Epstein-Barr virus-associated primary central nervous system lymphoma [Neurosurgery Wiki]

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====== Epstein-Barr virus-associated primary central nervous system lymphoma ======

[[Immunodeficiency]]-associated [[CNS lymphoma]]s are recognized in the ([[World Health Organization Classification of Tumors of the Central Nervous System 2016]]) as a specific subtype of [[Primary central nervous system lymphoma]]. 


This subtype of CNS lymphoma is [[Diffuse large B-cell lymphoma]] and [[Epstein–Barr virus]]-positive. Its accurate presurgical diagnosis is often unfeasible because it appears as ring-enhancing lesions mimicking [[glioblastoma]] or [[metastases]]. 

Patients without AIDS with histologically confirmed diffuse large B-cell Epstein-Barr virus-positive primary CNS lymphoma (December 2010 to January 2022) and diagnostic MR imaging without onco-specific treatment were retrospectively studied. Clinical, demographic, and conventional imaging data were reviewed. Previously published DSC-PWI time-intensity curve analysis methodology, to presurgically identify primary CNS lymphoma, was used in this particular lymphoma subtype and compared with a prior cohort of 33 patients with Epstein-Barr virus-negative CNS lymphoma, 35 with glioblastoma, and 36 with metastasis data. Normalized curves were analyzed and compared on a point-by-point basis, and previously published classifiers were tested. The standard percentage of signal recovery and CBV values were also evaluated.

Results: Seven patients with Epstein-Barr virus-positive primary CNS lymphoma were included in the study. DSC-PWI normalized time-intensity curve analysis performed the best for presurgical identification of Epstein-Barr virus-positive CNS lymphoma (area under the receiver operating characteristic curve of 0.984 for glioblastoma and 0.898 for metastasis), followed by the percentage of signal recovery (0.833 and 0.873) and CBV (0.855 and 0.687).

When a necrotic tumor is found in a potentially [[immunocompromised host]], neuroradiologists should consider [[Epstein-Barr virus-associated primary central nervous system lymphoma]]. [[DSC-PWI]] could be very useful for presurgical characterization, with especially strong performance of normalized time-intensity curves
((Pons-Escoda A, García-Ruíz A, Naval-Baudin P, Grussu F, Viveros M, Vidal N, Bruna J, Plans G, Cos M, Perez-Lopez R, Majós C. Diffuse Large B-Cell Epstein-Barr Virus-Positive Primary CNS Lymphoma in Non-AIDS Patients: High Diagnostic Accuracy of DSC Perfusion Metrics. AJNR Am J Neuroradiol. 2022 Oct 6. doi: 10.3174/ajnr.A7668. Epub ahead of print. PMID: 36202547.)).
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The incidence of Epstein-Barr virus (EBV)-associated primary central nervous system (CNS) lymphoma in Japan was assessed using in situ hybridization of EBV-encoded small ribonucleic acid-1 (EBER-1) to identify the presence of EBV in 22 cases of formalin-fixed and paraffin-embedded primary CNS lymphoma. All cases were B-cell lymphoma. EBER-1 expression was observed in the nuclei of 3 of 22 primary CNS lymphoma cases (13.6%). The incidence of EBV-positive lymphoma in Japanese cases is higher than previously reported from Western countries. Patients with EBV-positive primary CNS lymphoma showed shorter survival than those with negative tumors (median 4 months vs. 26 months). EBER-1 in situ hybridization for the detection of EBV infection is rapid and reliable. Infrequent association suggests a different pathogenetic mechanism in the evolution of these tumors. Geographical differences in the incidence of EBV-associated primary CNS lymphoma may reflect epidemiological factors
((Kitai R, Matsuda K, Adachi E, Saito Y, Nakajima T, Takeuchi H, Sato K, Imamura Y, Kubota T. Epstein-Barr virus-associated primary central nervous system lymphoma in the Japanese population. Neurol Med Chir (Tokyo). 2010;50(2):114-8. doi: 10.2176/nmc.50.114. PMID: 20185874.))