From: Childhood Non-Hodgkin Lymphoma Treatment (PDQ®)
PDQ Cancer Information Summaries [Internet].
Bethesda (MD): National Cancer Institute (US); 2002-.
NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.
Table 2. Major Histopathological Categories of Non-Hodgkin Lymphoma in Children and Adolescentsa
| WHO Classification | Immunophenotype | Clinical Presentation | Chromosome Abnormalities | Genes Affected | |
|---|---|---|---|---|---|
| Burkitt lymphoma | Mature B cell | Intra-abdominal (sporadic), head and neck (non-jaw, sporadic), jaw (endemic), bone marrow, CNS | t(8;14)(q24;q32), t(2;8)(p11;q24), t(8;22)(q24;q11) | MYC, TCF3, ID3, CCND3, TP53 | |
| High-grade B-cell lymphoma with 11q aberrations | Mature B cell | Nodal | 11q alteration, no MYC rearrangement | ||
| Large B-cell lymphoma with IRF4 rearrangement | Mature B cell | Nodal (typically head and neck) | Cryptic IRF1 rearrangement with IGH locus | IRF4 | |
| Diffuse large B-cell lymphoma | Mature B cell | Nodal, abdominal, bone, primary CNS (when associated with immunodeficiency), mediastinal | No consistent cytogenetic abnormality identified | ||
| Primary mediastinal (thymic) large B-cell lymphoma | Mature B cell, often CD30+ | Mediastinal, but may also have other nodal or extranodal disease (i.e., abdominal, often kidney) | 9p and 2p gains | CIITA, TNFAIP3, SOCS1, PTPN11, STAT6 | |
| ALK-positive large B-cell lymphoma | Generalized lymphadenopathy, bone marrow in 25% | t(2;5)(p23;q35); less common variant translocations involving ALK | ALK, NPM | ||
| T-lymphoblastic leukemia/lymphoma | T lymphoblasts (TdT, CD2, CD3, CD7, CD4, CD8) | Mediastinal mass, bone marrow | |||
| B-lymphoblastic leukemia/lymphoma | B lymphoblasts (CD19, CD79a, CD22, CD10, TdT) | Skin, soft tissue, bone, lymph nodes, bone marrow | |||
| Pediatric-type follicular lymphoma | Mature B cell | Nodal (typically head and neck) | TNFRSF14, MAP2K1 | ||
| Pediatric nodal marginal zone lymphoma | Mature B cell | Nodal (typically head and neck) | |||
CNS = central nervous system; TdT = terminal deoxynucleotidyl transferase; WHO = World Health Organization; + = positive.
aAdapted from Percy et al.[1]