From the Guidelines
The overall central nervous system (CNS) response to treatment for primary central nervous system (PCNS) lymphoma, specifically marginal zone lymphoma, is best achieved with high-dose methotrexate-based chemotherapy regimens, which may be combined with rituximab and other agents, and consolidation therapy with whole-brain radiation therapy or high-dose chemotherapy with autologous stem cell transplantation in eligible patients. When considering treatment options for CNS marginal zone lymphoma, it is essential to prioritize regimens that can effectively penetrate the blood-brain barrier and target the lymphoma cells within the CNS compartment 1.
Key Treatment Considerations
- High-dose methotrexate (3.5-8 g/m² every 2-3 weeks) is a common component of chemotherapy regimens for CNS marginal zone lymphoma, often combined with rituximab, which targets the CD20 antigen commonly expressed in marginal zone lymphomas 1.
- The addition of other agents such as cytarabine, thiotepa, or temozolomide may be considered based on patient factors and the specific characteristics of the lymphoma 1.
- Treatment response is assessed using the International PCNSL Collaborative Group criteria, which defines complete response as complete disappearance of all enhancing lesions on MRI with negative CSF cytology and resolution of ocular involvement if present 1.
- Consolidation therapy with whole-brain radiation therapy (23-45 Gy) or high-dose chemotherapy with autologous stem cell transplantation may be considered in eligible patients to improve long-term outcomes, with the choice of consolidation therapy depending on factors such as patient age, comorbidity, and frailty, as well as the consolidation strategy used in first-line treatment 1.
Emerging Therapies
- Immune checkpoint inhibitors such as nivolumab and pembrolizumab are being evaluated in relapsed or refractory PCNSL, with preliminary results showing acceptable toxicity and response rates 1.
- Chimeric antigen receptor T-cell (CAR-T) therapy targeting CD19 has demonstrated encouraging results in patients with relapsed or refractory PCNSL, although further investigation is needed to fully assess its efficacy and safety in this population 1.
From the Research
Overview of Primary Central Nervous System Marginal Zone Lymphoma Treatment
- Primary central nervous system (CNS) marginal zone lymphoma (MZL) is a rare and indolent lymphoma that can be cured with appropriate treatment 2, 3, 4.
- The most common treatment modalities for CNS MZL include surgery, radiation therapy, chemotherapy, and combinations of these 2, 3, 4.
Treatment Outcomes
- Complete remission can be achieved in a significant proportion of patients, with one study reporting a complete remission rate of 77% 4.
- Radiation therapy has been shown to be effective in achieving complete remission, with one case report demonstrating complete remission for 1 year and 10 months after radiation therapy 2.
- High-dose methotrexate-based regimens have also been shown to be effective in treating primary CNS lymphoma, with a pooled complete response rate of 41% across all regimens 5.
Consolidation Therapy
- Consolidation therapy with autologous stem cell transplantation (ASCT) has been associated with the best survival outcome, with a pooled 2-year overall survival of 80% and a 5-year overall survival of 77% 5.
- Whole-brain radiation therapy (WBRT) + chemotherapy is also a feasible approach for consolidation, with a pooled 2-year overall survival of 72% and a 5-year overall survival of 55% 5.
Treatment Considerations
- Treatment should be individualized based on the site and extent of disease, its resectability, and the expected adverse effects of systemic therapy 4.
- Localized treatment options, such as radiation therapy and surgery, should be considered preferentially 4.
- The risk of overtreatment should be carefully considered, and treatment should be tailored to the individual patient's needs 3.