title: Solitary juvenile xanthogranuloma mimicking intracranial tumor in children.
abstract: Juvenile xanthogranuloma (JXG) is primarily a benign cutaneous disorder of non-Langerhans hystiocytic proliferation. Systemic involvement occurs in 4% of patients; isolated central nervous system (CNS) lesions are rare. We report solitary CNS-JXG lesions in two patients. A 3.5-year-old boy with a parietal-occipital lesion underwent total resection with no surgical morbidity and no recurrence at 16-month follow-up. A 3.5-year-old girl underwent subtotal resection of a tumor extending from the left Meckel's cave and invading the cavernous sinus and left orbit with extensive cranial nerve involvement. Tumor regrowth with leptomeningeal spread at 9-month and 12-month follow-up was managed with steroids and chemotherapy (vinblastine and later cladribine). We present our experience and review the literature pertaining to rare reports of solitary CNS-JXG.
authors.affiliation: Department of Neurosurgery, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem 91120, Israel.
authors.forename: Idit
authors.initials: I
authors.lastname: Tamir
authors.forename: Rina
authors.initials: R
authors.lastname: Davir
authors.forename: Yakov
authors.initials: Y
authors.lastname: Fellig
authors.forename: Michael
authors.initials: M
authors.lastname: Weintraub
authors.forename: Shlomo
authors.initials: S
authors.lastname: Constantini
authors.forename: Sergey
authors.initials: S
authors.lastname: Spektor
mesh_terms: D000199:Actins / Q000378:metabolism; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D001921:Brain / Q000378:metabolism / Q000473:pathology / Q000648:ultrastructure; D001932:Brain Neoplasms / Q000503:physiopathology*; D002675:Child, Preschool; D005176:Factor XIII / Q000378:metabolism; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D046529:Microscopy, Electron, Transmission; D018396:Mucin-1 / Q000378:metabolism; D009418:S100 Proteins / Q000378:metabolism; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis* / Q000503:physiopathology* / Q000601:surgery; D000097382:CD68 Molecule
chemical_list: D000199:Actins; D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D005176:Factor XIII; D018396:Mucin-1; D009418:S100 Proteins; C067980:CD68 antigen, human; D000097382:CD68 Molecule; C064015:factor XIII subunit A