PMID- 31187530 OWN - NLM STAT- MEDLINE DCOM- 20190620 LR - 20190620 IS - 1099-1069 (Electronic) IS - 0278-0232 (Linking) VI - 37 Suppl 1 DP - 2019 Jun TI - Rare mature B-cell lymphomas in children and adolescents. PG - 53-61 LID - 10.1002/hon.2585 [doi] AB - Pediatric-type follicular lymphoma (PTFL), pediatric nodal marginal zone lymphoma (pnMZL), and large B-cell lymphoma (LBCL) with IRF4 rearrangement have been introduced into the current World Health Organization (WHO) classification. They account for 5% to 10% of mature B-cell lymphomas in children and adolescents. Both PTFL and pnMZL predominantly affect male adolescents and usually present with localized lymphadenopathy in the head and neck region. The cells within the follicles of PTFL typically show high-grade cytology, IGH monoclonality and lack the t(14;18) chromosomal alteration. In contrast, pnMZL is characterized by progressive transformation of germinal center (PTGC)-like features and interfollicular proliferation of the cells with expansion of the marginal zones with diffuse areas. Watch and wait after complete resection seems an adequate therapy with chemotherapy restricted to incompletely resected disease. All children with PTFL and pnMZL reported, so far, survived. B-cell lymphomas presenting in the Waldeyer's ring are characterized by the expression of IRF4/MUM1 and often associated with IRF4 rearrangements. Because of the frequent diffuse component, treatment often follows current protocols for mature B-NHL. The prognosis is excellent. CI - (c) 2019 John Wiley & Sons, Ltd. FAU - Woessmann, Wilhelm AU - Woessmann W AD - Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Quintanilla-Martinez, Leticia AU - Quintanilla-Martinez L AD - Institute of Pathology and Neuropathology, Eberhard Karls University of Tubingen and Comprehensive Cancer Center, Tubingen University Hospital, Tubingen, Germany. LA - eng PT - Journal Article PT - Review PL - England TA - Hematol Oncol JT - Hematological oncology JID - 8307268 RN - 0 (Interferon Regulatory Factors) RN - 0 (interferon regulatory factor-4) SB - IM MH - Adolescent MH - Age Factors MH - Biopsy MH - Child MH - Combined Modality Therapy MH - Humans MH - Immunohistochemistry MH - Interferon Regulatory Factors/genetics/metabolism MH - Lymphoma, B-Cell/*diagnosis/epidemiology/etiology/therapy MH - Lymphoma, B-Cell, Marginal Zone/diagnosis/epidemiology/therapy MH - Lymphoma, Follicular/diagnosis/epidemiology/therapy MH - Molecular Diagnostic Techniques MH - Neoplasm Grading MH - Neoplasm Staging MH - Treatment Outcome EDAT- 2019/06/13 06:00 MHDA- 2019/06/21 06:00 CRDT- 2019/06/13 06:00 PHST- 2019/06/13 06:00 [entrez] PHST- 2019/06/13 06:00 [pubmed] PHST- 2019/06/21 06:00 [medline] AID - 10.1002/hon.2585 [doi] PST - ppublish SO - Hematol Oncol. 2019 Jun;37 Suppl 1:53-61. doi: 10.1002/hon.2585.