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In this review of 168 pediatric RCC prospectively registered on Children's Oncology Group AREN03B2 protocol, six RCC (3.5%) that demonstrated a unique epithelioid morphology and a peculiar immunophenotypic profile that includes expression of ALK, TFE3, and retention of INI1 was identified. Further investigation revealed <jats:italic>ALK<\/jats:italic> rearrangements in all cases, manifested molecularly by fusion transcripts of either <jats:italic>VCL\u2010ALK<\/jats:italic> (3 patients all with sickle cell trait which had been previously reported) or <jats:italic>TPM3\u2010ALK<\/jats:italic> (3 patients, none with sickle cell trait). Based on the shared unique morphologic, immunophenotypic, and genetic features, it was proposed that these neoplasms belonged to a distinct subgroup of RCC frequently occurring in pediatric patients, which they have termed as <jats:italic>ALK<\/jats:italic>\u2010rearranged RCC. Importantly, additional therapeutic options may be available for these patients. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/gcc.22346","type":"journal-article","created":{"date-parts":[[2016,1,16]],"date-time":"2016-01-16T18:14:57Z","timestamp":1452968097000},"page":"442-451","source":"Crossref","is-referenced-by-count":59,"title":["<scp><i>ALK<\/i><\/scp><i>\u2010<\/i>rearranged renal cell carcinomas in children"],"prefix":"10.1002","volume":"55","author":[{"given":"Mariana M.","family":"Cajaiba","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann &amp; Robert H. Lurie Children's Hospital of Chicago and Northwestern University Feinberg School of Medicine Chicago IL"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lawrence J.","family":"Jennings","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann &amp; Robert H. 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