Primary Central Nervous System Lymphoma
BASIC DESCRIPTION
- Extranodal malignant lymphoma arising in the central nervous system (CNS) without concurrent systemic lymphoma
- Primarily non-Hodgkin/diffuse large B-cell lymphoma
PATHOLOGY
- Associated with Epstein–Barr virus infection in immunocompromised patients
- Primary CNS lymphoma (PCNSL) is an AIDS-defining illness
- Malignant cells infiltrate perivascular spaces and blood vessels
CLINICAL FEATURES
- Immunocompetent: mean patient age, 60 years
- Immunocompromised (HIV/AIDS, post-transplant, inherited immunodeficiency such as Wiskott–Aldrich syndrome): younger ages
- Male gender predilection
- Common presenting signs/symptoms: altered mental status, focal neurologic deficits, cognitive decline, headache
- Treatment: biopsy, chemotherapy ± radiation; steroids can offer some short-term benefits
- Poor prognosis
- Immunocompetent patients: median survival, <5 years
- Immunocompromised status, multiple lesions, and older age are negative prognosticators
IMAGING FEATURES
- General
- Imaging appearance varies with immune status
- Hypercellular mass
- Well marginated or infiltrating
- Solitary or multiple
- Nearly always enhances, with homogeneous, patchy, and/or peripheral pattern
- Usually supratentorial and involving the periventricular white or deep gray matter
- Commonly spreads across corpus callosum or involves the ventricles
- Often abuts ependymal surface with subependymal spread
- Imaging appearance varies with immune status
- CT
- Hyperdense mass
- ± Necrosis, hemorrhage
- Homogeneous/patchy enhancement on contrast-enhanced CT; may be ring-enhancing if immunocompromised
- MRI
- T1WI: homogeneously isointense to hypointense; can appear heterogeneous due to necrosis or hemorrhage in the immunocompromised
- T2WI: homogeneously isointense to hypointense; can appear heterogeneous due to necrosis or hemorrhage in the immunocompromised
- FLAIR: homogeneously isointense to hypointense
- DWI: diffusion restriction is often seen, although an unreliable differentiator of glioblastoma (GBM)
- MR spectroscopy/MR perfusion: elevated Cho, decreased NAA; less relative cerebral blood volume (rCBV) elevation than GBM
IMAGING RECOMMENDATIONS
- MRI without and with intravenous contrast including DWI and MR perfusion
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD, and Jacob A. Eitel, MD
References
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Kleinschmidt-DeMasters BK, Damek DM, Lillehei KO, et al. Epstein Barr virus-associated primary CNS lymphomas in elderly patients on immunosuppressive medications. J Neuropathol Exp Neurol 2008;67:1103–1111. doi.org/10.1097/NEN.0b013e31818beaea.
Lee IH, Kim ST, Kim HJ, et al. Analysis of perfusion weighted image of CNS lymphoma. Eur J Radiol 2010;76:48–51. doi.org/10.1016/j.ejrad.2009.05.013.
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Mansour A, Qandeel M, Abdel-Razeq H, et al. MR imaging features of intracranial primary CNS lymphoma in immune competent patients. Cancer Imaging 2014;14:22. doi.org/10.1186/1470-7330-14-22.
Matinella A, Lanzafame M, Bonometti MA, et al. Neurological complications of HIV infection in pre-HAART and HAART era: a retrospective study. J Neurol 2015;262:1317–1327. doi.org/10.1007/s00415-015-7713-8.
Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Elsevier, Philadelphia, PA; 2016.
Schlegel U, Schmidt-Wolf IG, Deckert M. Primary CNS lymphoma: clinical presentation, pathological classification, molecular pathogenesis and treatment. J Neurol Sci 2000;181: 1–12. doi.org/10.1016/s0022-510x(00)00385-3.
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