Primary central nervous system lymphoma: Clinical characteristics and therapeutic management

Primary central nervous system lymphoma (PCNSL) is a rare neoplasm but its incidence is increasing among immunocompetent and immunodeficient individuals. The peak of incidence occurs in the 6th and 7th decades in immunocompetent patients. Most patients present with symptoms of expansive cerebral mass. PCNSL can localise in brain, meninx, eyes and spinal cord. Generally, it is a B-cell monoclonal lymphoma of intermediate- or high-grade. PCNSL has a rapid growth, generally limited to CNS. Despite the high complete response rate obtained with conventional treatment (i.e. radiotherapy and corticosteroids), 80% of patients relapse within the first year of follow-up. Optimal radiotherapy seems to consist of whole brain irradiation with 45-50 Gy plus a boost with 10-15 Gy to the bulky area. Chemotherapy significantly improved the therapeutic outcomes. Any immunocompetent patient with PCNSL may be treated with a combined medality that include an initial chemotherapy followed by adequate radiotherapy. The salvage therapy seems to prolong the survival of relapsed patients.