Comprehensive approach to diagnosis and treatment of newly diagnosed primary CNS lymphoma

Abstract

Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that affects the brain parenchyma, spinal cord, eyes, and cerebrospinal fluid without evidence of systemic, non-CNS involvement. PCNSL is uncommon and only a few randomized trials have been completed in the first-line setting. Over the past decades, the prognosis of PCNSL has improved, mainly due to the introduction and widespread use of high-dose methotrexate, which is now the backbone of all first-line treatment polychemotherapy regimens. Despite this progress, durable remission is recorded in only 50% of patients, and therapy can be associated with significant late neurotoxicity. Here, we overview the epidemiology, clinical presentation, staging evaluation, prognosis, and current up-to-date treatment of immunocompetent PCNSL patients.

Keywords: PCNSL; diagnosis; methotrexate; staging; therapy.

Fig. 1

PCNSL imaging pattern on MRI. (A) MRI T1 sequence with gadolinium contrast (T1+c) reveals homogeneously enhancing deep lesions. (B) Lesions are iso- to hyperintense on T2 imaging with a relatively small amount of edema. (C) Diffusion-weighted imaging (DWI) demonstrates restricted diffusion in the tumor.

Fig. 2

PCNSL is highly chemosensitive. (A) PCNSL is highly chemosensitive with dramatic response on T1 with gadolinium (B) after initiation of methotrexate combination therapy.