{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T03:38:56Z","timestamp":1778125136557,"version":"3.51.4"},"reference-count":35,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,7,11]],"date-time":"2017-07-11T00:00:00Z","timestamp":1499731200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Purpose<\/jats:title><jats:p>The optimal management of central nervous system (CNS) relapse of rhabdomyosarcoma (RMS) is unclear. We examined diagnosis, management, and outcomes of patients with RMS developing CNS relapse.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Records of 23 patients diagnosed with CNS relapse between 1999 and 2016 were reviewed. Median age at presentation of CNS relapse was 15 years (range, 1\u201334 years). High\u2010risk features at initial presentation were as follows: 16 alveolar patients, 13 Stage IV, and 13 with primary tumor in parameningeal locations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>CNS relapse occurred at a median 12 months (range, 1\u201323 months) from diagnosis and most common presenting symptoms were headache (n = 9), nausea\/vomiting (n = 8), visual difficulty (n = 5), and none (n = 5). Leptomeningeal metastases were detected in 21 patients while only 2 developed parenchymal metastases without leptomeningeal involvement. Fifteen patients received CNS\u2010directed radiation therapy (RT), including craniospinal irradiation to a median 36 Gy (range, 18\u201336 Gy) and\/or whole brain radiotherapy to a median 30 Gy (range, 6\u201341.4 Gy). Three patients received concurrent chemotherapy. Follow\u2010up magnetic resonance imaging was conducted in 13 patients after RT initiation with 8 demonstrating improvement, 2 with stable disease, and 3 with progression. Twelve patients were tested for reactivity to I\u2010131\u2010labeled monoclonal antibody 8H9, and three tested positive and received at least one intra\u2010Ommaya dose; all three lived &gt;12 months post\u2010CNS relapse. Twenty\u2010one patients died of CNS disease and two of metastatic disease at other sites. Median survival post\u2010CNS relapse was 5 months (range, 0.1\u201349 months).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The prognosis for patients with RMS developing CNS relapse remains poor. Treatment including CNS\u2010directed RT should be considered and investigation into preventative therapies is warranted.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.26710","type":"journal-article","created":{"date-parts":[[2017,7,11]],"date-time":"2017-07-11T12:17:16Z","timestamp":1499775436000},"source":"Crossref","is-referenced-by-count":26,"title":["Central nervous system relapse of rhabdomyosarcoma"],"prefix":"10.1002","volume":"65","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3468-3359","authenticated-orcid":false,"given":"Brian","family":"De","sequence":"first","affiliation":[{"name":"Department of Radiation Oncology Memorial Sloan Kettering Cancer Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael D.","family":"Kinnaman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leonard 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