Epstein-Barr Virus-Associated Primary Central Nervous System Lymphoma in the Japanese Population

Abstract

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.

Primary central nervous system lymphomas and related diseases: Pathological characteristics and discussion of the differential diagnosis

“…Kitai et al . described shorter overall survival for patients with EBV positivity in their series . We also showed a tendency of an aggressive clinical course of EBV‐positive disease .…”

“…Kitai et al . observed EBV‐encoded RNA‐1 (EBER‐1) expression in three of 22 (13.6%) primary CNS B‐cell lymphomas, and speculated that the incidence of EBV‐associated primary CNS B‐cell lymphomas in the Fukui area of Japan is higher than that previously reported from Western countries . Saglam et al .…”

“…However, in a review of the reported cases, EBV‐positive diffuse large B‐cell lymphomas of the elderly did occur in the CNS of immunocompetent patients, arising in elderly patients aged > 50 years without predisposing immunodeficiencies, suggesting that this disease in the CNS also has a relationship with an immunological deterioration derived from the aging process. Therefore, taking into consideration additional common clinicopathological features, several investigators have recently considered that EBV presence in primary CNS B‐cell lymphomas in immunocompetent elderly patients should be categorized with primary CNS EBV‐positive diffuse large B‐cell lymphomas of the elderly (Table ) …”

“…Therefore, geographical differences in the incidence of primary CNS‐EBV positive diffuse large B‐cell lymphomas of the elderly should be investigated using a large cohort study in the future. It is unknown if EBV presence in primary CNS B‐cell lymphomas is associated with patient prognosis . Kitai et al .…”

“…Recent advances in investigation of primary CNS lymphomas and related diseases have provided clinicians and pathologists with new information about these diseases . Although primary diffuse large B‐cell lymphomas are designated as primary CNS lymphomas according to the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue, a variety of other CNS lymphomas, such as primary CNS Burkitt lymphoma, primary CNS B‐cell lymphomas that have features intermediate between diffuse large B‐cell lymphomas and Burkitt lymphoma, and primary CNS EBV‐positive diffuse large B‐cell lymphoma of the elderly, have been reported in recent years. In addition, there has been controversy regarding cases of primary CNS lymphoid granulomatosis …”