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Infantile Hepatic Hemangioma/Hemangioendothelioma

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Abstract

Infantile hepatic hemangioma (IHE; previously termed infantile hepatic hemangioendothelioma) is a vascular liver tumor presenting as solitary or multiple lesions and diagnosed almost exclusively in infants and children before 6 months of age. Apart from focal involvement of the liver, a diffuse phenotype is also recognized. IHE usually manifests as hepatomegaly, but part of patients show cardiovascular failure due to intratumoral shunting, coagulopathy, jaundice, respiratory distress, and a distinct form of consumptive hypothyroidism. IHE can be associated with angiomatous lesions in other organs, including chorangioma of the placenta. Macroscopically, IHE manifests as solitary or multiple, well-circumscribed, and spherical vascular tumors. Histology is characterized by a network of densely arranged and thin vascular channels with an endothelial lining. Calcifications and other regressive changes can develop. Part of patients show a histology with variable cellular atypias, previously termed type 2 lesions. There is an overlap between such lesions and a distinct form of infantile hepatic angiosarcoma.

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Zimmermann, A. (2017). Infantile Hepatic Hemangioma/Hemangioendothelioma. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_52

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  • DOI: https://doi.org/10.1007/978-3-319-26956-6_52

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