Researchers have identified a distinct subtype of primary central nervous system (PCNS) lymphoma that should be considered for surgical removal, suggesting a major shift in how this type of tumor is evaluated and managed.

Treatment for PCNS lymphoma — a rare but aggressive form of cancer in the brain that involves infection-fighting immune system cells — has traditionally been biopsy, radiotherapy, and high-dose chemotherapy with methotrexate, but surgical resection has not had a role because of the risk of damage to healthy brain tissue.

PCNS lymphoma is a form of non-Hodgkin lymphoma confined to the brain, eyes, spinal cord or tissues that cover the brain and spinal cord. It accounts for 1%-2% of central nervous system tumors, or approximately 1,400 new cases in the United States each year. It more often strikes the elderly and immunosuppressed. Ten-year survival is estimated at 10%-13%.

In a report on their study, published March 20, 2019, in World Neurosurgery, researchers from the Johns Hopkins University School of Medicine and Johns Hopkins Kimmel Cancer Center describe two subtypes of PCNS lymphomas easily distinguishable by MRI — one that is superficial and localized that might be considered for surgical removal and another that is deep-seated and diffuse and likely not suited to surgery. They estimate that about 20% of patients have the localized type of tumor and hold the potential for cure with surgery followed by treatment with methotrexate.

To see if that was the case, the researchers performed what they described as a comprehensive and systematic review of studies of PCNS lymphoma published between January 1968 and May 2018 on patients who had biopsies of their tumor or surgeries to remove them. They tracked treatment, side effects, progression-free survival and overall survival, comparing the outcomes and complications of patients who had biopsies only with those who had surgical resections.

Overall, they identified 1,291 citations and 244 manuscripts, and selected information from 24 for a focused data review. The selected studies included information on 15,280 patients and met certain criteria, such as involving human subjects, including data on at least five patients, reporting primary data, and providing survival or complication data on stereotactic biopsy versus resection of PCNS lymphoma.

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