Primary CNS Lymphoma

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Brain Biopsy
Frozen Section/Touch Prep
Brain Biopsy for Suspected
Neoplasm
•
•
•
•
Is it Abnormal?
Is it Neoplastic?
What Type of Neoplasm is it?
What is the Neoplasm’s Grade?
Brain Biopsy for Suspected
Neoplasm
•
•
•
•
Is it Abnormal? Yes
Is it Neoplastic? Yes
What Type of Neoplasm is it?
What is the Neoplasm’s Grade?
TOUCH PREP-GLIOBLASTOMA MULTIFORME
TOUCH PREP-CNS LYMPHOMA
TOUCH PREP-PRESENT CASE
H&E
CD20
EBER
Ki67
Diagnosis:
Malignant Lymphoma, High Grade,
Diffuse Large B Cell Type,
EBV positive
CNS Lymphomas
• Primary CNS Lymphoma
– Immunosuppressed Patients
– Immunocompetent Patients
• Secondary CNS Lymphoma
– 10% of systemic non-Hodgkin’s lymphomas
involve CNS
– Leptomeninges, epidural space are favored
locations
Primary CNS Lymphoma
• Immunosuppressed Host
– AIDS, s/p transplant, inherited immunodeficiency
– 95% EBV positive
– Median survival=1 year
• Immunocompetent Host
– Usually >60 years old
– 10% EBV positive
– Median survival=3 years
Primary CNS Lymphoma
• Epidemiology
– Incidence increased 10X worldwide due to AIDS
• Clinical
– 10% of patients have a history of another cancer
– Respond dramatically to corticosteroids
• “ghost tumor”, “sentinel lesion”
• Radiology
– Often homogeneously enhancing, periventricular
• Pathology
– Most Diffuse Large B Cell Lymphoma
– Hodgkins lymphoma=rare
• Treatment
– Gross total resection not indicated
– Biopsy for diagnosis followed by chemotherapy
Quandary
• There are no B lymphocytes normally in the
CNS
• How do you get Primary B cell lymphomas
in CNS?
Primary CNS Lymphoma:
Possible Etiologies
• B cell transformed elsewhere in body that
develops adhesion molecules specific for
CNS endothelium
• Systemic lymphoma that is eradicated by
immune system but is protected in CNS
• Clonal B cell neoplasm arising in
polyclonal intracerebral inflammatory
response
Argani’s rule of tumor pathology
Stuff happens
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