{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T19:27:24Z","timestamp":1779910044521,"version":"3.53.1"},"reference-count":33,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T00:00:00Z","timestamp":1764115200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Atypical Teratoid\/Rhabdoid Tumor (AT\/RT) is a highly aggressive neoplasm of the central nervous system (CNS), most commonly affecting infants and young children. Originally recognized as a distinct entity following cytogenetic identification of monosomy 22 in renal Rhabdoid Tumors, AT\/RT now encompasses CNS tumors characterized by SMARCB1 (INI-1) or SMARCA4 (BRG-1) alterations within the SWI\/SNF chromatin-remodeling complex. The integration of immunohistochemical markers with advanced molecular diagnostics\u2014including next-generation sequencing, DNA methylation profiling, and gene enrichment analyses\u2014has facilitated robust tumor classification and the identification of three molecular subgroups: TYR, SHH, and MYC. Despite its distinctive histopathologic features, AT\/RT remains diagnostically challenging in adolescent and adult populations due to age-related bias and potential morphologic heterogeneity. Differential considerations, including epithelioid sarcoma, poorly differentiated chordoma, CRINET, choroid plexus carcinoma, and rare composite tumors, further complicate the diagnostic landscape. A comprehensive, multimodal diagnostic approach combining histologic, immunophenotypic, and molecular data is essential to accurately identify AT\/RT and guide clinical management, particularly in diagnostically ambiguous or atypical cases.<\/jats:p>","DOI":"10.3390\/cancers17233768","type":"journal-article","created":{"date-parts":[[2025,11,26]],"date-time":"2025-11-26T10:04:57Z","timestamp":1764151497000},"page":"3768","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Atypical Teratoid Rhabdoid Tumor: How Tumor Diagnostic Methods in the Laboratory Have Evolved over the Past 40 Years"],"prefix":"10.3390","volume":"17","author":[{"given":"Heather L.","family":"Smith","sequence":"first","affiliation":[{"name":"Department of Pathology, Loyola University Medical Center, Maywood, IL 60153, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascale","family":"Aouad","sequence":"additional","affiliation":[{"name":"Department of Medical Imaging, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5816-2585","authenticated-orcid":false,"given":"Nitin R.","family":"Wadhwani","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1937","DOI":"10.1002\/1097-0142(197805)41:5<1937::AID-CNCR2820410538>3.0.CO;2-U","article-title":"Histopathology and prognosis of Wilms tumors: Results from the First National Wilms\u2019 Tumor Study","volume":"41","author":"Beckwith","year":"1978","journal-title":"Cancer"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1056\/NEJM198909283211317","article-title":"Monosomy 22 in rhabdoid or atypical teratoid tumors of the brain","volume":"321","author":"Biegel","year":"1989","journal-title":"N. Engl. J. 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