{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T01:38:03Z","timestamp":1793410683685,"version":"4.5.0"},"reference-count":97,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2021,5,16]],"date-time":"2021-05-16T00:00:00Z","timestamp":1621123200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"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"}]},{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["P30 CA008748"],"award-info":[{"award-number":["P30 CA008748"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Histiocytoses are heterogeneous hematopoietic diseases characterized by the accumulation of CD68(+) cells with various admixed inflammatory infiltrates. The identification of the pivotal role of the mitogen-activated protein kinase (MAPK) pathway has opened new avenues of research and therapeutic approaches. We review the neurologic manifestations of 3 histiocytic disorders with frequent involvement of the brain and spine: Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), and Rosai-Dorfman-Destombes disease (RDD). Central nervous system (CNS) manifestations occur in 10%-25% of LCH cases, with both tumorous or neurodegenerative forms. These subtypes differ by clinical and radiological presentation, pathogenesis, and prognosis. Tumorous or degenerative neurologic involvement occurs in 30%-40% of ECD patients and affects the hypothalamic-pituitary axis, meninges, and brain parenchyma. RDD lesions are typically tumorous with meningeal or parenchymal masses with strong contrast enhancement. Unlike LCH and ECD, neurodegenerative lesions or syndromes have not been described with RDD. Familiarity with principles of evaluation and treatment both shared among and distinct to each of these 3 diseases is critical for effective management. Refractory or disabling neurohistiocytic involvement should prompt the consideration for use of targeted kinase inhibitor therapies.<\/jats:p>","DOI":"10.1093\/neuonc\/noab107","type":"journal-article","created":{"date-parts":[[2021,5,13]],"date-time":"2021-05-13T15:22:06Z","timestamp":1620919326000},"page":"1433-1446","source":"Crossref","is-referenced-by-count":95,"title":["Histiocytosis and the nervous system: from diagnosis to targeted therapies"],"prefix":"10.1093","volume":"23","author":[{"given":"Fleur","family":"Cohen Aubart","sequence":"first","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de M\u00e9decine Interne 2, Centre National de R\u00e9f\u00e9rence Maladies Syst\u00e9miques Rares et Histiocytoses, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Idbaih","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de Neurologie 1, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean-Fran\u00e7ois","family":"Emile","sequence":"additional","affiliation":[{"name":"Assistance Publique H\u00f4pitaux de Paris, H\u00f4pital Ambroise Par\u00e9, D\u00e9partement de Pathologie, Universit\u00e9 Versailles-Saint Quentin, Boulogne, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zahir","family":"Amoura","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de M\u00e9decine Interne 2, Centre National de R\u00e9f\u00e9rence Maladies Syst\u00e9miques Rares et Histiocytoses, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Omar","family":"Abdel-Wahab","sequence":"additional","affiliation":[{"name":"Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin H","family":"Durham","sequence":"additional","affiliation":[{"name":"Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien","family":"Haroche","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, Service de M\u00e9decine Interne 2, Centre National de R\u00e9f\u00e9rence Maladies Syst\u00e9miques Rares et Histiocytoses, H\u00f4pital Piti\u00e9-Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eli L","family":"Diamond","sequence":"additional","affiliation":[{"name":"Department of Neurology, Memorial Sloan Kettering Cancer Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,5,16]]},"reference":[{"issue":"9","key":"2021090111013851300_CIT0001","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1056\/NEJMra1607548","article-title":"Langerhans-cell histiocytosis","volume":"379","author":"Allen","year":"2018","journal-title":"N Engl J Med."},{"issue":"2","key":"2021090111013851300_CIT0002","doi-asserted-by":"crossref","first-page":"e113","DOI":"10.1016\/S1470-2045(17)30031-1","article-title":"Histiocytoses: emerging neoplasia behind inflammation","volume":"18","author":"Haroche","year":"2017","journal-title":"Lancet Oncol."},{"issue":"22","key":"2021090111013851300_CIT0003","doi-asserted-by":"crossref","first-page":"2672","DOI":"10.1182\/blood-2016-01-690636","article-title":"Revised 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