Clinical Review & Education

Images in Neurology

Magnetic Resonance Imaging Observations in Primary Central Nervous System Lymphoma Justin T. Jordan, MD; Scott Plotkin, MD, PhD; Jorg Dietrich, MD, PhD

A 63-year-old woman with no medical history presented with two weeks of personality changes and social withdrawal, ignoring family, friends, and work. There were no apparent hallucinations or delusions. Her son brought her for evaluation after she began having difficulty recalling personal information and family names. On examination, the patient was awake but inattentive, with a flat affect and no spontaneous speech. With prompting, all components of her language were otherwise normal. Her concentration was

poor, as was remote and working memory. Her examination was otherwise normal for cranial nerves, strength, sensation, coordination, balance, and gait. Magnetic resonance imaging of the brain showed a symmetric enhancing lesion in the bilateral frontal lobes and anterior corpus callosum (Figure 1). Cerebral spinal fluid demonstrated normal glucose and protein levels, with 21 nucleated cells per mm3, 90% of which were lymphocytes. A stereotactic biopsy of the lesion revealed diffuse large

Figure 1. Magnetic Resonance Imaging of the Brain A

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C

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A, T2/fluid-attenuated inversion recovery symmetric hyperintensities are seen in the bilateral frontal lobes with callosal and forniceal thickening. B, Post-gadolinium T1-weighted images show homogeneous enhancement on coronal images. C, Restricted diffusion is seen on diffusion-weighted imaging. D, Apparent diffusion map hypointensity confirms restricted diffusion. 918

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Images in Neurology Clinical Review & Education

Figure 2. Stereotactic Biopsy of Brain Lesion Demonstrates Diffuse Large B-cell Lymphoma A

B

C

A and B, Hematoxylin-eosin stain (original magnification x10 [A] and x40 [B]) of biopsy shows hypercellular, infiltrative characteristic. C, Tumor stains positively for CD-20.

B-cell lymphoma (Figure 2). Cytologic examination of the cerebrospinal fluid was positive for lymphoma. Human immunodeficiency virus test results were negative. Positron emission tomography of the body revealed no other sites of disease, consistent with a diagnosis of primary central nervous system lymphoma. The patient was treated with a high-dose intravenous methotrexate-based therapy and showed rapid clinical and radiographic improvement.

Discussion Careful consideration of the magnetic resonance imaging of the brain was integral to diagnosing our patient. The lesion was unusually symmetric and prompted considerations of metabolic or toxic encephalopathies, ischemic encephalopathy, leukodysARTICLE INFORMATION Author Affiliations: Pappas Center for NeuroOncology, Department of Neurology, Massachusetts General Hospital, Boston. Corresponding Author: Justin T. Jordan, MD, Pappas Center for Neuro-Oncology, Department of Neurology, Massachusetts General Hospital, 55 Fruit St, Yawkey Bldg, Ste 9E, Boston, MA 02114 ([email protected]). Accepted for Publication: January 14, 2014. Published Online: May 26, 2014. doi:10.1001/jamaneurol.2014.70. Author Contributions: Drs Jordan and Dietrich had full access to all the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: All authors.

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trophies, or tumor. Several noteworthy characteristics, however, indicated lymphoma and guided our evaluation and management. First, the subtle mass effect on the lateral ventricles and expansion of the corpus callosum suggested this lesion was a mass. Second, the contrast enhancement was homogeneous1,2 and uniquely avid,2,3 as seen in immunocompetent patients with central nervous system lymphoma. Third, the periventricular lesion location is not specific to lymphoma but is commonly seen, 2,4 as is involvement of the corpus callosum. 3 Finally, restricted diffusion is common among central nervous system lymphoma lesions.4 Understanding disease-specific imaging characteristics may focus diagnostic considerations and subsequent investigations, aiding both patients and physicians.

Acquisition, analysis or interpretation of data: All authors. Drafting of the manuscript: All authors. Critical revision of the manuscript for important intellectual content: All authors. Administrative, technical, or material support: Plotkin, Dietrich. Study supervision: Plotkin, Dietrich. Conflict of Interest Disclosures: None reported. Additional Contributions: We thank Maria Cobos Sillero, MD, PhD, fellow in the Department of Neuropathology at the Massachusetts General Hospital, for her assistance in obtaining pathological photographs. REFERENCES

at presentation in 100 patients. J Neurooncol. 2005;72(2):169-177. 2. Roman-Goldstein SM, Goldman DL, Howieson J, Belkin R, Neuwelt EA. MR of primary CNS lymphoma in immunologically normal patients. AJNR Am J Neuroradiol. 1992;13(4):1207-1213. 3. Bühring U, Herrlinger U, Krings T, Thiex R, Weller M, Küker W. MRI features of primary central nervous system lymphomas at presentation. Neurology. 2001;57(3):393-396. 4. Yap KK, Sutherland T, Liew E, Tartaglia CJ, Pang M, Trost N. Magnetic resonance features of primary central nervous system lymphoma in the immunocompetent patient: a pictorial essay. J Med Imaging Radiat Oncol. 2012;56(2):179-186.

1. Küker W, Nägele T, Korfel A, et al. Primary central nervous system lymphomas (PCNSL): MRI features

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Magnetic resonance imaging observations in primary central nervous system lymphoma.

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