Primary central nervous system lymphomas with massive intratumoral hemorrhage: Clinical, radiological, pathological, and molecular features of six cases
Corresponding Author
Seiji Yamada
Department of Diagnostic Pathology, Fujita Health University School of Medicine, Toyoake, Japan
Correspondence: Seiji Yamada, MD, PhD, Department of Diagnostic Pathology, Fujita Health University School of Medicine 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi 470-1192, Japan. Email: [email protected]
Search for more papers by this authorJun Muto
Department of Neurosurgery, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorSachiko Iba
Department of Hematology, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorKazuya Shiogama
Division of Morphology and Cell Function, Faculty of Medical Technology, Fujita Health University School of Health Sciences, Toyoake, Japan
Search for more papers by this authorYuta Tsuyuki
Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan
Search for more papers by this authorAkira Satou
Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute, Japan
Search for more papers by this authorShigeo Ohba
Department of Neurosurgery, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorKazuhiro Murayama
Joint Research Laboratory of Advanced Medical Imaging, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorYasuo Sugita
Department of Neuropathology, St. Mary's Hospital, Kurume, Japan
Search for more papers by this authorShigeo Nakamura
Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan
Search for more papers by this authorHideaki Yokoo
Department of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Japan
Search for more papers by this authorAkihiro Tomita
Department of Hematology, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorYuichi Hirose
Department of Neurosurgery, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorTetsuya Tsukamoto
Department of Diagnostic Pathology, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorMasato Abe
Department of Pathology, School of Health Sciences, Fujita Health University, Toyoake, Japan
Search for more papers by this authorCorresponding Author
Seiji Yamada
Department of Diagnostic Pathology, Fujita Health University School of Medicine, Toyoake, Japan
Correspondence: Seiji Yamada, MD, PhD, Department of Diagnostic Pathology, Fujita Health University School of Medicine 1-98 Dengakugakubo, Kutsukake-cho, Toyoake, Aichi 470-1192, Japan. Email: [email protected]
Search for more papers by this authorJun Muto
Department of Neurosurgery, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorSachiko Iba
Department of Hematology, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorKazuya Shiogama
Division of Morphology and Cell Function, Faculty of Medical Technology, Fujita Health University School of Health Sciences, Toyoake, Japan
Search for more papers by this authorYuta Tsuyuki
Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan
Search for more papers by this authorAkira Satou
Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute, Japan
Search for more papers by this authorShigeo Ohba
Department of Neurosurgery, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorKazuhiro Murayama
Joint Research Laboratory of Advanced Medical Imaging, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorYasuo Sugita
Department of Neuropathology, St. Mary's Hospital, Kurume, Japan
Search for more papers by this authorShigeo Nakamura
Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan
Search for more papers by this authorHideaki Yokoo
Department of Human Pathology, Gunma University Graduate School of Medicine, Maebashi, Japan
Search for more papers by this authorAkihiro Tomita
Department of Hematology, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorYuichi Hirose
Department of Neurosurgery, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorTetsuya Tsukamoto
Department of Diagnostic Pathology, Fujita Health University School of Medicine, Toyoake, Japan
Search for more papers by this authorMasato Abe
Department of Pathology, School of Health Sciences, Fujita Health University, Toyoake, Japan
Search for more papers by this authorAbstract
Primary central nervous system lymphomas (PCNSLs) rarely exhibit intratumoral hemorrhage. The differential diagnosis of hemorrhagic neoplasms of the central nervous system (CNS) currently includes metastatic carcinomas, melanomas, choriocarcinomas, oligodendrogliomas, and glioblastomas. Here we present the clinical, radiological, pathological, and molecular genetic features of six cases of PCNSL associated with intratumoral hemorrhage. The median age of patients was 75 years, with male predominance. While conventional PCNSLs were associated with low cerebral blood volume (CBV), perfusion magnetic resonance imaging (MRI) revealed elevated CBV in three cases, consistent with vascular proliferation. All six cases were diagnosed pathologically as having diffuse large B-cell lymphoma (DLBCL) with a non-germinal center B-cell-like (non-GCB) phenotype; marked histiocytic infiltrates and abundant non-neoplastic T-cells were observed in most cases. Expression of vascular endothelial growth factor and CD105 in the lymphoma cells and the small vessels, respectively, suggested angiogenesis within the neoplasms. Neoplastic cells were immunohistochemically negative for programmed cell death ligand 1 (PD-L1), while immune cells in the microenvironment were positive for PD-L1. Mutations in the MYD88 gene (MYD88) (L265P) and the CD79B gene (CD79B) were detected in five and one case, respectively. As therapeutic modalities used for PCNSLs differ from those that target conventional hemorrhagic neoplasms, full tissue diagnoses of all hemorrhagic CNS tumors are clearly warranted.
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