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Diagnostic Slide Session 2011-2 Mark Samols 1, Kari-Elise Codispoti 1, Marc Rosenblum 2, Barbara...
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Transcript of Diagnostic Slide Session 2011-2 Mark Samols 1, Kari-Elise Codispoti 1, Marc Rosenblum 2, Barbara...
Diagnostic Slide Session 2011-2Mark Samols1, Kari-Elise Codispoti1,
Marc Rosenblum2, Barbara Crain1
1 Johns Hopkins Hospital, Baltimore MD2 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, 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
DIAGNOSIS?
GFAP
GFAP
CD68
CD3
CD3
CD20
CD20
CD10
Diagnosis
• Lymphomatosis Cerebri
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
LymphomatosisCerebri
• 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. 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.