ABCL-100: Primary Central Nervous System Lymphoma: Experience from a Large Center in Argentina

Abstract

Context: Primary central nervous system lymphoma (PCNSL) is a rare aggressive extranodal non-Hodgkin lymphoma, and the best treatment strategy in these patients is still unknown. Objective: To compare the clinical characteristics, treatment strategies, and prognosis in patients with PCNSL. Materials and Methods: Observational retrospective study, which consecutively included adult patients with histopathological diagnosis of PCNSL between August 2010 and July 2019. Data extracted included clinical characteristics, anatomical site, laboratory parameters, treatment received, and degree of response achieved. IELSG (International Extranodal Lymphoma Study Group) score scale was calculated. Results: 16 patients were analyzed, median age was 62.6 ± 13 years, 62.5% were women. Median follow-up was 20 months. All were diffuse large B-cell lymphoma. 50% had an intermediate-high IELSG score (≥2). Most common clinical presentation was focal neurological deficit (75%). 50% had cerebral lobes compromise: temporal and frontal lobes were the most affected. Mean time from diagnosis to treatment initiation was 16 ± 9 days. First-line therapy was: 13 chemotherapy, 1 radiotherapy, and 2 refused to receive treatment. With the exception of 1 patient who received RCHOP with intraocular Methotrexate (MTX), all regimens were based on high-dose MTX (>3 gr/m2). After the first-line therapy, 64.2% had complete remission (CR), 7.1% partial response (PR), 21.4% progressive disease (PD). 33.3% presented relapse of the disease. Consolidation with autologous bone marrow transplantation was used in first remission (CR1) in 2 patients and in second remission (CR2) in 1 patient. One- and 2-year survival was 62.5% and 56%, respectively. One-year mortality in patients who did not receive treatment was 100%, compared to 23% in those who were treated (P Conclusion: Our results were similar to those previously reported. Despite numerous advances in the field of PCNSL, high chemo-radiosensitivity and early start of treatment, prognosis of these patients continues to be ominous. New therapeutic strategies based on genetic-molecular knowledge of B-cells could have a promising future.