<unk>  CNS lymphoma (PCNSL) is a highly aggressive non-Hodgkin lymphoma confined to the CNS, including the brain, spine, cerebrospinal fluid (CSF), and eyes. Unlike other brain tumors, it often has a favorable response to both chemotherapy and radiation therapy, but compared with lymphomas outside the CNS, survival is usually inferior. Moreover, the prognosis for PCNSL that has failed first-line therapy remains poor. Although new therapeutic approaches have improved survival, the management of this disease still poses a challenge in neuro-oncology.<unk>  central nervous<unk>  lymphoma may originate from a germinal center to an early postgerminal center in a secondary lymphoid organ, which may be capable of further maturation steps. EBV is almost always associated with<unk>  CNS lymphoma in immunodeficient individuals, and latent EBV infection of B-cells leads to it's immortalization and to CNS tropism. In immunocompetent individuals, the B-cells infected with EBV are held in check by T-cells, and with the severity of immunosuppression, T cells gradually fall, leading to the B-cell proliferation and dissemination. Genes involved in the pathogenesis of<unk>  central nervous<unk>  lymphoma include IG, BCL6, CD95, PAX5, and PIM1.<unk>  central nervous<unk>  lymphoma is highly associated with Epstein-Barr virus (EBV) infection (> 90%) in immunodeficient patients (such as those with AIDS and iatrogenically immunosuppressed).<unk>  central nervous<unk>  lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter (more common in low-grade lesions). On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage. On microscopic histopathological analysis,<unk>  central nervous<unk>  lymphoma is characterized by accumulation of large atypical mononuclear cells around small calibre vascular channels with invasion of the walls of some vessels. The atypical cells have large round, oval and angulated hyperchromatic and vesicular nuclei, and a narrow rim of pale cytoplasm. An occasional mitotic figure is noted.<unk>  central nervous<unk>  lymphoma is demonstrated by positivity to tumor markers, including B lymphocyte markers, such as CD19, CD20, CD79a, Ki-67, GFAP, BCL-2, and BCL-6.<unk>  central nervous<unk>  lymphoma presents as a solitary or multiple, well circumscribed, greater than 2 cm in diameter, well-defined or infiltrating mass lesion/s that can arise in the cortex, white matter, or deep grey matter. On gross pathology, the lesions could be brownish, gray-tan, or yellow, firm, homogenous, centrally necrotic with areas of hemorrhage. The lesion is mainly located at supratentorial level, usually in the periventricular regions, infiltrating the corpus callosum and the basal ganglia. Multiple lesions are reported in 38%–55% of non-AIDS<unk>  CNS lymphomas. Multifocal intraparenchymal lesions, without a dural involvement, are very rare. Frontal lobe is affected in 20%–43% of<unk>  CNS lymphoma. Other sites iclude brain stem, cerebellum, leptomeninges, spinal cord, and eyes. They may demonstrate areas of necrosis, especially in immunodeficient patients.
Primary central nervous system lymphoma pathophysiology
Primary central nervous system lymphoma classification
Primary central nervous system lymphoma epidemiology and demographics
Primary central nervous system lymphoma medical therapy
Primary central nervous system lymphoma other diagnostic studies
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Primary central nervous system lymphoma other imaging findings
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