Primary central nervous system lymphoma: initial features, outcome, and late effects in 75 children and adolescents

Abstract

Key Points• Children with PCNSL and no immunodeficiency have a good outcome when treated by a histological subtype-driven and radiation-free protocol.• New treatment guidelines are needed for PCNSL in children and adolescents with an underlying immunodeficiency.

Primary Central Nervous System Lymphomas: A Diagnostic Overview of Key Histomorphologic, Immunophenotypic, and Genetic Features

“…In the pediatric setting (0–19 years), PCNSL is very rare, comprising approximately 1% of all PCNSL cases [ 20 ], with an incidence of 0.01 per 100,000 in the period of 2009–2013 [ 3 ]. It affects males more than females (male-to-female ratio of 1.7:1) and the median age of diagnosis is 12.5–14 years [ 21 , 22 ]. Children with immunodeficiency, either congenital or acquired, are at highest risk of developing PCNSL, although most cases of pediatric PCNSL in the last decades were reported in immunocompetent children [ 3 , 22 ].…”

“…It affects males more than females (male-to-female ratio of 1.7:1) and the median age of diagnosis is 12.5–14 years [ 21 , 22 ]. Children with immunodeficiency, either congenital or acquired, are at highest risk of developing PCNSL, although most cases of pediatric PCNSL in the last decades were reported in immunocompetent children [ 3 , 22 ]. Similar to adults, diffuse large B-cell lymphoma (DLBCL) accounts for the majority (49–70%) of PCNSL in the pediatric population ( Figure 3 B–D), followed by anaplastic large cell lymphoma (17–23%), Burkitt lymphoma (7–12%) and lymphoblastic lymphoma (7%) [ 22 , 23 ].…”

“…Its rarity and the lack of data from large prospective trials pose a challenge to determine the most appropriate treatment for pediatric PCNSL patients. However, a recent study by Attarbaschi et al showed that histological subtype-driven therapy, which included high-dose methotrexate, high-dose cytarabine, steroids, and no irradiation (except for anaplastic large cell lymphoma), resulted in a good outcome [ 22 ]. Their study reported a 5-year event-free survival (EFS) and overall survival (OS) of 74% ± 5% and 85% ± 4%, respectively [ 22 ].…”

“…However, a recent study by Attarbaschi et al showed that histological subtype-driven therapy, which included high-dose methotrexate, high-dose cytarabine, steroids, and no irradiation (except for anaplastic large cell lymphoma), resulted in a good outcome [ 22 ]. Their study reported a 5-year event-free survival (EFS) and overall survival (OS) of 74% ± 5% and 85% ± 4%, respectively [ 22 ]. These were in line with the previously reported numbers, 2-year progression-free survival (PFS) and overall survival (OS) rates of 61% and 86%, respectively [ 21 ].…”

“…PCNSL in children appears to have a better prognosis than in adults, and the better prognosis could be due to the higher tolerance of very young patients to higher doses of methotrexate and different biology of the lymphomas [ 21 ]. In their study, Attarbaschi et al found that preexisting disorders were associated with inferior EFS and OS, and that the usage of high-dose methotrexate, high-dose cytarabine, and alkylators was associated with improved prognosis [ 22 ].…”