Pediatric Mature T/NK-cell neoplasms
2023-06-25 Sheng Xiao, MD , Chunxiao Yang Affiliation1.Brigham and Women's Hospital , Harvard Medical School, Boston , MA (USA)
2. Brigham and Women's Hospital, Harvard Medical School, Boston , MA (USA)
Classification
Definition
Two diseases are grouped under pediatric mature T/NK-cell neoplasms: pediatric peripheral T-cell lymphoma, not otherwise specified (pPTCL-NOS) and pediatric aggressive NK-cell leukemia (pANKL). pPTCL-NOS is a diverse and heterogeneous group of lymphomas that do not fit into any specific defined subtype of T-cell lymphoma. While pediatric patients may commonly present with systemic symptoms such as fever, night sweats, and weight loss, adult PTCL-NOS may have a higher frequency of nodal involvement or extranodal manifestations.1,2
pANKL is a high-risk form of leukemia characterized by its aggressive nature. Studies indicate that the median survival for ANKL is typically around 2-3 months post-diagnosis.3 However, due to the extreme rarity of pANKL, specific survival rates are unknown.4
| Pediatric Mature T/NK-cell neoplasms | Genetic marker(s) | |
|---|---|---|
| Peripheral T-cell lymphoma NOS | PTCL-NOS is a diverse group of mature T-cell lymphomas that do not fit into any specific PTCL category and is typically exhibiting an aggressive clinical course. IDH2, RHOA, TET2, CDKN2A, CDKN2B, and TP53 are mutated in pediatric PTCL, NOS, although are less frequent as compared to their adult counterparts. Chromosome aberrations include complex karyotypes with both numerical and structural changes.5,6 | |
| Aggressive NK-cell leukemia | ANKL is neoplastic NK cell proliferation with an acute presentation. Activation of the JAK/STAT and the RAS/MAPK signaling plays a central role in tumorigenesis of aggressive NK cell leukemia. Common mutations include TP53 (34%), DDX3X (29%), TET2 (28%), CREBBP (21%), KMT2D (21%), STAT3(17%-21%).7-10 TRBV20OR9-2 clonality is typically negative. Recurrent cytogenetic aberrations include del(6q), del(11q), del(7p), del(17p), and 1q gain.11,12 |
Article Bibliography
| Reference Number | Pubmed ID | Last Year | Title | Authors |
|---|---|---|---|---|
| 1 | 22829967 | 2012 | Pediatric T- and NK-cell lymphomas: new biologic insights and treatment strategies. | El-Mallawany NK et al |
| 2 | 33576080 | 2021 | Comprehensive genomic analysis identifying heterogeneity in peripheral T-cell lymphoma. | Yoshida N et al |
| 3 | 33050313 | 2020 | Aggressive NK Cell Leukemia: Current State of the Art. | El Hussein S et al |
| 4 | 35795499 | 2022 | A Case Report on Aggressive Natural Killer Cell Leukemia in a Pediatric Patient. | Donato UM Jr et al |
| 5 | 34363798 | 2021 | Clinicopathological features and prognostic significance of programmed death ligand 1 in pediatric ALK-positive anaplastic large cell lymphoma: results of the ALCL99 treatment in Japan. | Iwafuchi H et al |
| 6 | 34390161 | 2021 | Molecular features of non-anaplastic peripheral T-cell lymphoma in children and adolescents. | Au-Yeung RKH et al |
| 7 | 27631517 | 2017 | EBV-negative Aggressive NK-cell Leukemia/Lymphoma: Clinical, Pathologic, and Genetic Features. | Nicolae A et al |
| 8 | 29263771 | 2017 | The Cost of Hematopoietic Stem-Cell Transplantation in the United States. | Broder MS et al |
| 9 | 29148541 | 2018 | Integrated genomic analysis identifies deregulated JAK/STAT-MYC-biosynthesis axis in aggressive NK-cell leukemia. | Huang L et al |
| 10 | 32590457 | 2020 | Genomic and Immunophenotypic Landscape of Aggressive NK-Cell Leukemia. | El Hussein S et al |
| 11 | 17261497 | 2007 | Aggressive natural killer cell leukemia: report of a Chinese series and review of the literature. | Ryder J et al |
| 12 | 16049916 | 2005 | Genome-wide array-based comparative genomic hybridization of natural killer cell lymphoma/leukemia: different genomic alteration patterns of aggressive NK-cell leukemia and extranodal Nk/T-cell lymphoma, nasal type. | Nakashima Y et al |
Citation
Sheng Xiao, MD ; Chunxiao Yang
Pediatric Mature T/NK-cell neoplasms
Atlas Genet Cytogenet Oncol Haematol. 2023-06-25
Online version: http://atlasgeneticsoncology.org/solid-tumor/209169/pediatric-mature-t-nk-cell-neoplasms
