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A case report of Kaposiform haemangioendothelioma; response with propranolol and steroids

BACKGROUND: Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. It is a locally aggressive tumor usually seen in infants. Here we report a case of kaposiform hemagioendothelioma in a child who responded to propranolol and steroids. CASE PRESENTAT...

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Detalles Bibliográficos
Autores principales: Verma, Saurav, Dhamija, Ekta, Barwad, Adarsh, Kumar, Venkatesan S., Rastogi, Sameer
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7394668/
https://www.ncbi.nlm.nih.gov/pubmed/32765826
http://dx.doi.org/10.1186/s13569-020-00134-8
Descripción
Sumario:BACKGROUND: Kaposiform haemangioendothelioma is a rare vascular tumor and may involve skin, deep soft tissue or bone. It is a locally aggressive tumor usually seen in infants. Here we report a case of kaposiform hemagioendothelioma in a child who responded to propranolol and steroids. CASE PRESENTATION: A 3-year-old male child presented with a swelling below his right knee with characteristic violet skin lesion. There was no evidence of Kasabach–Merritt phenomenon. After no improvement with several attempts at debridement and anti-tubercular treatment; a diagnosis of Kaposiform Haemangioendothelioma was reached on the basis of overall clinical picture and histology. The child was treated with propranolol and steroids and had an excellent clinical response and a near complete resolution on imaging at 5 months. CONCLUSIONS: These cases are often misdiagnosed and despite a delay in diagnosis have good outcomes with appropriate multimodality management. This case highlights the unique and typical characteristics of kaposiform haemangioendothelioma.