title: Gliomatosis cerebri: cytologic and autopsy findings in a case involving the entire neuraxis.
abstract: We describe the case of a 7-year-old girl who was clinically diagnosed as having a pontine glioma based on magnetic resonance imaging studies. Neoplastic cells were identified upon cytologic examination of cerebrospinal fluid. Autopsy studies revealed an anaplastic astrocytoma (WHO grade III) diffusely infiltrating the cerebral hemispheres, brain stem, cerebellum, leptomeninges, and spinal cord to the level of the conus medullaris. The Ki-67 labeling index focally approached 30%. Although many of the neoplastic cells displayed elongated twisted nuclei reminiscent of microglia, these cells stained intensely for glial fibrillary acidic protein, supporting an astrocytic origin. Unusual features of this case of gliomatosis cerebri include involvement of the entire central neuraxis, correlation with pre-mortem lumbar puncture cytology, and a markedly elevated Ki-67 labeling index.
authors.affiliation: Department of Pathology, Duke University Medical Center, Durham, North Carolina, USA.
authors.forename: T J
authors.initials: TJ
authors.lastname: Cummings
authors.forename: C M
authors.initials: CM
authors.lastname: Hulette
authors.forename: D C
authors.initials: DC
authors.lastname: Longee
authors.forename: K S
authors.initials: KS
authors.lastname: Bottom
authors.forename: R E
authors.initials: RE
authors.lastname: McLendon
authors.forename: C T
authors.initials: CT
authors.lastname: Chu
mesh_terms: D001254:Astrocytoma / Q000378:metabolism / Q000473:pathology; D001344:Autopsy; D001921:Brain / Q000473:pathology; D016543:Central Nervous System Neoplasms / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000473:pathology*; D002555:Cerebrospinal Fluid / Q000166:cytology; D002648:Child; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D005910:Glioma / Q000134:cerebrospinal fluid / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D019394:Ki-67 Antigen / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D013116:Spinal Cord / Q000473:pathology
chemical_list: D005904:Glial Fibrillary Acidic Protein; D019394:Ki-67 Antigen