How is 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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Answer

Answer

The optimal treatment for PCNSL has not been established. Standard systemic chemotherapy regimens such as CHOP (ie, cyclophosphamide, doxorubicin, vincristine, prednisone) are ineffective, which presumably reflects the difficulty of penetration of the blood-brain barrier by chemotherapeutic drugs.

Methotrexate is the single most effective chemotherapeutic agent for PCNSL.

Initial chemotherapy without radiation therapy results in excellent initial tumor response rates and avoids the toxicity associated with combined modality therapy (ie, radiation and chemotherapy).

The following recommendations are based on an ongoing protocol at the author's and several other institutions, and on observation that radiation therapy alone, while initially effective, produces a median survival duration of only 18 months. Combination chemotherapy and radiation therapy more than doubled survival time, but such success was achieved at the price of a greater than 50% incidence of dementia in patients who survived more than 18 months on these regimens.

The decision to offer chemotherapy as the sole initial treatment modality, therefore, must be made while keeping in mind that optimal dose and timing are still under investigation. Current active protocols for the treatment of PCNSL have been described by Hoang-Xuan and Delattre.


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