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REVIEW
www.nature.com/clinicalpractice/neuro
Primary CNS lymphoma
Fred H Hochberg*, Joachim M Baehring and Ephraim P Hochberg
SUMMARY
REVIEW CRITERIA
We performed a search of the PubMed database for articles on primary CNS lymphoma published between 1970 and September 2006. We used combinations of the following search terms: lymphoma, NHL, brain, nervoussystem, PCNSL, ocular lymphoma, vitreal, PTLD, transplantation, intravascular, neurolymphomatosis, and diffuse large B-cell lymphoma. We selected 75 articles that we judged to be of high impact on the field. Articles not written in English were excluded.
INTRODUCTION
Primary CNS lymphoma (PCNSL) is a nervous-system-seeking extranodal non-Hodgkins lymphoma (NHL). Microscopically, PCNSL resembles systemic diffuse large B-cell lymphoma (DLBCL) and the two disorders share common molecular features; however, the underlying molecular mechanisms of PCNSL, including the causative role of somatic gene alterations, remain uncertain. Survival and functional outcome in patients with PCNSL have improved markedly since the deferral of cranial irradiation and the provision of methotrexate-based chemotherapy became standard of care, but most patients relapse, and the 5-year survival rate remains inferior to that of patients over 60 years of age with DLBCL outside the nervous system. This Review highlights advances in our understanding of PCNSL, as well as current approaches to its diagnosis and therapy.
EPIDEMIOLOGY AND RISK FACTORS Immunocompetent hostPCNSL represents 3.1% of all primary brain tumors and 23% of NHLs.1,2 After a three-fold rise observed two decades ago, the incidence of PCNSL has increased only slightly in the past 10 years in individuals above the age of 60, and now stands at 0.46 per 100,000 patient-years.1,3 A slight male predominance is observed. In our experience, a larger than expected number of patients with PCNSL have experienced prior autoimmune disease of systemic or neurologic sites (e.g. conditions resembling chronic inflammatory demyelinating polyneuropathy or multiple sclerosis) or have had therapy with corticosteroids or immunosuppressive agents.
Immunocompromised host
Patients with acquired or congenital immuno-deficiency are highly predisposed to develop PCNSL, and the disease represents the most frequent brain tumor in this patient population. EpsteinBarr virus (EBV) infection increases the risk of brain involvement in PCNSL.4 The
Non-Hodgkins lymphoma invades the brain, the vitreous body and nerves of the eye, the meninges, and the nerve roots of brain and spine, leading to the development of a primary CNS...