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Pediatric lymphomas: overview and diagnostic challenges
John Kim Choi1, Leticia Quintanilla-Martinez2
1Department of Pathology, The University of Alabama at Birmingham, WP P30N, 619 19Th Street South, Birmingham, AL, 35249-7331, USA. johnchoi@uabmc.edu.
Insights
Pediatric lymphomas differ from adult types, with unique subtypes and genetic alterations. This review highlights the 2022 World Health Organization classification, focusing on diagnostic criteria and better prognoses for pediatric cases.
Area of Science:
- Hematology
- Pediatric Oncology
- Pathology
Background:
- Pediatric lymphomas are rare, accounting for only 10% of new diagnoses in the USA.
- While diagnostic criteria are shared, significant differences exist in certain pediatric lymphoma subtypes compared to adult forms.
- The World Health Organization (WHO) 2022 classification now includes specific guidelines for pediatric hematopoietic tumors.
Purpose of the Study:
- To review the WHO classification of pediatric lymphomas.
- To summarize diagnostic criteria, genetic findings, and differences from adult counterparts.
- To discuss emerging subtypes and diagnostic challenges in pediatric lymphomas.
Main Methods:
- Review of the World Health Organization (WHO) 2022 classification of pediatric hematopoietic tumors.
- Analysis of diagnostic criteria, genetic alterations, and prognostic factors for pediatric lymphomas.
- Comparative review of pediatric versus adult lymphoma subtypes.
Main Results:
- Pediatric lymphomas often exhibit distinct frequencies, genetic mutations, and prognoses, with generally better outcomes.
- Emerging B-cell lymphomas with specific gene alterations (e.g., IRF4 rearrangement, 11q gain/loss) are highlighted.
- Overlapping features suggest pediatric-type follicular lymphoma (PTFL) and pediatric nodal marginal zone lymphoma (PNMZL) may represent a single disease spectrum.
Conclusions:
- The WHO 2022 classification provides a framework for understanding pediatric lymphomas.
- Genetic insights and recognition of unique subtypes are crucial for accurate diagnosis and management.
- Further research into EBV's role and novel entities like PTFL/PNMZL is warranted.
Abstract:
Only 10% of new lymphoma diagnoses in the USA occur in children < 15 years. Although the same diagnostic criteria apply to both adult and pediatric lymphomas, there are important differences in some lymphoma subtypes. These differences are recognized by the World Health Organization (WHO) with the recent 2022 classification of pediatric tumors including pediatric hematopoietic tumors. Here, we review the WHO classification scheme for pediatric lymphomas and summarize the diagnostic criteria, recent genetic findings, and differences from their adult counterparts for some subtypes including those yet to be included as a definitive subtype. In general, there are differences in relatively frequency, genetic mutation, and prognosis with the pediatric counterpart often having better prognosis. Emerging B-cell lymphomas with recurrent gene alterations such as IRF4 rearrangement and 11q gain/loss chromosomal alterations will be reviewed. The overlapping pathological, clinical, and molecular features between pediatric-type follicular lymphoma (PTFL) and pediatric nodal marginal zone lymphoma (PNMZL) suggesting one disease with broad morphological spectrum will be discussed. The pathogenetic role of EBV in subclassifying Burkitt lymphoma is highlighted. The revised classification of the EBV-positive lymphoproliferative disorders in children is discussed. This review will focus on novel findings, areas of special interest, and diagnostic challenges in pediatric lymphomas.
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