Diagnostic Slide Session Mark Samols 1, Kari-Elise Codispoti 1, Marc Rosenblum 2, Barbara Crain 1 1 Johns Hopkins Hospital, Baltimore MD 2 Memorial Sloan-Kettering Cancer Center, New York NY

Clinical History 96 year old female with a two-year history of an ill-defined dementia which became rapidly progressive 6 months before death PMH: HTN, COPD, CAD s/p CABG in 1999 FH: son with Huntington’s disease A brain only autopsy was performed

Autopsy Findings Brain weight 1100 g –reference range, g Moderate global atrophy Old lacunar infarcts –left frontal white matter –Left putamen No neuritic plaques –CERAD score of 0 Moderate neurofibrillary tangles in hippocampus and entorhinal cortex –Braak score of II/VI

DIAGNOSIS?

GFAP

CD68

CD3

CD20

CD10

Diagnosis Lymphomatosis Cerebri

Rare subset of primary CNS lymphoma First defined in 1999 due to resemblance to gliomatosis cerebri Presents as a rapidly progressive dementia in immunocompetent patients –EBV negative MRI shows diffuse patchy white matter enhancement with preservation of grey- white junction Image from: Rollins et al, Human Pathology 2005

Lymphomatosis Cerebri Diffuse infiltration of white matter by individual neoplastic cells –Mixed with reactive astrocytes and normal lymphocytes Typically involves periventricular areas without bulky tumors

References R. Bakshi, J. C. Mazziotta, P. S. Mischel, R. Jahan, D. B. Seligson, and H. V. Vinters, “Lymphomatosis cerebri presenting as a rapidly progressive dementia: clinical, neuroimaging and pathologic findings,” Dementia and Geriatric Cognitive Disorders, vol. 10, no. 2, pp , Apr K. E. Rollins, B. K. Kleinschmidt-DeMasters, J. R. Corboy, D. M. Damek, and C. M. Filley, “Lymphomatosis cerebri as a cause of white matter dementia,” Human Pathology, vol. 36, no. 3, pp , Mar E. Raz et al., “MRI Findings in Lymphomatosis Cerebri: Description of a Case and Revision of the Literature,” Journal of Neuroimaging, vol. 21, no. 2, pp. e183-e186, April 2011.