ArticleBlood advances2022
A unifying hypothesis for PNMZL and PTFL: morphological variants with a common molecular profile.
Article in Blood advances, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 43 citations in OpenAlex.
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- DNA methylation-based classification of hematolymphoid neoplasms.Blood advances · 2026Article
- [Diagnostic challenges in pediatric lymphomas].Pathologie (Heidelberg, Germany) · 2026Review
- Extramedullary plasmacytoma in children is a genetically distinct localized neoplasia curable by surgical resection.Blood advances · 2025Article
- Indolent pediatric lymphomas/lymphoproliferative disorders: a report of the 2024 EA4HP/SH lymphoma workshop.Virchows Archiv : an international journal of pathology · 2025Review
- Genomic landscape of testicular follicular lymphoma is characterized by frequent 1p36/TNFRSF14 alterations.Blood advances · 2025Article
- Pediatric lymphomas: overview and diagnostic challenges.Virchows Archiv : an international journal of pathology · 2025Review
- Dedicated diagnostic approaches for mature B-cell non-Hodgkin lymphomas occurring in children, adolescents, and young adults.Histopathology · 2025Review
- Navigating Illumina DNA methylation data: biology versus technical artefacts.NAR genomics and bioinformatics · 2024Article
- Pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma: additional evidence to support they are a single disease with variation in the histologic spectrum.Virchows Archiv : an international journal of pathology · 2024Article
- Genetic alterations in mature B- and T-cell lymphomas - a practical guide to WHO-HAEM5.Medizinische Genetik : Mitteilungsblatt des Berufsverbandes Medizinische Genetik e.V · 2024Article
- Evolution of Lymphoma Diagnosis in the Era of Personalized Medicine: A Marriage of Pathology and Genomics for Clinical Practice.The American journal of pathology · 2023Review
- The 2022 classifications of lymphoid neoplasms : Keynote.Pathologie (Heidelberg, Germany) · 2023Review
- Review
- [Lymphomas in children and adolescents].Pathologie (Heidelberg, Germany) · 2023Review
- The clinical and molecular taxonomy of t(14;18)-negative follicular lymphomas.Blood advances · 2023Article
- The many faces of nodal and splenic marginal zone lymphomas. A report of the 2022 EA4HP/SH lymphoma workshop.Virchows Archiv : an international journal of pathology · 2023Review
- [Classification of indolent B-cell lymphomas : Novelties and open questions].Pathologie (Heidelberg, Germany) · 2023Review
- Comparison of histological and molecular features of pediatric-type follicular lymphoma and pediatric nodal marginal zone lymphoma.Virchows Archiv : an international journal of pathology · 2023Article
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Authors and funding
22 authors at 6 institutions in 4 countries.
Funding
Abstract
Pediatric nodal marginal zone lymphoma (PNMZL) is an uncommon B-cell neoplasm affecting mainly male children and young adults. This indolent lymphoma has distinct characteristics that differ from those of conventional nodal marginal zone lymphoma (NMZL). Clinically, it exhibits overlapping features with pediatric-type follicular lymphoma (PTFL). To explore the differences between PNMZL and adult NMZL and its relationship to PTFL, a series of 45 PNMZL cases were characterized morphologically and genetically by using an integrated approach; this approach included whole-exome sequencing in a subset of cases, targeted next-generation sequencing, and copy number and DNA methylation arrays. Fourteen cases (31%) were diagnosed as PNMZL, and 31 cases (69%) showed overlapping histologic features between PNMZL and PTFL, including a minor component of residual serpiginous germinal centers reminiscent of PTFL and a dominant interfollicular B-cell component characteristic of PNMZL. All cases displayed low genomic complexity (1.2 alterations per case) with recurrent 1p36/TNFRSF14 copy number-neutral loss of heterozygosity alterations and copy number loss (11%). Similar to PTFL, the most frequently mutated genes in PNMZL were MAP2K1 (42%), TNFRSF14 (36%), and IRF8 (34%). DNA methylation analysis revealed no major differences between PTFL and PNMZL. Genetic alterations typically seen in conventional NMZL were absent in PNMZL. In summary, overlapping clinical, morphologic, and molecular findings (including low genetic complexity; recurrent alterations in MAP2K1, TNFRSF14, and IRF8; and similar methylation profiles) indicate that PNMZL and PTFL are likely part of a single disease with variation in the histologic spectrum. The term "pediatric-type follicular lymphoma with and without marginal zone differentiation" is suggested.
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