title: CNS lymphomatoid granulomatosis with lymph node and bone marrow involvements.
abstract: Lymphomatoid granulomatosis (LYG) in the CNS is an uncommon lymphoproliferative disease with characteristic angiocentric lymphoreticular proliferative and granulomatous lesions exhibiting low-grade malignant potential. Here we report a rare case of CNS-LYG, which disseminated to the lymph node and bone marrow. A 50-year-old man was diagnosed with CNS-LYG based on brain biopsy showing perivascular infiltration of CD3-positive small T-lymphocytes without overt nuclear atypism. Eight months after the initial neurological symptoms, inguinal lymph node swelling was found and histopathologically diagnosed as peripheral T-cell lymphoma. TCRgamma-gene rearrangement study using both paraffin-embedded specimens of brain and inguinal lymph node demonstrated an identical clonal band. Considering the clinical course, we concluded lymph node involvement of CNS-LYG, suggesting the malignant potential of CNS-LYG.
authors.affiliation: Laboratory of Molecular and Cellular Pathology, Hokkaido University School of Medicine, Kita-Ku, Sapporo, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Takiyama
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nishihara
authors.forename: Ukihide
authors.initials: U
authors.lastname: Tateishi
authors.forename: Taichi
authors.initials: T
authors.lastname: Kimura
authors.forename: Lei
authors.initials: L
authors.lastname: Wang
authors.forename: Wang
authors.initials: W
authors.lastname: Lei
authors.forename: Katsuji
authors.initials: K
authors.lastname: Marukawa
authors.forename: Tomoo
authors.initials: T
authors.lastname: Itoh
authors.forename: Satoshi
authors.initials: S
authors.lastname: Hashino
authors.forename: Kazuo
authors.initials: K
authors.lastname: Nagashima
authors.forename: Shinya
authors.initials: S
authors.lastname: Tanaka
mesh_terms: D001853:Bone Marrow / Q000473:pathology*; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology*; D053144:Cell Nucleus Shape; D015329:Gene Rearrangement, T-Lymphocyte; D019675:Genes, T-Cell Receptor gamma; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology*; D016411:Lymphoma, T-Cell, Peripheral / Q000473:pathology; D008230:Lymphomatoid Granulomatosis / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D018807:Polymorphism, Single-Stranded Conformational; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology