There are many names for primary central nervous system lymphoma (PCNSL), such as reticulum cell sarcoma, diffuse histiocytic lymphoma, and microglioma. Initially, there was uncertainty about the origin of the cell, which is reflected in the proliferation of names. PCNSL is an extranodal, high-grade non-Hodgkin B-cell neoplasm that usually consists of large cells or immunoblasts. Brain tissue, cerebrospinal fluid, the spinal cord, or the eyes can produce it. The disease typically affects the CNS, but systemic manifestations may occur in 4 – 7% of patients with newly diagnosed PCNSL and 10% of patients with relapsed PCNSL. A high level of suspicion is required to diagnose this disease since the affected areas of the CNS differ from patient to patient. PCNSL accounts for 4 – 6% of extranodal lymphomas as well as 4% of newly diagnosed CNS tumors. The condition is more common in males than in females, and it can affect both immunocompromised and immunocompetent patients. Despite its lymphocyte origin, PCNSL should be considered a brain tumor because its therapeutic challenges are the same as those associated with other brain tumors. Because of the bloodbrain barrier, drug delivery is impaired, and treatment modalities are limited by cerebral toxicity. Approximately 90% of PCNSLs are diffuse large B-cell lymphomas (DLBCL); the remaining 10% are T-cells, mantle cells, Burkitt lymphomas, or indolent B-cell lymphomas. Depending on the histologic subtype, 5-year survival rates range from 30% in DLBCL to 79% in marginal zone lymphoma. As mentioned, PCNSL is a rare lymphoma that occurs in the CNS and has a low chance of spreading to other parts of the body. It is often difficult for patients with PCNSL to achieve a positive outcome. Since PCNSL is uncommon, we know little about its optimal treatment. The standard treatment for PCNSL patients who can tolerate it is chemotherapy based on high-dose methotrexate (HDMTX). Even though whole-brain radiotherapy (WBRT) can lead to remission in 90% of patients, it is often associated with poor long-term disease control when given alone and with delayed neurotoxicity when given after HD-MTX. PCNSL is prevalent in the elderly, and they account for the majority of cases. Patients older than 70 years have experienced a median survival rate of 6 – 7 months over the last 40 years. It is difficult to determine treatment tolerability or predict treatment-related toxicity based on chronological age alone, and the definition of elderly is not uniform. When patients are fit, they may be able to tolerate induction, consolidation, and even high-dose chemotherapy with autologous stem cell transplantation (ASCT), whereas others with multiple comorbidities may only be able to tolerate intermediate doses of methotrexate with decreased renal and bone marrow function. There may be a benefit to maintenance treatment in the latter case. A high-dose chemotherapy alternative such as whole-brain irradiation can also have detrimental cognitive side effects on the elderly. It remains unclear what is the best treatment. To evaluate the risk of CNS and systemic toxicity associated with treatment, a comprehensive comorbidity and geriatric assessment is necessary. Optimal survival must be achieved while minimizing adverse effects. It would be helpful for future studies to assess how new agents can improve outcomes and maintain quality of life.
doi: 10.17756/jnen.2024-116
Citation: Yagnamurthy ES, Joshi FH, Rohit K, Manichandan M. 2024. Managing Primary Central Nervous System Lymphoma in Immunocompetent Elderly Patients. J Neurol Exp Neurosci 10(2): 33-38.
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