title: Successful treatment of congenital systemic juvenile xanthogranuloma with Langerhans cell histiocytosis-based chemotherapy.
abstract: Juvenile xanthogranuloma (JXG), one of the most common forms of non-Langerhans cell histiocytosis (LCH), usually presents in young children as spontaneously regressing cutaneous lesions. However, the systemic type of JXG is difficult to treat in newborn infants, and fatal cases have been reported. In the patient described here, solid masses were discovered by fetal sonography during the 38th gestational week. At birth she had multiple tumors on the back, cheek, and hip as well as marked hepatosplenomegaly accompanied by respiratory failure. Laboratory results indicated pancytopenia, obstructive liver dysfunction, and coagulopathy. Brain magnetic resonance imaging revealed a tumor at the left pontine angle, and dysmorphic histiocytes were present in her spinal fluid. She was diagnosed with systemic JXG by histopathologic findings of the hip mass. The LCH-based multiagent chemotherapy including cytarabine, vincristine, methotrexate, and prednisolone ameliorated the symptoms rapidly. She was treated for 12 months and is currently doing well as a normally developing 2-year-old.
authors.affiliation: Department of Pediatrics, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, Kyoto, Japan. t-nakatani@mb2.seikyou.ne.jp
authors.forename: Takuya
authors.initials: T
authors.lastname: Nakatani
authors.forename: Akira
authors.initials: A
authors.lastname: Morimoto
authors.forename: Ryuichi
authors.initials: R
authors.lastname: Kato
authors.forename: Sachiko
authors.initials: S
authors.lastname: Tokuda
authors.forename: Tohru
authors.initials: T
authors.lastname: Sugimoto
authors.forename: Kazuaki
authors.initials: K
authors.lastname: Tokiwa
authors.forename: Yasunari
authors.initials: Y
authors.lastname: Tsuchihashi
authors.forename: Shinsaku
authors.initials: S
authors.lastname: Imashuku
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy*; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D016896:Treatment Outcome; D014972:Xanthogranuloma, Juvenile / Q000151:congenital* / Q000188:drug therapy* / Q000473:pathology