title: Primary platelet-derived growth factor-producing, spindle-shaped diffuse large B-cell lymphoma of the skull: a case report and literature review.
abstract: A 39-year-old woman with a right frontal mass underwent a cranial bone tumor biopsy. Histopathologic examination of hematoxylin and eosin-stained slides showed spindle-shaped tumor cells in a storiform pattern, appearing somewhat like a sarcoma. However, the tumor cells were CD20-positive by immunohistochemical staining. Therefore, a diagnosis of spindle-shaped diffuse large B-cell lymphoma (Sp-DLBCL) was made. There have been at least 35 cases of Sp-DLBCL documented in the literature, and most were of the germinal center type, while the present case is the first report of a vimentin-positive primary Sp-DLBCL of the skull. The DLBCL in this case was immunohistochemically stained for six representative cytokines that might give rise to fibrosis, due to the evidence of fibroblastic proliferation. The DLBCL cells were positive for platelet-derived growth factor (PDGF), and some cells were also positive for tumor necrosis factor (TNF) α. Based on these findings, it was inferred that the PDGF and TNFα produced by DLBCL cells induced fibroblastic proliferation. The resultant conspicuous fibrosis caused interfibrous impingement on the DLBCL cells, which deformed them into a spindle shape. The present case is the first reported case of a PDGF-producing Sp-DLBCL.
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Kei-Ji
authors.initials: KJ
authors.lastname: Sugimoto
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Asami
authors.initials: A
authors.lastname: Shimada
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Kunimoto
authors.initials: K
authors.lastname: Ichikawa
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Mutsumi
authors.initials: M
authors.lastname: Wakabayashi
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Sekiguchi
authors.affiliation: Department of Pathology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Izumi
authors.affiliation: Department of Pathology, Research Hospital, The Institute of Medical Science, The University of Tokyo Japan.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Ota
authors.affiliation: Department of Hematology, Juntendo University School of Medicine Tokyo, Japan.
authors.forename: Norio
authors.initials: N
authors.lastname: Komatsu
authors.affiliation: Department of Hematology, Juntendo University Urayasu Hospital Urayasu, Japan.
authors.forename: Masaaki
authors.initials: M
authors.lastname: Noguchi
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D005355:Fibrosis / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D010982:Platelet-Derived Growth Factor / Q000096:biosynthesis*; D012888:Skull Neoplasms / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor; D010982:Platelet-Derived Growth Factor
keywords: Spindle cell; diffuse large B-cell lymphoma; platelet-derived growth factor; skull; storiform; vimentin