Primary peripheral T-Cell lymphoma of the central nervous system

A 47-year-old man was admitted to our hospital with a nine-week history of visual disturbance, headache, disorientation and facial nerve palsy. Serial cranial magnetic resonance imaging (MRI) revealed progressive bilateral occipital edema with hemorrhage and meningeal involvement. There were no hints of hereditary or acquired immunodeficiency. Laboratory examination for bacterial and viral causes was negative. Open brain biopsy revealed primary central nervous system lymphoma of the extraordinary rare so-called "peripheral" T-cell type. The further course was fatal; the patient died 10 weeks after the onset of symptoms from tumor progression before planned chemotherapy could be started. Conclusion: If primary central nervous system lymphoma (PCNSL) is suspected, brain biopsy - either open biopsy or stereotactic biopsy - should be performed straight away to enable a rapid start of chemotherapy and/or radiotherapy. Peripheral T-cell lymphoma was highly aggressive in this case leading to the patient's death within several weeks.