Primary central nervous system lymphoma
Central Nervous System Neoplasms
Primary central nervous system lymphoma (PCNSL) is a rare non-Hodgkin lymphoma restricted to the nervous system, affecting the brain, cerebrospinal fluid, eyes, or spinal cord. It occurs with increased frequency in immunocompromised patients, particularly those with human immunodeficiency virus infection. Diagnosis is usually established by stereotactic biopsy as resection is not an essential component of therapy. Initial treatment should include chemotherapy, particularly regimens containing high-dose methotrexate, which is the single most important drug for the treatment of PCNSL. Complete response rates can approach 80% but, even with vigorous therapy, relapse is common. Median survival is approximately 3-4 years with high-dose methotrexate-based regimens with or without cranial irradiation. Combined modality treatment is associated with a high rate of neurotoxicity, with older age and long follow-up as the primary risk factors. Current approaches have focused on reducing relapse rates and improving disease control while minimizing neurotoxicity. These include: (1) reducing the dose of cranial irradiation in patients who have achieved a complete response to chemotherapy; (2) intensifying chemotherapy using autologous stem cell rescue regimens; and (3) intensifying standard chemotherapy alone. © 2012 Elsevier B.V.
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