{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T09:18:46Z","timestamp":1774430326846,"version":"3.50.1"},"reference-count":53,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2022,3,22]],"date-time":"2022-03-22T00:00:00Z","timestamp":1647907200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100007880","name":"Associazione Bianca Garavaglia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100007880","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Histopathology"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:sec><jats:title>Aims<\/jats:title><jats:p>Renal cell carcinomas (RCCs) represent 2\u20135% of kidney malignancies in children and adolescents. Appropriate diagnostic and classification are crucial for the correct management of the patients and in order to avoid inappropriate pre\u2010operative chemotherapy, which is usually recommended if a Wilms' tumour is suspected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods and results<\/jats:title><jats:p>A French\u2013Italian series of 93 renal cell carcinomas collected from 1990 to 2019 in patients aged less than 18\u2009years was reclassified according to the 2016 World Health Organization (WHO) classification and the latest literature. <jats:italic>TFE3<\/jats:italic> and <jats:italic>TFEB<\/jats:italic> fluorescence <jats:italic>in\u2010situ<\/jats:italic> hybridisation (FISH) analyses and a panel of immunohistochemical stains were applied. The median age at diagnosis was 11\u2009years (range\u2009=\u20099\u2009months\u201317\u2009years). <jats:italic>MiT<\/jats:italic> family (<jats:italic>MiTF<\/jats:italic>) translocation RCCs accounted for 52% of the tumours, followed by papillary (20%) and unclassified RCCs (13%). Other subtypes, such as SDHB\u2010deficient and fumarate hydratase\u2010deficient RCCs, represented 1\u20133% of the cases. We also described a case of <jats:italic>ALK\u2010<\/jats:italic>rearranged RCC with a metanephric adenoma\u2010like morphology.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>A precise histological diagnosis is mandatory, as targeted therapy could be applied for some RCC subtypes, i.e. <jats:italic>MiTF<\/jats:italic>\u2010translocation and <jats:italic>ALK<\/jats:italic>\u2010translocation RCC. Moreover, some RCC subtypes may\u2009be associated with a predisposition syndrome that will impact patients' and family's management and genetic counselling. A precise RCC subtype is also mandatory for the clinical management of the\u2009patients and inclusion in new prospective clinical trials.<\/jats:p><\/jats:sec>","DOI":"10.1111\/his.14634","type":"journal-article","created":{"date-parts":[[2022,3,3]],"date-time":"2022-03-03T09:52:25Z","timestamp":1646301145000},"page":"928-945","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Renal cell carcinoma in children and adolescents: a retrospective study of a French\u2013Italian series of 93 cases"],"prefix":"10.1111","volume":"80","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8618-7691","authenticated-orcid":false,"given":"Thomas","family":"Denize","sequence":"first","affiliation":[{"name":"Department of Pathology, Sorbonne Universit\u00e9 Assistance Publique H\u00f4pitaux de Paris, H\u00f4pital Armand Trousseau Paris 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