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

Abstract

Although primary diffuse large B-cell lymphomas of the CNS are designated as primary CNS lymphomas according to the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue in 2008, a variety of other lymphomas (Burkitt lymphomas, EBV-positive diffuse large B-cell lymphoma of the elderly) and related diseases (lymphomatoid granulomatosis) that are also found in the CNS have been spotlighted in recent years. The histopathology of primary CNS Burkitt lymphomas mimics that of primary diffuse large B-cell lymphomas of the CNS after steroid administration. Therefore, for correct diagnosis of the involved lymphoma, comprehensive fluorescent in situ hybridization analysis for c-MYC and BCL2 is recommended in all primary CNS lymphoma cases with aggressive clinical course, multifocal involvement of the CNS, and a high proliferation index. The pathological characteristics of primary CNS EBV-positive diffuse large B-cell lymphoma of the elderly have similarities with those of the latency phenotype III, EBV lymphoproliferative disorders that arise in the setting of immunodeficiency. These age-related lymphomas usually occur in elderly immunocompetent patients, and the incidence of this disease was estimated to range from 4.0% to 13.6% of all primary CNS lymphomas. Shorter overall survival has been reported for patients with this disease. Lymphomatoid granulomatosis (LYG) is a systemic, EBV-driven, angiocentric and angiodestructive lymphoproliferative disorder. Primary LYG that shows distinct clinicopathological features compared with systemic LYG was recently reported. Finally, this review focuses on the relationship between primary CNS lymphomas and demyelinating diseases, and the concomitant use of intraoperative cytology and frozen sections that are helpful in rapid intraoperative diagnosis.

Biopsy‐proven case of Epstein–Barr virus (EBV)‐associated vasculitis of the central nervous system

“…In this case, steroids and anti‐herpes virus agents did not appear to have any significant effects on EBV infection. Previous studies suggested the use of high‐dose antiviral therapy with azidothymidine and ganciclovir in patients with EBV‐positive primary CNS lymphomas . In addition, anti‐herpes viral therapy with acyclovir or foscarnet sodium may be proposed and corticosteroid therapy also appears to be reasonable when vasculitis is suspected.…”

The perivascular microenvironment in Epstein–Barr virus positive primary central nervous system lymphoma: The role of programmed cell death 1 and programmed cell death ligand 1

“…In addition, in a recent review of reported cases, EBV‐positive DLBCLs of the elderly have been shown to occur in the CNS of immunocompetent patients, arising in elderly patients aged >50 years without predisposing immunodeficiencies . A large cohort study of the prevalence and clinicopathological features of EBV‐positive primary CNS DLBCLs of the elderly, reported that 4% to 13.6% of cases expressing EBV sequences by in situ hybridization occur in immunocompetent patients . Therefore, it is important to consider the role of EBV in the pathogenesis of PCNSLs.…”

A Small Case Series of Intravascular Large B-Cell Lymphoma with Unexpected Findings: Subset of Cases with Concomitant Extravascular Central Nervous System (CNS) Involvement Mimicking Primary CNS Lymphoma

“…Primary CNS B-cell lymphoma, also a rare variant of DLBCL, has a proclivity to show angiocentric proliferation of neoplastic B cells [26]. In one instance, the perivascular growth pattern in the CNS in a patient with established IVLBCL resulted in an entirely separate diagnosis of primary CNS lymphoma being made, potentially creating unnecessary confusion [6].…”