title: Treatment of severe disseminated juvenile systemic xanthogranuloma with multiple lesions in the central nervous system.
abstract: Juvenile xanthogranuloma (JXG), one of the most common forms of Langerhans-dendritic cell proliferation in young children, usually presents as spontaneously regressing cutaneous lesions. JXG with systemic (extracutaneous) involvement is a rare histiocytic disorder in which significant morbidity and death may occur. The systemic type, especially combined with multiple central nervous system lesions in young children, has a very poor prognosis. The patient described here presented with disseminated disease including lungs, liver, kidneys, ribs, scalp, and central nervous system. The patient was treated with multiagent chemotherapy based on the Langerhans cell histiocytosis II treatment protocol. The regimen used included an additional intrathecal therapy with methotrexate and prednisolone to control central nervous system lesions. The patient was treated for 28 months and has been in remission for almost 5 years.
authors.affiliation: Department of Pediatric Oncology and Hematology, University of Greifswald, Germany. pae.onko@uni-greifswald.de
authors.forename: Regina
authors.initials: R
authors.lastname: Dölken
authors.forename: Steffen
authors.initials: S
authors.lastname: Weigel
authors.forename: Henry
authors.initials: H
authors.lastname: Schröder
authors.forename: Maite
authors.initials: M
authors.lastname: Hartwig
authors.forename: Dieter
authors.initials: D
authors.lastname: Harms
authors.forename: James F
authors.initials: JF
authors.lastname: Beck
mesh_terms: D002493:Central Nervous System Diseases / Q000188:drug therapy* / Q000601:surgery; D003131:Combined Modality Therapy; D003399:Craniotomy; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007278:Injections, Spinal; D008107:Liver Diseases / Q000188:drug therapy; D008171:Lung Diseases / Q000188:drug therapy; D015122:Mercaptopurine / Q000008:administration & dosage / Q000627:therapeutic use*; D008727:Methotrexate / Q000008:administration & dosage / Q000627:therapeutic use*; D010291:Paresis / Q000209:etiology; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use*; D012871:Skin Diseases / Q000188:drug therapy; D014747:Vinblastine / Q000008:administration & dosage / Q000627:therapeutic use*; D014972:Xanthogranuloma, Juvenile / Q000188:drug therapy* / Q000601:surgery
chemical_list: D014747:Vinblastine; D011239:Prednisolone; D015122:Mercaptopurine; D008727:Methotrexate