded as synonymous with angiosarcoma) rather than benign infantile hemangioma of the liver. Subsequent skin biopsies confirmed that her multiple cutaneous lesions were infantile hemangiomas and not metastatic angiosarcoma. We report this case and a review of the literature on pediatric angiosarcoma of the liver associated with cutaneous infantile hemangiomas.
多发性皮肤血管瘤可与内脏血管瘤相关,其中肝脏是最常见的受累部位。本文报告1例早产女婴,出生时即出现心力衰竭,皮肤有典型的婴儿血管瘤,并伴有肝脏血管病变。其临床表现被认为与皮肤及肝脏婴儿血管瘤相符。在对全身类固醇和化疗无反应后,她接受了肝移植。肝脏组织病理学评估显示诊断为2型婴儿肝血管内皮瘤(被认为与血管肉瘤同义)而非肝脏良性婴儿血管瘤。随后的皮肤活检证实其多发性皮肤病变为婴儿血管瘤而非转移性血管肉瘤。我们报告此病例并复习了关于与皮肤婴儿血管瘤相关的儿童肝脏血管肉瘤的文献。