Primary cerebral non-Hodgkin's lymphoma (PCNSL): a review of new trends in management.

Primary central nervous system lymphoma (PCNSL) is a rare tumor but it is rising in incidence in both AIDS and non AIDS populations. It is a non-Hodgkin's lymphoma that usually presents itself as a brain tumor, but leptomeninges, eyes and also spinal cord are frequently affected. The management of PCNSL patients has evolved in the last years. The role of surgery has been restricted for diagnosis only because the exeresis of the tumor is ineffective owing to its multifocal and infiltrative nature. Therefore, stereotactic biopsy is the way of choice for documenting the diagnosis. The standard treatment was radiotherapy started after diagnosis and followed by chemotherapy at recurrence. PCNSL is radiosensitive and chemosensitive: about 70% of patients respond to one or other of these treatment modalities but usually PCNSL recurs locally after radiotherapy. More recently the inclusion of high doses ARA-C and Methotrexate delivered prior radiotherapy have shown significant high responses and prolonged survivals.