title: Primary leptomeningeal meningiomatosis with widespread whorl formation.
abstract: We report a 10-year-old girl with a primary leptomeningeal tumor. She presented with a 5-week history of increased intracranial pressure, progressive cranial nerve deficits, and spinal compression signs. She had previously had a granulosa cell tumor, a benign estrogen-producing ovarian tumor, which was resected 6 months before the initial neurological symptoms developed. At autopsy, the brain and spinal cord showed diffuse neoplastic involvement of the leptomeninges. The tumor was composed of small cells with a high nucleus/cytoplasm ratio, which were immunoreactive for vimentin but not for epithelial membrane antigen or cytokeratin. In addition, the tumor contained many small cellular whorls with desmosome-like junctional complexes between the cells, suggesting that the tumor was a meningioma, basically of the meningothelial type. The term "meningeal meningiomatosis" has been used synonymously with "primary meningeal sarcomatosis." The present tumor was considered to be a rare example of "meningeal meningiomatosis" of true meningothelial cell origin.
authors.affiliation: Brain Disease Research Center, Brain Research Institute, Niigata University, 1 Asahimachi, Niigata 951-8585, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Wakabayashi
authors.forename: K
authors.initials: K
authors.lastname: Kawasaki
authors.forename: K
authors.initials: K
authors.lastname: Ono
authors.forename: K
authors.initials: K
authors.lastname: Nishiyama
authors.forename: R
authors.initials: R
authors.lastname: Tanaka
authors.forename: H
authors.initials: H
authors.lastname: Takahashi
mesh_terms: D001099:Arachnoid; D002648:Child; D017809:Fatal Outcome; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D008577:Meningeal Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D010841:Pia Mater; D012509:Sarcoma / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D014746:Vimentin / Q000378:metabolism
chemical_list: D005904:Glial Fibrillary Acidic Protein; D014746:Vimentin