Primary nervous-system lymphoma

Abstract

Primary nervous-system lymphoma is a rare type of non-Hodgkin lymphoma, which is confined to the nervous system. This disease is managed quite differently from the usual treatment of either primary brain tumours or systemic non-Hodgkin lymphoma. Although whole-brain radiotherapy results in responses in more than 90% of cases, this treatment is associated with high relapse rates and with delayed neurotoxicity in elderly patients. First-generation chemotherapy regimens used successfully in systemic non-Hodgkin lymphoma (eg cyclo-phosphamide, adriamycin, vincristine, and prednisone) are ineffective in primary nervous-system lymphoma, partly because of the blood-brain barrier. Median survival of patients treated with radiotherapy alone or chemotherapy plus radiotherapy is similar, and ranges from 10 to 16 months. The addition of methotrexate-based chemotherapy has improved survival for these patients, extending median survival to more than 30 months. When used alone, methotrexate-based chemotherapy is associated with significantly fewer treatment-associated toxic effects. Leptomeningeal lymphoma and intraocular lymphoma are topics of particular relevance in primary nervous-system lymphoma and are addressed in this review.