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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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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
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author Attia, Kamal H.
Al Boukai, Ahmad A.
Mohammed, Muammar H.
Arafah, Maha
author_facet Attia, Kamal H.
Al Boukai, Ahmad A.
Mohammed, Muammar H.
Arafah, Maha
author_sort Attia, Kamal H.
collection PubMed
description 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.
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spelling pubmed-100436012023-03-29 Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report Attia, Kamal H. Al Boukai, Ahmad A. Mohammed, Muammar H. Arafah, Maha BJR Case Rep 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 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. The British Institute of Radiology. 2022-12-21 /pmc/articles/PMC10043601/ /pubmed/36998339 http://dx.doi.org/10.1259/bjrcr.20220105 Text en © 2022 The Authors. Published by the British Institute of Radiology https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International License (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.
spellingShingle Case Report
Attia, Kamal H.
Al Boukai, Ahmad A.
Mohammed, Muammar H.
Arafah, Maha
Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report
title Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report
title_full Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report
title_fullStr Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report
title_full_unstemmed Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report
title_short Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report
title_sort renal and mediastinal perivascular epithelioid cell tumors (pecomas) in a young child with tuberous sclerosis; a rare case report
topic Case Report
url 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
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