Presenting Authors: Erin K. Yeung, PharmD, BCPS, BCOP, and Brian Primeaux, PharmD, BCOP, University of Texas MD Anderson Cancer Center, Houston, TX
Co-Authors: Chelsea Luo, PharmD, BCOP, Caitlin Linger, PharmD, BCOP, Sheree Chen, PharmD, BCOP, and Bryan Do, PharmD, BCOP, University of Texas MD Anderson Cancer Center Houston, TX
BACKGROUND: Primary central nervous system lymphoma (PCNSL) is a rare and aggressive lymphoma that affects the CNS in the absence of other systemic involvement. High-dose methotrexate (HD-MTX)–based regimens are recommended as frontline treatment, followed by consolidation with high-dose chemotherapy regimens, whole brain radiation therapy (WBRT) with or without temozolomide (TMZ), or autologous stem-cell transplant (autoSCT). Despite treatment advancements with the introduction of HD-MTX and rituximab, up to half of patients will have disease relapse, 10% to 15% may have primary refractory disease, and the median survival is approximately 2 months without additional intervention. Treatment for relapsed or refractory disease can widely vary because preferred regimens in this setting are not well-established.
OBJECTIVES: The primary objective was to characterize the therapies used in relapsed or refractory PCNSL. The secondary objective was to characterize the consolidation methods used after frontline treatment.
METHODS: This retrospective, descriptive analysis included adult patients with PCNSL who received an HD-MTX–based frontline regimen between April 1, 2016, and July 1, 2022. Patients who received HD-MTX for the treatment of secondary CNS lymphoma, PCNSL arising from non–B cell origin, and intraocular lymphoma were excluded.
RESULTS: A total of 54 patients were included in this study, with a median age of 67 years. Thirty-one (57%) patients received consolidation therapy with rituximab and high-dose cytarabine (R-HDAC), WBRT, or both. Thirteen (24%) patients proceeded with autoSCT. Twenty-five patients had disease progression, with 17 patients opting for second-line treatment. The second-line treatments were WBRT (24%), clinical trial (18%), rituximab with lenalidomide (18%), re-induction with HD-MTX–based regimens (18%), ibrutinib plus rituximab (12%), and R-HDAC (12%). Seven patients had disease progression, and all received third-line treatment. The treatments varied, including rituximab with lenalidomide; ibrutinib with or without HD-MTX; rituximab, methotrexate, and cytarabine; R-HDAC; rituximab plus nivolumab; and WBRT. Five patients received a fourth-line regimen, including rituximab with or without lenalidomide, rituximab plus HD-MTX, and nivolumab monotherapy. The regimens for the 3 patients who received fifth-line treatment and beyond included rituximab plus TMZ and pembrolizumab monotherapy in addition to the previously mentioned regimens.
CONCLUSION: To our knowledge, this is the first real-world, retrospective descriptive analysis of regimen utilization for relapsed or refractory PCNSL. As evidenced by this analysis, regimen selection varies and is highly dependent on physician preference and patient factors, including clinical trial eligibility, previous therapies, performance status, organ function, and treatment intent. Prospective clinical trials are desperately needed to guide the management of this disease in patients with a poor prognosis.
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