title: CNS involvement of Langerhans cell histiocytosis. Report of 23 surgically treated cases.
abstract: We present 23 cases of Langerhans cell histiocytosis with central nervous system (CNS) involvement. The major complaints were a mass on the cranial vault in fifteen (65%), visual disturbance in four (16%), polyurea-polydipsia in three (13%), and progressive weakness in all extremities in one patient (4%). Neurological examination revealed no abnormality in sixteen patients (70%), cranial nerve palsy, visual field defect and optic atrophy in six (26%) and paraparesis in one (4%). Tumoral mass was found to be located on the cranial vault (65%), in the suprasellar region (21%) and in the spinal column (8%). The cranium and spinal column were both involved in one patient. All patients underwent surgery; craniectomy with grossly total tumor excision plus cranioplasty (65%), craniotomy with subtotal tumor excision (26%), and vertebrectomy with grafting (13%) were performed. The clinical, radiological and histopathological features, as well as therapeutical considerations are discussed and the pertinent literature is reviewed.
authors.affiliation: Hacettepe University School of Medicine, Ankara, Turkey.
authors.forename: D
authors.initials: D
authors.lastname: Belen
authors.forename: A
authors.initials: A
authors.lastname: Colak
authors.forename: O E
authors.initials: OE
authors.lastname: Ozcan
mesh_terms: D000293:Adolescent; D000328:Adult; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D012008:Recurrence; D012888:Skull Neoplasms / Q000175:diagnosis / Q000601:surgery*; D013125:Spinal Neoplasms / Q000175:diagnosis / Q000601:surgery*; D014057:Tomography, X-Ray Computed