Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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September 28, 2015
Intraventricular Primary Central Nervous System Lymphoma
- Backgounds:
- Primary CNS lymphoma is a rare tumor accounting for 4% of all brain neoplasms.
- It is always the non-Hodgkin type, and the vast majority are B-cell lymphomas.
- Intraventricular primary CNS lymphoma is very rare, with few cases reported in the literature.
- Clinical Presentation: Diffuse headache over several weeks, weight loss, and gait disturbances
- Key Diagnostic Features:
- Lesions are hypo-to-isointense to the gray matter on T1WI and iso-to-hyperintense on T2WI.
- In immunocompetent patients, there is almost always solid contrast enhancement.
- MR spectroscopy has demonstrated elevated lipid peaks combined with high Cho/Cr ratio and decreased peak of NAA.
- A further workup with CT of the chest, abdomen, and pelvis can help confirm the absence of systemic disease involvement.
- DDx:
- Metastases
- Choroid plexus carcinoma
- Ependymoma
- Meningioma
- Prognosis: Primary intraventricular lymphomas seem to have a worse prognosis compared to the more common parenchymal type.
- Treatment: Chemotherapy and radiotherapy
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