{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,13]],"date-time":"2026-04-13T12:07:22Z","timestamp":1776082042201,"version":"3.50.1"},"reference-count":30,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2020,5,8]],"date-time":"2020-05-08T00:00:00Z","timestamp":1588896000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2020,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Juvenile xanthogranuloma (JXG) is the most common non\u2010Langerhans cell histiocytosis in children. The mortality and morbidity of JXG with extracutaneous lesions remain unclear.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data of patients aged\u00a0&lt;\u00a018 years who were diagnosed with JXG between 2001 and 2010 were retrospectively collected through a nationwide survey.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twenty patients (11 male and nine female) had extracutaneous lesions. The median observation time was 10 years (range, 0\u201017). Six patients presented with symptoms at birth. The median age at diagnosis was 8.5 months (range, 0 month\u201013 years). Fifteen patients underwent treatment for JXG, including chemotherapy (<jats:italic>n<\/jats:italic>\u00a0=\u00a011), and five did not receive treatment. All patients except one survived; 17 were disease\u2010free and two survived with disease. One newborn\u2010onset patient with liver, spleen, and bone marrow involvement died of the disease. Permanent sequelae included central diabetes insipidus, growth hormone deficiency, and panhypopituitarism detected at diagnosis in three, one, and two patients, respectively. Four patients had visual impairment (optic nerve compression and intraocular invasion in two each), three had epilepsy, one had mental retardation, and one had a skin scar. Eight patients who had intracranial lesions were older at diagnosis, and had a higher frequency of disease\u2010related comorbidities and permanent sequelae than those without intracranial involvement.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Patients with extracutaneous JXG had good outcomes, although those with intracranial lesions had serious permanent sequelae. Effective and safe treatment regimens for patients with intracranial JXG need to be developed.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.28381","type":"journal-article","created":{"date-parts":[[2020,5,8]],"date-time":"2020-05-08T13:07:30Z","timestamp":1588943250000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":18,"title":["Long\u2010term outcomes of children with extracutaneous juvenile xanthogranulomas in Japan"],"prefix":"10.1002","volume":"67","author":[{"given":"Miho","family":"Maeda","sequence":"first","affiliation":[{"name":"Department of Pediatrics Nippon Medical School  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9168-5409","authenticated-orcid":false,"given":"Akira","family":"Morimoto","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Jichi Medical University School of Medicine  Shimotsuke Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoko","family":"Shioda","sequence":"additional","affiliation":[{"name":"Children's Cancer Center National Center for Child Health and Development  Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3275-1979","authenticated-orcid":false,"given":"Takeshi","family":"Asano","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Nippon Medical School Chiba Hokuso Hospital  Chiba Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0056-9988","authenticated-orcid":false,"given":"Yuhki","family":"Koga","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Graduate School of Medical Sciences Kyushu University  Fukuoka 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University  Fukuoka Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eiichi","family":"Ishii","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Ehime University Graduate School of Medicine  Toon Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"Histiocytosis Study Group of the Japanese Society of Pediatric 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