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This work was supported by Asociacion Espanola Contra el Cancer (PRYGN21911-SALA), Fondo de Investigaciones Sanitarias Instituto de Salud Carlos III (PI18/00471, PI21/00479) to IS, and PID2021-123054OB-I00 funded by MCIN/AEI/10.13039/501100011033 to EC and, as appropriate, by "ERDF A way of making Europe". Furthermore, the authors would like to thank the support of the Generalitat de Catalunya Support Grups de Recerca (2017-SGR-1107 and 2021-SGR-01293 to IS and 2021-SGR-01172 to EC). IS was supported by Miguel Servet II ISCIII (MS18/00015). SM holds a PFIS ISCIII fellowship (FI22/00063), AC holds an Investigo contract from Generalitat de Catalunya (100028TC7) and NG was supported by Accio instrumental d'incorporacio de cientifics fi cs i tecnolegs PERIS 2020 (SL017/20/000204) from Generalitat de Catalunya. EC is an Academia Researcher of the "Institucio Catalana de Recerca i Estudis Avancats" (ICREA) of the Generalitat de Catalunya. DWS is supported by a Michael Smith Foundation Research, Health Professional Investigator Award. This work was developed at the Centro Esther Koplowitz, Barcelona, Spain.

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MYC-rearranged mature B-cell lymphomas in children and young adults are molecularly Burkitt Lymphoma

Publicado en:Blood Cancer Journal. 14 (1): 171- - 2024-10-07 14(1), DOI: 10.1038/s41408-024-01153-0

Autores: Mato, Sara; Castrejon-de-Anta, Natalia; Colmenero, Ariadna; Carita, Lorenzo; Salmeron-Villalobos, Julia; Ramis-Zaldivar, Joan Enric; Nadeu, Ferran; Garcia, Noelia; Wang, Luojun; Verdu-Amoros, Jaime; Andres, Mara; Conde, Nuria; Celis, Veronica; Ortega, Maria Jose; Galera, Ana; Astigarraga, Itziar; Perez-Alonso, Vanesa; Quiroga, Eduardo; Jiang, Aixiang; Scott, David W; Campo, Elias; Balague, Olga; Salaverria, Itziar

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Aggressive B-cell non-Hodgkin lymphomas (NHL) in children, adolescents, and young adults (CAYA) include Burkitt lymphoma (BL), diffuse large B-cell lymphoma (DLBCL), and a subset of high-grade tumors with features intermediate between these entities whose genetic and molecular profiles have not been completely elucidated. In this study, we have characterized 37 aggressive B-NHL in CAYA, 33 with high-grade morphology, and 4 DLBCL with MYC rearrangement (MYC-R), using targeted next-generation sequencing and the aggressive lymphoma gene expression germinal center B-cell-like (GCB), activated B-cell-like (ABC), and dark zone signatures (DZsig). Twenty-two tumors had MYC-R without BCL2 breaks, and two MYC-non-R cases had BCL6 translocations. MYC-R cases, including DLBCL, carried BL-related mutations and copy number alterations. Conversely, MYC-non-R lymphomas had alterations in the B-cell receptor signaling/NF-kappa B pathway (71%). DZsig was expressed in 12/13 of MYC-R tumors but only in 2/10 of MYC-non-R GCB tumors (P < 0.001). The 3-year event-free survival (EFS) of the whole cohort was 79.6%. TP53 and KMT2C mutations conferred inferior outcome (3-year EFS P < 0.05). Overall, MYC-R lymphomas in CAYA have a molecular profile similar to BL regardless of their high-grade or DLBCL morphology, whereas MYC-non-R has more heterogeneous genetic alterations closer to that of DLBCL.

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