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.