How I treat secondary CNS involvement by aggressive lymphomas

Abstract

Secondary central nervous system lymphoma (SCNSL) is a rare but clinically challenging scenario with historically disappointing outcomes. SCNSL refers to lymphoma that has spread into the CNS concurrently with systemic disease, or CNS relapse during or after frontline immunochemotherapy, presenting with or without systemic lymphoma. Diffuse large B-cell lymphoma (DLBCL) denotes the most common entity but an increased incidence is observed in other histologies such as Burkitt lymphoma and mantle cell lymphoma. The incidence, timing in disease course, location, evidence supporting the use of CNS prophylaxis, and treatment pathways varies by histology. No randomized data exist to delineate the best treatment approaches with current recommendations based on retrospective and single-arm studies. However, a regimen comprised of immunochemotherapy, incorporating agents that cross the blood-brain barrier, followed by thiotepa-containing conditioning and autologous stem-cell transplant outlined in the international MARIETTA study demonstrated improvement in outcomes representing a major accomplishment in the care of DLBCL patients with SCNSL. Anti-CD19 chimeric antigen receptor T-cell denotes a paradigm shift in the treatment of patients with systemic aggressive lymphomas with emerging data also demonstrating efficacy without higher neurotoxicity in those with SCNSL. In this manuscript we discuss five clinical scenarios and review the evidence supporting our recommendations.

Altered Mental Status in Cancer

“…45 Workup for CNS involvement of lymphoma also includes MRI of the brain and spine with contrast, LP with flow cytometry or cytology, and in many cases biopsy. 46 Though any systemic lymphoma may spread to the CNS, histology at highest risk of secondary CNS involvement include Burkitt's lymphoma ($ 20%) and diffuse large B cell lymphoma (DLBCL, $5%). 46 Among patients with DLBCL, the incidence of CNS involvement increases to 15% in patients who have activated B cell molecular subtype, and who fall under the "high-risk" category according to the CNS International Prognostic Index (CNS-IPI), which accounts for age, LDH, extranodal sites, and other variables.…”

“…46 Though any systemic lymphoma may spread to the CNS, histology at highest risk of secondary CNS involvement include Burkitt's lymphoma ($ 20%) and diffuse large B cell lymphoma (DLBCL, $5%). 46 Among patients with DLBCL, the incidence of CNS involvement increases to 15% in patients who have activated B cell molecular subtype, and who fall under the "high-risk" category according to the CNS International Prognostic Index (CNS-IPI), which accounts for age, LDH, extranodal sites, and other variables. 47 A rare manifestation of lymphoma that bears mention is intravascular large B cell lymphoma, wherein lymphoma cells affect small and medium sized blood vessels, leading to end-organ dysfunction, commonly involving the CNS and skin.…”

Optimising Rehabilitation Strategies for Primary Central Nervous System Neoplasm-Related Neurological Deficits Using a Comprehensive Multimodality Approach Including Robotic Gloves Technology: A Comprehensive Case Report

“…Hemiparesis can occur due to the tumour compressing or invading the motor cortex, which is responsible for controlling voluntary movements of the body [ 3 ]. Primary neoplasm of the CNS can occur in people of all ages, but it is more common in older adults, and specific to gender, it is more common in males than females [ 4 ]. The exact cause of the primary neoplasm of the CNS is not known, but it is believed to be related to a weakened immune system [ 5 ].…”