Primary lymphoma of the central nervous system: Epidemiology, pathology and current approaches to diagnosis, prognosis and treatment

University of California, San Francisco, CA, USA.
Leukemia & lymphoma (Impact Factor: 2.89). 01/2008; 49 Suppl 1(s1):43-51. DOI: 10.1080/10428190802311441
Source: PubMed


An overview of the current approaches to the management of patients with primary central nervous system lymphoma (PCNSL) is provided. Although accumulating evidence demonstrates that PCNSL is a curable type of brain tumor, in many cases establishing the diagnosis and overcoming chemotherapeutic resistance remain significant obstacles. The issue of treatment-related neurotoxicity is also a central consideration in treatment planning. The introduction of highly active antiretroviral therapy has had a major impact on this disease in that the incidence of AIDS-related central nervous system lymphoma, once highly prevalent in the 1980s and 1990s, has now virtually disappeared. However, the problem of diagnostic delays secondary to steroid effects, radiation-induced neurotoxicity and methotrexate resistance represent unique and important problems in this disease. The use of anti-CD20 antibody in this disease represents the first application of biologically based targeted therapies for PCNSL; however, the overall impact of this modality in brain lymphoma awaits further evaluation in ongoing studies The application of proteomic as well as gene expression technologies is yielding insights into PCNSL pathogenesis, in particular specific oncogenic pathways, which may be exploited to develop new therapies.

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    • "Primary central nervous lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma accounting for 1–4% of all brain neoplasms [1] [2] [3] [4] [5] [6]. It is usually associated with immunodeficiency(most commonly HIV) but is increasingly observed in immunocompetent patients with no known etiology or risk factors [4]. "
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    ABSTRACT: Primary central nervous lymphoma(PCNSL) is a rare form of non-Hodgkin lymphoma confined to the central nervous system. Most of the lesions are supratentorial and periventricular, often involving deep structures such as corpus callosum and basal ganglion. Isolated intraventricular lymphoma is rare and only a few case reports. We report, to the best of our knowledge, the seventh case of isolated PCNSL in the fourth ventricle in an immunocompetent patient. A 61-year-old male presenting with 3 months of headache and dizziness followed with unsteady gait for days. The MR imaging of brain revealed a homogeneously enhancing lesion occupying almost the whole 4th ventricle.The tumor was removed subtotally via suboccipital craniotomy. Histopathology revealed the lesion be a diffuse large B-cell lymphoma. PCNSL is an important consideration in the differential diagnosis of intracranial mass lesion. The unusual location in surgically accessible fourth ventricle in posterior fossa, the isolation of the tumor may present a compelling indication for surgical resection. We suggest that primary lymphoma should be considered with homogenous lesions of the 4th ventricle. Also aggressive surgical resection in this surgically accessible location, instead of biopsy only, is rational. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.
    Full-text · Article · Jul 2015 · International Journal of Surgery Case Reports
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    • "Unlike systemic lymphoma, primary cerebral lymphoma (PCL) and primary intraocular lymphoma (PIOL) are subsets of primary central nervous system lymphoma (PCNSL), and they affect immunologically privileged organs. Both usually appear as a diffuse large B-cell non-Hodgkin lymphoma in which malignant lymphoid cell types not normally present in the brain or eye are detected [12]. The internal tissues of the brain and eye are usually protected from the inflammatory processes mediated by the immune system. "
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    ABSTRACT: Background Toll-like receptor (TLR) agonists have important properties that can be exploited for immunotherapy against tumors. Locally injected immunostimulatory oligodeoxynucleotides containing CpG motifs (CpG-ODNs), which are TLR9 agonists, have shown promise in cancer models. Several studies have demonstrated that these motifs have immunologic effects similar to those of bacterial DNA and can stimulate monocytes, macrophages, dendritic, and B cells, which then produce several proinflammatory cytokines. However, these CpG-ODNs appear to produce opposite effects on tumor B cells. Methods In this study, we investigated the direct effects of a murine class B CpG (1826) ODNs on lymphoma B cells in vitro and in vivo, using mouse models of non-Hodgkin B lymphomas developing in immunoprivileged sites, specifically the brain and the eye, and in subcutaneous sites. Results In vitro, CpG-ODNs produced antiproliferative and proapoptotic effects on lymphoma B cells. In vivo, it had an antitumor effect when injected into tumors in murine models of subcutaneous lymphoma (SCL) and primary cerebral lymphoma (PCL). However, its intravitreal administration into a primary intraocular lymphoma (PIOL) mouse model did not produce an antitumor effect. In vitro experiments using supernatant from mouse PIOL samples demonstrated that the PIOL molecular microenvironment inhibits the antiproliferative effect of CpG-ODNs on lymphoma B-cells. Conclusions Responsiveness to CpG stimulation differs in subcutaneous, cerebral, and ocular tumors, according to the tumoral and molecular microenvironment, and this should be considered for further therapeutic approaches.
    Full-text · Article · Apr 2013 · Journal of Experimental & Clinical Cancer Research
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    • "Primary central nervous system lymphoma has received more attention in recent years, especially for its unsatisfactory therapy and poor prognosis, resulting in increasing scientific awareness [4–6]. It is clear that its unsatisfactory therapy and poor prognosis are connected with the chemosensitivity and radiosensitivity [7, 8]. Special attention has been paid in recent years to factors related to the molecular biological characteristics of the tumor, in an attempt to predict and improve the prognosis. "
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    ABSTRACT: The aim of our study was to detect the expression of Ku80 in primary central nervous system lymphoma and to evaluate the relationship between Ku80 expression level and clinical outcomes. Thirty-eight patients with primary central nervous system lymphoma (PCNSL) were included in this retrospective study. The expression of Ku80 in tumor samples was determined by immunohistochemistry. One thousand neoplastic cells per specimen were counted. The expression levels were compared with the clinical data and statistically analyzed. The results of this study show that the expression of Ku80 can be found in the majority of PCNSLs. The mean expression level of Ku80 in 38 PCNSL is 64.1 ±24.5. A significant difference in Ku80 expression could be found between the age < 65 years group and age ≥ 65 years group (P = 0.006). Kaplan-Meier analysis revealed that patients who showed a high Ku80 expression had a significantly shorter median survival time (MST) than patients who had low Ku80 expression (P = 0.036). Patients' age, tumor location, and treatment protocol were significantly related to prognosis in PCNSL (P < 0.05). The expression of Ku80 was observed in the majority of PCNSLs. Ku80 was a predictive factor for survival in this study. In addition to Ku80, other clinical variables including age, tumor location and therapeutic protocol are correlated significantly with overall survival.
    Full-text · Article · Mar 2013 · Contemporary Oncology / Wspólczesna Onkologia
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