Primary central nervous system lymphoma (PCNSL) is a nervous-system-seeking extranodal non-Hodgkin's lymphoma whose incidence has increased in both immunocompromised and immunocompetent patients. Corticosteroids are used for symptomatic management but can interfere with pathological diagnosis. The well-established treatments such as radiotherapy, chemotherapy or a combination of both remain the mainstays. Traditionally, the most important role for surgery is to obtain and sample adequate tissue to confirm a diagnosis with stereotactic biopsy. Though no benefit could be demonstrated for debulking surgery, a subset of patients with large space occupying lesions which could be well circumscribed and surgically accessible may benefit from tumor resection. Moreover, surgical procedures to carry out interstitial chemotherapy and/or brachytherapy with or without tumor resection hold promise. Complications such as hydrocephalus could be managed by ventriculoperitoneal shunting. Further application of surgical procedures in the treatment of PCNSL is recommended with caution and strict patient selection criteria, and should be used in combination with radiotherapy and/or chemotherapy.

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