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Diagnostic Slide Session 2011-2

Diagnostic Slide Session 2011-2. 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.

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Diagnostic Slide Session 2011-2

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  1. Diagnostic Slide Session 2011-2 Mark Samols1, Kari-Elise Codispoti1, Marc Rosenblum2, Barbara Crain1 1 Johns Hopkins Hospital, Baltimore MD 2 Memorial Sloan-Kettering Cancer Center, New York NY

  2. 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

  3. Autopsy Findings • Brain weight 1100 g • reference range, 1050-1550 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

  4. DIAGNOSIS?

  5. GFAP

  6. GFAP

  7. CD68

  8. CD3

  9. CD3

  10. CD20

  11. CD20

  12. CD10

  13. Diagnosis • Lymphomatosis Cerebri

  14. 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

  15. LymphomatosisCerebri • Diffuse infiltration of white matter by individual neoplastic cells • Mixed with reactive astrocytes and normal lymphocytes • Typically involves periventricular areas without bulky tumors

  16. 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. 152-157, Apr. 1999.   • 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. 282-290, Mar. 2005. • 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.  

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