Primary Central Nervous System Lymphomatoid Granulomatosis: Systemic Review

Abstract

Lymphomatoid granulomatosis (LYG) is an infrequent lymphoproliferative disease that typically involves the lungs, but may also affect the central nervous system (CNS). Isolated CNS involvement is very rare, and its clinicopathological features have not been fully elucidated. Here, we systematically reviewed the English literature through PubMed to collect all relevant case reports and small case series with pathologically confirmed primary CNS-LYG. A total of 29 relevant articles with 40 cases were included in this systemic review. In cases where T cells and B cells were compared, T cells were predominant in 19 (79.2%), and B cells were predominant in 5 (20.8%). The overall infection rate of EBV was 48.1% (13/27), among which the infection rate was 40.9% (9/22) in immunocompetent patients and 80% (4/5) in immunodeficient (HIV-infected) patients. Among the patients who underwent pathological grading, 35.7% (5/14) were at grade I, 42.9% (6/14) were at grade II, and 21.4% were at grade III. In conclusion, primary CNS-LYG is closely related to EBV infection and some cases may be predominantly T-cell phenotype. Surgical resection may be effective for mass-like lesions, although there is still a lack of standard therapeutic regimen. Accurate grading of lesions is essential for treatment selection and prognosis evaluation.

The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee

“…162,163 Isolated central nervous system (CNS) or gastrointestinal tract involvement by an EBV-positive lesion resembling LyG is observed usually in the context of known causes of defective immune surveillance (EBV latency III). 164,165 In this scenario, the diagnosis of EBVpositive polymorphic B-cell LPD or EBV-positive DLBCL, NOS should be rendered.…”

Lymphomatoid granulomatosis in one patient with newly diagnosed HIV infection and Kaposi’s sarcoma: a case report and literature review

“…The pathogenesis of EBV-associated lymphomas including LYG in people with HIV is considered the result of the concerted action of different factors, mainly including impaired immune surveillance, genetic alterations, viral infection, and chronic B-cell activation. LYG may be present in other immunosuppressive states than HIV, for example, patients with chronic lymphocytic leukemia [8][9][10][11][12][13][14].…”

“…Treatment selection is based on histologic grade and underlying pathobiology with low-grade disease hypothesized to be immune-dependent and typically polyclonal and highgrade disease to be immune independent and typically oligoclonal or monoclonal. Methods of augmenting the immune response to EBV in low-grade LYG include treatment with interferon-α2b, whereas high-grade disease almost always requires immunochemotherapy [9,15].…”

Unpacking the CNS Manifestations of Epstein-Barr Virus: An Imaging Perspective

“…The differential diagnosis for LG includes CNS lymphoma and vasculitis. 50,51 Imaging findings are nonspecific and overlap with multiple tumoral and nontumoral pathologies, including glioblastoma, vasculitis, and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). [50][51][52][53][54] Treatment options include observation, resection, chemoradiation, corticosteroids, interferon, immunoglobulin, and rituximab.…”