title: Isolated tumorous Langerhans cell histiocytosis of the brainstem: a diagnostic and therapeutic challenge.
abstract: Langerhans cell histiocytosis (LCH) of the CNS is a rare entity, known to involve primarily the hypothalamicpituitary region, with the clinical hallmark of diabetes insipidus. There have been a few reports of CNS LCH involving the brainstem as intraparenchymal enhancing lesions, but this has never been the presenting complaint of LCH. The authors report on a 7-year-old boy who presented with right cerebellopontine syndrome, in whom a well-defined, solid, enhancing lesion in the brainstem was diagnosed. Clinicoradiological differential diagnosis included glioma and tuberculosis. Biopsy revealed atypical histiocytes positive for CD68, CD1a, and S100 protein; these are the diagnostic features of LCH on histopathological examination. The rapid growth of the lesion was controlled with a chemotherapeutic regimen of cladribine.
authors.affiliation: Departments of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India. ameysavardekar@gmail.com
authors.forename: Amey
authors.initials: A
authors.lastname: Savardekar
authors.forename: Manjul
authors.initials: M
authors.lastname: Tripathi
authors.forename: Deepak
authors.initials: D
authors.lastname: Bansal
authors.forename: Kim
authors.initials: K
authors.lastname: Vaiphei
authors.forename: Sunil K
authors.initials: SK
authors.lastname: Gupta
mesh_terms: D015703:Antigens, CD / Q000032:analysis; D018949:Antigens, CD1 / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001706:Biopsy; D001933:Brain Stem / Q000737:chemistry / Q000473:pathology*; D002648:Child; D017338:Cladribine / Q000627:therapeutic use; D020790:Diabetes Insipidus, Neurogenic / Q000175:diagnosis / Q000209:etiology; D003937:Diagnosis, Differential; D005910:Glioma / Q000175:diagnosis; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008297:Male; D009418:S100 Proteins / Q000032:analysis; D014376:Tuberculosis / Q000175:diagnosis; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D018949:Antigens, CD1; D015214:Antigens, Differentiation, Myelomonocytic; D000970:Antineoplastic Agents; D017338:Cladribine; D009418:S100 Proteins; C419393:CD1a antigen; C067980:CD68 antigen, human; D000097382:CD68 Molecule