{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T21:26:46Z","timestamp":1789075606026,"version":"build-2803163510"},"reference-count":93,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2026,4,5]],"date-time":"2026-04-05T00:00:00Z","timestamp":1775347200000},"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 rare, highly aggressive embryonal central nervous system malignancy occurring predominately in infants and toddlers. Spinal AT\/RT (spAT\/RT) cases are even more limited, and as a result, little is known regarding prognostic factors and optimal treatment regimens. Molecularly, AT\/RT is divided into three groups: AT\/RT-SHH, AT\/RT-TYR and AT\/RT-MYC. spAT\/RT is predominantly of the MYC subtype. Additionally, a third of patients with AT\/RT have a germline Rhabdoid Tumor Predisposition Syndrome (RTPS) that increases the likelihood of developing additional rhabdoid tumors, including renal rhabdoid tumors. Due to the rarity of these tumors, there is a lack of consensus on treatment strategies to be employed. This review paper details the published literature on spAT\/RT, with particular emphasis on the recent advances in understanding the biology of these aggressive tumors and currently available therapeutic options, and highlights the challenges associated with the management of this extremely rare condition.<\/jats:p>","DOI":"10.3390\/cancers18071171","type":"journal-article","created":{"date-parts":[[2026,4,6]],"date-time":"2026-04-06T00:19:32Z","timestamp":1775434772000},"page":"1171","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Pediatric Spinal Atypical Teratoid Rhabdoid Tumor: Recent Advances in Biology and Management Options"],"prefix":"10.3390","volume":"18","author":[{"given":"Ruby","family":"Siada","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Michigan, Ann Arbor, MI 48109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaushik","family":"Banerjee","sequence":"additional","affiliation":[{"name":"Department of Biological Chemistry, University of Michigan, Ann Arbor, MI 48109, USA"},{"name":"Department of Pediatrics, Division of Pediatric Hematology Oncology, University of Michigan, Ann Arbor, MI 48109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Payal","family":"Malhotra","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology-Oncology & BMT, Rajiv Gandhi Cancer Institute & Research Centre, Delhi 110085, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohannad","family":"Ibrahim","sequence":"additional","affiliation":[{"name":"Department of Radiology, University of Michigan, Ann Arbor, MI 48109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel C.","family":"Moreira","sequence":"additional","affiliation":[{"name":"Department of Oncology, Division of Neuro-Oncology, St. Jude Children\u2019s Research Hospital, Memphis, TN 38105, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R.","family":"Prensner","sequence":"additional","affiliation":[{"name":"Department of Biological Chemistry, University of Michigan, Ann Arbor, MI 48109, USA"},{"name":"Department of Pediatrics, Division of Pediatric Hematology Oncology, University of Michigan, Ann Arbor, MI 48109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6101-149X","authenticated-orcid":false,"given":"Santhosh A.","family":"Upadhyaya","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Hematology Oncology, University of Michigan, Ann Arbor, MI 48109, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,4,5]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1097\/00043426-200206000-00004","article-title":"Atypical teratoid\/rhabdoid tumor of the central nervous system: Report on workshop","volume":"24","author":"Packer","year":"2002","journal-title":"J. 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