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Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma
Elizabeth Huai-Feng Li1, Claire Davila2, Connor Zuraski3
1School of Medicine, UC San Diego, San Diego, CA, United States.
Frontiers in Radiology
|December 10, 2024
Summary
Cerebral lymphomatosis (CL), a rare brain lymphoma, can present with superimposed primary central nervous system lymphoma (PCNSL). This case highlights that PCNSL may develop within diffuse CL, impacting diagnosis and treatment strategies.
Area of Science:
- Neuropathology
- Neuroradiology
- Oncology
Background:
- Cerebral lymphomatosis (CL) is a rare central nervous system lymphoma characterized by diffuse infiltration of atypical lymphoid cells.
- Primary central nervous system lymphoma (PCNSL) typically presents as a discrete mass, differing from the diffuse pattern of CL.
Observation:
- A 66-year-old woman presented with subacute cognitive decline and weakness, initially showing diffuse white matter abnormalities on MRI without a discrete mass.
- Cerebrospinal fluid studies revealed lymphocytosis and elevated IL-10.
- Despite treatment, her condition worsened, and autopsy revealed diffuse CL with superimposed areas of frank PCNSL.
Findings:
- The case demonstrates a unique presentation of diffuse CL with concurrent, localized PCNSL.
- Radiologic findings initially suggested diffuse CL, but later MRI showed new restricted diffusion and hyperperfusion in specific areas, corresponding to autopsy-confirmed PCNSL.
- MR spectroscopy indicated a hypercellular process with elevated choline and decreased NAA.
Implications:
- This case challenges the distinct classification of CL, suggesting it may represent a precursor or background for PCNSL development.
- Early consideration of diffuse CL in patients with subacute neurological decline and diffuse leukoencephalopathy on MRI is crucial.
- The findings underscore the importance of advanced imaging and potentially biopsy for accurate diagnosis in complex CNS lymphoma cases.
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