A clinicopathologic analysis of primary central nervous system lymphomatoid granulomatosis: case report and literature review

Abstract

Objective To investigate the clinical, neuroimaging and histopathological features of primary central nervous system lymphomatoid granulomatosis (LG). Methods The clinical manifestation, neuroimaging, histopathological and biological features of a patient with primary central nervous system LG were presented, and the related literatures were reviewed. Results A 57⁃year⁃old male presented with memory impairment, weak in orientation, calculation, apprehension and judgment for 3 months. Magnetic resonance imaging (MRI) showed space⁃occupying lesions in bilateral frontal lobes, with T1WI isointensity and T2WI hyperintensity, and the enhancement was irregular. The lesion was slight expansive with yellow surface and gray⁃white section in color and soft texture and abundant blood supply. Microscopically, the lesion was characterized by angiocentric and angiodestructive lymphoproliferation, partly showed the structure of LG characterized by T cell predominant proliferation, macrophage infiltration, astrocyte activation, small vessel proliferation and hyalinization, and partly showed the structure of lymphoma characterized by diffuse atypical B cell proliferation, with IgK monoclonal production. Epstein⁃Barr virus (EBV) was negative. Conclusion As a precursor disease of lymphoma, LG should be considered in the differential diagnosis of both diffuse and multifocal lesions of the central nervous system. The relavance between primary central nervous system LG and EBV infection should be further discussed. DOI：10.3969/j.issn.1672⁃6731.2012.05.017

Keywords

• Lymphomatoid granulomatosis
• Central nervous system neoplasms
• Lymphoma
• Herpesvirus 4, human