A Case Report of Primary CNS Lymphoma of Choroid Plexus

Primary CNS Lymphoma (PCNSL) of the choroid plexus is a very rare tumor. Its clinical and radiological manifestations are not entirely clear due to the rarity of the disease; and hence diagnosis of this condition can be delayed due lack of knowledge associated with the disease. We present a case of a 63-year-old man who presented with a few weeks of cognitive deficits with dysphasia and marked impairments in short term memory. His MRI brain showed subtle FLAIR and T1 contrast abnormalities near the lateral ventricles abutting the temporal lobe at the choroid plexus. On brain biopsy he was noted to have perivascular atypical B lymphocytes consistent with PCNSL. High-dose methotrexate, teniposide, BCNU, prednisolone and Rituximab, followed by 5 months of consolidative chemotherapy with cytarabine resulted in clinical and radiological improvement. He subsequently relapsed twice and salvage chemotherapy with consolidative radiotherapy was delivered. Unfortunately, 6 months later his condition worsened and he was referred to palliative care, and then deceased. PCNSL of the choroid plexus is very rare and cases of relapsed disease generally have poor outcome despite a variety of chemo and radiotherapy options. Targeted therapies with optimal penetration of the blood brain barrier are warranted.