How is progressive or recurrent primary central nervous system lymphoma (PCNSL) treated?

Updated: Jan 19, 2018
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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The management of progressive (ie, methotrexate failure) or recurrent PCNSL is not yet well established. The age and performance status of the patient must be considered. In general, a greater than 25% enlargement of previous areas of gadolinium-contrast enhancement, the appearance of new contrast-enhancing lesions, or the appearance of malignant cells in the CSF, vitreous or, rarely, elsewhere in the body, constitutes treatment failure. Treatment options include the following:

  • Return from monthly (maintenance) to biweekly methotrexate therapy

  • Consider IV cytarabine

  • Radiation therapy is probably the best second-line anti-PCNSL treatment

  • Chemotherapy failure indicates that combined therapy is necessary, accepting that the risk of neurotoxicity from radiation and chemotherapy is considerable

Until the efficacy of repeat intensive methotrexate therapy is established in relapsed disease or until other chemotherapeutic agents are tested more extensively, whole-brain radiation therapy with 4000 cGy in 20-25 daily treatments is recommended.

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