Primary central nervous system lymphoma: Imaging features and differential diagnosis

Abstract

Primary central nervous system lymphoma (PCNSL) represents 5% of malignant primary brain tumors. The clinical presentation typically includes focal neurological symptoms, increased intracranial pressure, seizures, and psychiatric symptoms. Although histological examination remains the gold standard for diagnostic confirmation, non-invasive imaging plays a crucial role for the diagnosis. In immunocompetent individuals, PCNSL usually appears as a single, well-defined, supratentorial lesion with a predilection for periventricular areas, iso- or hypointense on T1- and T2-weighted magnetic resonance imaging, with restricted diffusion, slightly increased perfusion, and homogenous gadolinium-enhancement. Differential diagnoses include high-grade glioma and pseudotumoral demyelinating disease. In immunocompromised patients, PCNSL may present as multiple lesions, with a higher likelihood of hemorrhage and necrosis and less restricted diffusion than immunocompetent individuals. Differential diagnoses include neurotoxoplasmosis, progressive multifocal leukoencephalopathy, and cerebral abscess. Atypical forms of lymphoma are characterized by extra-axial lymphoma, lymphomatosis cerebri, and intravascular lymphoma. Extra-axial lymphoma presents as single or multiple extra-axial dural lesions with diffuse leptomeningeal contrast-enhancement. Lymphomatosis cerebri appears as an infiltrative and symmetric lesion, primarily affecting deep white matter and basal ganglia, appearing hyperintense on T2-weighted imaging, without significant contrast-enhancement or perfusion changes. Intravascular lymphoma presents as multiple rounded or oval-shaped “infarct-like” lesions, located cortically or subcortically. This study aims to highlight the imaging characteristics of PCNSL, focusing on magnetic resonance imaging and its differential diagnosis.