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

Autor: Mato S; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.; University of Barcelona (UB), Barcelona, Spain., Castrejón-de-Anta N; University of Barcelona (UB), Barcelona, Spain.; Hematopathology Section, Laboratory of Pathology, Hospital Clinic de Barcelona, Barcelona, Spain., Colmenero A; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.; University of Barcelona (UB), Barcelona, Spain., Carità L; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain., Salmerón-Villalobos J; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain., Ramis-Zaldivar JE; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain., Nadeu F; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain., Garcia N; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain., Wang L; Hematopathology Section, Laboratory of Pathology, Hospital Clinic de Barcelona, Barcelona, Spain., Verdú-Amorós J; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain.; Pediatric Oncology Department, Hospital Clínico Universitario, Biomedical Research Institute INCLIVA de Valencia, Valencia, Spain., Andrés M; Pediatric Oncology Department, Hospital La Fe, Valencia, Spain., Conde N; Pediatric Oncology Department, Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain., Celis V; Pediatric Oncology Department, Hospital Sant Joan de Déu, Esplugues de Llobregat, Spain., Ortega MJ; Pediatric Oncology Department, Hospital Virgen de las Nieves, Granada, Spain., Galera A; Pediatric Oncohematology Department, Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain., Astigarraga I; Pediatric Oncology Unit, Hospital Universitario Cruces Osakidetza, Biocruces Bizkaia Health Research Institute, Barakaldo, Spain., Perez-Alonso V; Pediatric Oncology Department, Hospital Universitario 12 de Octubre, Madrid, Spain., Quiroga E; Pediatric Oncology Department, Hospital Virgen del Rocio, Sevilla, Spain., Jiang A; BC Cancer's Centre for Lymphoid, Vancouver, BC, Canada., Scott DW; BC Cancer's Centre for Lymphoid, Vancouver, BC, Canada.; Division of Medical Oncology, Department of Medicine, University of British Columbia, Vancouver, BC, Canada., Campo E; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain.; University of Barcelona (UB), Barcelona, Spain.; Hematopathology Section, Laboratory of Pathology, Hospital Clinic de Barcelona, Barcelona, Spain.; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain., Balagué O; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain. obalague@clinic.cat.; University of Barcelona (UB), Barcelona, Spain. obalague@clinic.cat.; Hematopathology Section, Laboratory of Pathology, Hospital Clinic de Barcelona, Barcelona, Spain. obalague@clinic.cat.; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain. obalague@clinic.cat., Salaverria I; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain. isalaver@recerca.clinic.cat.; Centro de Investigación Biomédica en Red de Cáncer (CIBERONC), Madrid, Spain. isalaver@recerca.clinic.cat.
Jazyk: angličtina
Zdroj: Blood cancer journal [Blood Cancer J] 2024 Oct 07; Vol. 14 (1), pp. 171. Date of Electronic Publication: 2024 Oct 07.
DOI: 10.1038/s41408-024-01153-0
Abstrakt: 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-κB 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.
(© 2024. The Author(s).)
Databáze: MEDLINE
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