Clinical and pathological features of the new WHO-entities large B-cell lymphoma with IRF4 rearrangement and Burkitt-like lymphoma with 11q aberration within the NHL-BFM Group

Abstract

Background Two of the new entities which have been newly introduced into the Revised Fourth Edition of the WHO Classification of Tumours of Lymphoid Tissue in 2017 are relevant for pediatric oncologist, since they frequently occur in the children and adolescents: large B-cell lymphoma with IRF4 rearrangement and Burkitt-like lymphoma with 11q aberration. Clinical experience with these entities is very limited and diagnosis and management pose a challenge for pediatric oncologists. Methods We screened the files of the Haematopathology Section and Lymph Node Registry, University Hospital Schleswig-Holstein, Campus Kiel for children and adolescents of less than 18 years of age who were classified as large B-cell lymphoma with IRF4 rearrangement or Burkitt-like lymphoma with 11q aberration by fluorescence-in-situ hybridization. Pathological features are generated from central review and clinical data are collected from the files of the NHL-BFM study center. Results Among 280 patients <18 years of age with mature B-cell lymphoma that were analyzed in our reference pathology center in Kiel from 2012 to present, we identified six patients with large B-cell lymphoma with IRF4-rearrangement and 11 patients with Burkitt-like lymphoma with 11q aberration. We only included patient diagnosed after the initial publication of these lymphoma entities in 2011 and 2014, respectively. Data collection is currently ongoing and final data on clinical presentation, response to therapy and follow-up will be presented. Conclusions This study shares the clinical and pathological characteristics, management and outcome of children and adolescents diagnosed with these to new WHO entities from the NHL-BFM study group. Both entities are rare in children and adolescents and estimation of the incidences of these entities is difficult bases on our data since systematic screening has only been performed for the recent years.