@article{Marius Rohde_Bettina R. Bonn_Martin Zimmermann_Jonas Lange_Anja Möricke_Wolfram Klapper_Ilske Oschlies_Monika Szczepanowski_Inga Nagel_Martin Schrappe_MMML-MYC-SYS Project_ICGC MMML-Seq Project_Markus Loeffler_Reiner Siebert_Alfred Reiter_Birgit Burkhardt_2017, place={Pavia, Italy}, title={Relevance of ID3-TCF3-CCND3 pathway mutations in pediatric aggressive B-cell lymphoma treated according to the non-Hodgkin Lymphoma Berlin-Frankfurt-Münster protocols}, volume={102}, url={https://haematologica.org/article/view/8100}, DOI={10.3324/haematol.2016.156885}, abstractNote={Mature B-cell non-Hodgkin lymphoma is the most common subtype of non-Hodgkin lymphoma in childhood and adolescence. B-cell non-Hodgkin lymphomas are further classified into histological subtypes, with Burkitt lymphoma and Diffuse large B-cell lymphoma being the most common subgroups in pediatric patients. Translocations involving the <em>MYC</em> oncogene are known as relevant but not sufficient for Burkitt lymphoma pathogenesis. Recently published large-scale next-generation sequencing studies unveiled sets of additional recurrently mutated genes in samples of pediatric and adult B-cell non-Hodgkin lymphoma patients. <em>ID3, TCF3</em> and <em>CCND3</em> are potential drivers of Burkitt lymphomagenesis. In the study herein, frequency and clinical relevance of mutations in <em>ID3, TCF3</em> and <em>CCND3</em> were analyzed within a well-defined cohort of 84 uniformly diagnosed and treated pediatric B-cell non-Hodgkin lymphoma patients of the Berlin-Frankfurt-Münster group. Mutation frequency was 78% (<em>ID3</em>), 13% (<em>TCF3</em>) and 36% (<em>CCND3</em>) in Burkitt lymphoma (including Burkitt leukemia). <em>ID3</em> and <em>CCND3</em> mutations were associated with more advanced stages of the disease in <em>MYC</em> rearrangement positive Burkitt lymphoma. In conclusion, <em>ID3-TCF3-CCND3</em> pathway genes are mutated in more than 88% of <em>MYC</em&gt;-rearranged pediatric B-cell non-Hodgkin lymphoma and the pathway may represent a highly relevant second hit of Burkitt lymphoma pathogenesis, especially in children and adolescents.}, number={6}, journal={Haematologica}, author={Marius Rohde and Bettina R. Bonn and Martin Zimmermann and Jonas Lange and Anja Möricke and Wolfram Klapper and Ilske Oschlies and Monika Szczepanowski and Inga Nagel and Martin Schrappe and MMML-MYC-SYS Project and ICGC MMML-Seq Project and Markus Loeffler and Reiner Siebert and Alfred Reiter and Birgit Burkhardt}, year={2017}, month={May}, pages={1091-1098} }