Efficacy and Safety of Autologous Stem-Cell Transplantation as Part of First-Line Treatment for Newly Diagnosed Primary Central Nervous System Lymphoma: A Systematic Review and Meta-Analysis

ObjectiveThe reviewed literature supports a treatment regimen for primary central nervous system lymphoma (PCNSL) that includes induction chemotherapy, followed by one consolidation therapy. High-dose chemotherapy supported by autologous stem-cell transplantation (ASCT) is the most studied option, but its effects are controversial. The aim of this study was to evaluate the efficacy and safety of ASCT for newly diagnosed PCNSL by means of a meta-analysis.MethodsThe PubMed, Embase, and Cochrane Library databases were systematically searched for studies published until May 20, 2021. Included studies were prospective studies of patients with newly diagnosed PCNSL treated with ASCT. The pooled rates and 95% confidence intervals (CIs) were determined for all outcomes. Subgroup analysis was conducted to compare the relative risk (RR) with 95% CIs for the complete remission (CR) rate and the hazard ratios (HRs) with 95% CIs for progression-free survival (PFS) and overall survival (OS).ResultsThirteen prospective studies including 348 patients were analyzed. The pooled CR rate, overall response rate, and relapse rate were 80% (95% CI, 71–88%, I2 = 67.06%, p = 0.00), 95% (95% CI, 87–100%, I2 = 73.65%, p= 0.00), and 19% (95% CI, 15–24%, I2 = 76.18%, p = 0.00), respectively. The pooled 2- and 5-year PFS and OS rates were 74% (95% CI, 68–80%, I2 = 3.90%), 65% (95% CI, 51–77%, I2 = 74.61%), 80% (95% CI, 72–88%, I2 = 57.54%), and 69% (95% CI, 53–83%, I2 = 83.89%), respectively. Hematological toxicity and infections were more common adverse events above grade 3. The pooled treatment-related mortality was 3% (95% CI, 1–6%, I2 = 28.18%, p = 0.16). In the group analysis of ASCT compared with whole-brain radiotherapy, there were no significant differences in the CR rate (RR, 1.00, 95% CI, 0.88–1.14, p = 0.971), relapse rate (RR, 0.44, 95% CI, 0.06–3.10, p = 0.408), PFS (HR, 1.28, 95% CI, 0.81–2.01, p = 0.29), or OS (HR, 1.62, 95% CI, 0.97–2.69, p = 0.06). Cognitive functions were preserved or improved after ASCT.ConclusionsASCT is a feasible approach for consolidation with good tolerability for newly diagnosed PCNSL patients. High-quality randomized controlled trials are still needed to confirm the effects of ASCT.Systematic Review Registrationhttps://www.crd.york.ac.uk/prospero/, identifier CRD42021268422.

Primary Central Nervous System Lymphoma: A Real-World Comparison of Therapy Access and Outcomes by Hospital Setting

Background This study analyzes sociodemographic barriers for primary CNS lymphoma (PCNSL) treatment and outcomes at a public safety-net hospital versus a private tertiary academic institution. We hypothesized that these barriers would lead to access disparities and poorer outcomes in the safety-net population. Methods We reviewed records of PCNSL patients from 2007-2020 (n = 95) at a public safety-net hospital (n = 33) and a private academic center (n = 62) staffed by the same university. Demographics, treatment patterns, and outcomes were analyzed. Results Patients at the safety-net hospital were significantly younger, more commonly Black or Hispanic, and had a higher prevalence of HIV/AIDS. They were significantly less likely to receive induction chemotherapy (67% vs 86%, p = 0.003) or consolidation autologous stem cell transplantation (0% vs. 44%, p = 0.001), but received more whole-brain radiation therapy (35% vs 15%, p = 0.001). Younger age and receiving any consolidation therapy were associated with improved progression-free (PFS, p = 0.001) and overall survival (OS, p = 0.001). Hospital location had no statistical impact on PFS (p = 0.725) or OS (p = 0.226) on an age-adjusted analysis. Conclusions Our study shows significant differences in treatment patterns for PCNSL between a public safety-net hospital and an academic cancer center. A significant survival difference was not demonstrated, which is likely multifactorial, but likely was positively impacted by the shared multidisciplinary care delivery between the institutions. As personalized therapies for PCNSL are being developed, equitable access including clinical trials should be advocated for resource-limited settings.

Ibrutinib in Refractory or Relapsing Primary Central Nervous System Lymphoma: A Systematic Review

Primary Central Nervous System Lymphoma (PCNSL) is a rare variant of Non-Hodgkin Lymphoma (NHL) representing 1–2% of all NHL cases. PCNSL is defined as a lymphoma that occurs in the brain, spinal cord, leptomeninges, or eyes. Efforts to treat PCNSL by traditional chemotherapy and radiotherapy have generally been unsuccessful as a significant proportion of patients have frequent relapses or are refractory to treatment. The prognosis of patients with Refractory or Relapsed (R/R) PCNSL is abysmal. The optimal treatment for R/R PCNSL is poorly defined as there are only a limited number of studies in this setting. Several studies have recently shown that ibrutinib, a Bruton tyrosine kinase (BTK) inhibitor, has promising results in the treatment of R/R PCNSL. However, these are preliminary studies with a limited sample size. In this systematic review, we explored and critically appraised the evidence about the efficacy of the novel agent ibrutinib in treating R/R PCNSL.

Marginal Zone B-cell Lymphoma of the Gasserian Ganglion: Case Report and Review of the Literature

Primary central nervous system lymphoma (PCNSL) is a rare subtype of extranodal non-Hodgkin's lymphoma that accounts for 4% of newly diagnosed central nervous system (CNS) tumors. Most primary lymphomas of the central nervous system are of the subtype of diffuse large B-cell lymphomas, which have highly aggressive behavior and may involve the brain, leptomeninges, eyes or spinal cord without evidence of systemic disease. Primary CNS lymphomas are very rare in immunocompetent patients, but their rates are increasing. So far, only 11 primary Gasser ganglion lymphomas have been reported, with an incidence of 2.5 cases per 30,000,000 inhabitants. However, B cell lymphomas of the marginal zone of the Gasserian ganglion have been very rarely reported. We report here a clinical presentation characteristic of B cell lymphoma of the marginal zone of the Gasser ganglion in an immunocompetent patient who was treated with surgery and radiotherapy, evolving with improvement of symptoms and without recurrence in 3 months of follow-up.

PRIMARY CNS LYMPHOMA MIMICKING TUBERCULOSIS INFECTION - A CASE REPORT

Primary central nervous system lymphoma (PCNSL) is a rare variety of extra nodal non-Hodgkin lymphoma that reportedly involves leptomeninges, the brain, spinal cord, eyes, or may involve other organs systemically. We present a case of 46-yearold woman with complaints of headache and fever for three weeks, associated with right-sided weakness & altered state of consciousness for one week. The most common presentation of primary central nervous lymphoma is diffuse or multifocal supratentorial masses causing cognitive deterioration and involvement of vitreous, retina, and optic nerve. Most cases ofPCNSL are left undiagnosed due to uncommon

Primary Central Nervous System Lymphoma High Incidence And Poor Survival In Finnish Population-Based Analysis

Background: We report here the first population-based incidence rates and prognosis of primary central nervous system lymphoma (PCNSL) in Finland. Methods: Finnish Cancer Registry data by histological diagnosis and tumor location (2007-2017) for cases with diffuse large B-cell lymphoma.Results: During 2007–2017, 392 new cases of PCNSL were reported (195 males, 197 females). The average age-adjusted incidence was 0.68/100.000 person-years. Incidence for males was 0.74/100.000 and for females 0.63/100.000, respectively. The incidence was highest, 2.93/100.000, among people aged 75–79 years. Concerning all cases in 2007-2017 the 2-year age-adjusted relative survival rate was 35% and the corresponding 5-year survival rate was 28%. Among patients under the age of 70, the age-adjusted 5-year relative survival rate increased from 36% in 2007-2012 to 43% for 2013-2017. Among patients aged 70+ the corresponding survival rates were poor, 7% and 9%.Conclusions: PCNSL incidence in Finland is among the highest reported in the world. The annual increase in incidence was 2.4%. The prognosis is still dismal, especially in elderly patients.