Lymphatic Malformation Responsive to Sirolimus in Keratinocytic Epidermal Nevus Syndrome with KRAS Mutation: A Case and Brief Literature Discussion

We present a rare case of KRAS keratinocytic epidermal nevus syndrome with lymphatic malformation, responsive to treatment with sirolimus, an mTOR inhibitor. A brief review of the current literature regarding sirolimus use in vascular malformations, lymphatic malformations, regional overgrowth syndr...

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Detalles Bibliográficos
Autores principales: Sideris, Emily, Tng, Er Tsing Vivian, Chee, Paul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2021
Materias:
Case and Review
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8488422/
https://www.ncbi.nlm.nih.gov/pubmed/34703427
http://dx.doi.org/10.1159/000515247
Descripción
Sumario:We present a rare case of KRAS keratinocytic epidermal nevus syndrome with lymphatic malformation, responsive to treatment with sirolimus, an mTOR inhibitor. A brief review of the current literature regarding sirolimus use in vascular malformations, lymphatic malformations, regional overgrowth syndromes, and RASopathies is discussed.

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