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Epithelioid Hemangioendothelioma in a Child

Epithelioid hemangioendothelioma (EHE) is a rare tumour arising from the vascular endothelium of soft-tissue, bone and viscera. Skin involvement is rare. The disease has an indolent course, with the potential for recurrence and often associated with multi-systemic localisations. We present a rare ca...

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Detalles Bibliográficos
Autores principales: Madura, C, Sacchidanand, S, Barde, Nitin G, Biligi, Dayanand
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3884891/
https://www.ncbi.nlm.nih.gov/pubmed/24470723
http://dx.doi.org/10.4103/0974-2077.123414
Descripción
Sumario:Epithelioid hemangioendothelioma (EHE) is a rare tumour arising from the vascular endothelium of soft-tissue, bone and viscera. Skin involvement is rare. The disease has an indolent course, with the potential for recurrence and often associated with multi-systemic localisations. We present a rare case of cutaneous EHE without systemic involvement in 9-year-old boy. The tumour was completely excised and histopathologic diagnosis was consistent with EHE. Complete systemic assessment showed no internal localisation. Wide excision is the mainstay of therapy and regular follow-up is suggested due to the potential for recurrence. The majority of documented cases of EHE is in adults and has developed in association with an underlying systemic involvement. To the best of our knowledge, this is the second reported case of isolated cutaneous EHE in paediatric age group.