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Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report

We describe a unique case of perivascular epithelioid cell tumors occurring as mediastinal and left renal soft tissue masses discovered incidentally in a 5-year-old tuberous sclerosis patient upon presentation to the emergency department for upper respiratory illness. The radiographic features were...

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Detalles Bibliográficos
Autores principales: Attia, Kamal H., Al Boukai, Ahmad A., Mohammed, Muammar H., Arafah, Maha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The British Institute of Radiology. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10043601/
https://www.ncbi.nlm.nih.gov/pubmed/36998339
http://dx.doi.org/10.1259/bjrcr.20220105
Descripción
Sumario:We describe a unique case of perivascular epithelioid cell tumors occurring as mediastinal and left renal soft tissue masses discovered incidentally in a 5-year-old tuberous sclerosis patient upon presentation to the emergency department for upper respiratory illness. The radiographic features were non-specific. However, the similar CT characteristics of both lesions and background history raised the suspicion of a synchronous mesenchymal tumor, and histopathology confirmed the diagnosis. The rarity of these tumors in the pediatric population and lack of specific diagnostic criteria impose reporting the case and emphasize the need for further research on imaging features of such tumors.