Kamal H Attia, Ahmad A Al Boukai, Muammar H Mohammed, Maha Arafah
{"title":"Renal and mediastinal perivascular epithelioid cell tumors (PEComas) in a young child with tuberous sclerosis; a rare case report.","authors":"Kamal H Attia, Ahmad A Al Boukai, Muammar H Mohammed, Maha Arafah","doi":"10.1259/bjrcr.20220105","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":45216,"journal":{"name":"BJR Case Reports","volume":null,"pages":null},"PeriodicalIF":0.5000,"publicationDate":"2023-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10043601/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"BJR Case Reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1259/bjrcr.20220105","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"RADIOLOGY, NUCLEAR MEDICINE & MEDICAL IMAGING","Score":null,"Total":0}
引用次数: 0
Abstract
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.