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Repeat biopsy at dissemination revealed pleomorphic histiocytes with tetraploidy, suggesting evolution to a clonal histiocytic neoplasm. Despite further chemotherapy, the patient died of disease progression. This case highlights the clinical and pathological heterogeneity of JXG and the difficulty of treating multi\u2010focal CNS disease. Pediatr Blood Cancer 2008;50:927\u2013930. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/pbc.21252","type":"journal-article","created":{"date-parts":[[2007,5,27]],"date-time":"2007-05-27T01:38:02Z","timestamp":1180229882000},"page":"927-930","source":"Crossref","is-referenced-by-count":52,"title":["Central nervous system juvenile xanthogranuloma with malignant transformation"],"prefix":"10.1002","volume":"50","author":[{"given":"Andrea","family":"Orsey","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michele","family":"Paessler","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beverly J.","family":"Lange","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kim E.","family":"Nichols","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2007,5,22]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(96)70247-0"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000147395.01229.06"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200305000-00003"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00234-002-0819-6"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1097\/00043426-200406000-00007"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.mph.0000199588.83679.76"},{"key":"e_1_2_5_8_2","first-page":"1","article-title":"Prolonged severe pancytopenia preceding the cutaneous lesions of juvenile xanthogranuloma","volume":"135","author":"Hara T","year":"2005","journal-title":"Pediatr Blood Cancer"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1991.74.6.0872"},{"key":"e_1_2_5_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00294464"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2000.93.2.0335"},{"key":"e_1_2_5_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(86)70049-2"},{"key":"e_1_2_5_13_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.1987.tb00787.x"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/0090-3019(91)90012-X"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF02016261"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1016\/0090-3019(93)90092-F"},{"key":"e_1_2_5_17_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.1993.tb00018.x"},{"key":"e_1_2_5_18_2","doi-asserted-by":"publisher","DOI":"10.1080\/02688699550041629"},{"key":"e_1_2_5_19_2","doi-asserted-by":"publisher","DOI":"10.1159\/000121212"},{"key":"e_1_2_5_20_2","unstructured":"ChidambaramB SantoshV.Giant orbital and intracranial xanthogranuloma Neurol India.2001;49:208\u2013210."},{"key":"e_1_2_5_21_2","first-page":"123","article-title":"15 year old girl with intracranial lesion","volume":"11","author":"Gutnik H","year":"2001","journal-title":"Brain Pathol"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.21252","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.21252","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,12]],"date-time":"2023-10-12T03:55:23Z","timestamp":1697082923000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.21252"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,5,22]]},"references-count":20,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2008,4]]}},"alternative-id":["10.1002\/pbc.21252"],"URL":"https:\/\/doi.org\/10.1002\/pbc.21252","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2007,5,22]]}}}