Primary CNS lymphoma

• Primary CNS lymphoma is usually diffuse large B cell lymphoma. It accounts for <1% of all non-Hodgkin’s lymphoma subtypes.
• Clinical features include:

  • Seizures
  • Headache
  • Altered mental status
  • Systemic symptoms: fever, night sweats, unintentional weight loss
  • Diplopia
  • Vertigo
  • Dysphagia
• Relevant investigations include:

  • MRI +/- CT
  • HIV testing: CD4 count, viral load
  • Lumbar puncture: may show increased WCC and/or cytology positive for lymphoma
  • Epstein-Barr virus PCR: may identify Epstein-Barr virus if it is present
• Management options for primary CNS lymphoma include:

  • Chemotherapy: usually methotrexate, cytarabine may be used in addition
  • Radiation therapy: whole-brain radiation therapy alongside chemotherapy
  • Steroids: dexamethasone
  • Surgery: tumours are typically not resectable. However, if mass effect is present, resection may be considered as a life-sparing procedure.
• The prognosis is usually poor. In patients co-diagnosed with AIDS, median survival is four months with radiotherapy. With methotrexate-based chemotherapy, median survival is 10-18 months.