Multifocal Kaposiform Hemangioendothelioma Successfully Treated With Sirolimus Monotherapy

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically presents in infancy and may be associated with the Kasabach-Merritt phenomenon (KMP). We present a challenging case of multifocal KHE on the leg of an infant, initially suspected at birth to be a reticulate port wine birthmark. Skin biopsy and imaging supported the rare diagnosis of multifocal KHE. Complicated by KMP, he was started on sirolimus monotherapy with significant improvement in his widespread disease.

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Publication Details

Journal
UNC Libraries
Published
2025-08-28
DOI
https://doi.org/10.17615/gte5-rg36
Primary Topic
Vascular Malformations and Hemangiomas
Type
article
Field-Weighted Citation Impact
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article

Multifocal Kaposiform Hemangioendothelioma Successfully Treated With Sirolimus Monotherapy

Matthew J. Mahoney, Emily Greengard, Daniel D. Miller, Sheilagh Maguiness et al.
UNC Libraries
Vascular Malformations and Hemangiomas
article

Multifocal Kaposiform Hemangioendothelioma Successfully Treated With Sirolimus Monotherapy

Matthew J. Mahoney, Emily Greengard, Daniel D. Miller, Sheilagh Maguiness, Bradley J. Segura, Michael A. Murati, John Leister, Alex Hoover, Erin P. Fritz
article en

Abstract

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically presents in infancy and may be associated with the Kasabach-Merritt phenomenon (KMP). We present a challenging case of multifocal KHE on the leg of an infant, initially suspected at birth to be a reticulate port wine birthmark. Skin biopsy and imaging supported the rare diagnosis of multifocal KHE. Complicated by KMP, he was started on sirolimus monotherapy with significant improvement in his widespread disease.

UNC Libraries
University of North Carolina at Chapel Hill (US)
Openalex Percentile: Top 47%
Vascular Malformations and Hemangiomas
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Multifocal Kaposiform Hemangioendothelioma Successfully Treated With Sirolimus Monotherapy — Matthew J. Mahoney, Emily Greengard, et al. · UNC Libraries (2025) | TGRS Research Map | TGRS