Brain, Primary diffuse large B cell lymphoma of the central nervous system [LMP57588]

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Diagnostic Modality: 
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Disease Category: 
Neoplasia
Diagnosis: 
Primary diffuse large B cell lymphoma of the central nervous system
Species: 
Human
Organ System: 
Central Nervous System
Gender: 
Female
Age: 
67 years
Diagnosis Code: 
ICD-10: C83.3 | ICD-O: 9680/3
Slide Contributor Information
Gao, A., Keith, J.

Keywords

Primary CNS lymphoma, PCNSL, DLBCL

Clinical History

67-year-old woman presents with rapidly progressing left-sided weakness. Neuroimaging shows a large enhancing mass lesion within the right frontal lobe. A thorough workup reveals no systemic disease.

Clinical Discussion

Primary CNS DLBCL is a unique entity defined by the WHO as an extranodal non-Hodgkin lymphoma restricted to the brain or eye; lymphomas of the dura, intravascular large B cell lymphoma, CNS involvement by systemic lymphomas, and immunodeficiency-associated lymphomas are excluded. Overall, primary CNS DLBCL is rare and most commonly occurs in older adults. Microscopically, lymphoma cells usually show a diffuse growth pattern while an angiocentric pattern and single-cell infiltration of the brain parenchyma can be seen at the periphery. The cells are large with round to irregular nuclei and prominent nucleoli. Tumour cells stain for B cell markers (CD20, 79a, etc), MUM1 (90%), bcl2 (60-80%), and sometimes CD10 (10-20%). Bcl2 is usually positive. EBV is negative, in contrast to CNS DLBCL associated with HIV or immunocompromise. Prognosis is generally unfavourable.