EJC paediatric oncology最新文献

{"title":"Impact of disease burden and educational attainment on health-related unemployment among childhood cancer survivors: A mediation analysis within the Nordic SALiCCS research programme","authors":"Hanna Mogensen ,&nbsp;Javier Louro ,&nbsp;Thomas Maltesen ,&nbsp;Line Kenborg ,&nbsp;Henrik Hasle ,&nbsp;Anna Sällfors Holmqvist ,&nbsp;Sirpa Heinävaara ,&nbsp;Maria Feychting ,&nbsp;Line Elmerdahl Frederiksen ,&nbsp;Friederike Erdmann","doi":"10.1016/j.ejcped.2026.100490","DOIUrl":"10.1016/j.ejcped.2026.100490","url":null,"abstract":"<div><h3>Background</h3><div>The interplay between underlying mechanisms of long-term social consequences after childhood cancer is largely unknown. Using a mediation analysis framework, we assessed the pathways through somatic diseases, psychiatric disorders, and educational attainment on the risk of being unemployed for health reasons among adult long-term survivors of childhood cancer.</div></div><div><h3>Methods</h3><div>In a Nordic register-based cohort, we identified 8825 survivors of childhood cancer diagnosed age &lt; 20 years old in Denmark, Finland, and Sweden since 1971, and compared these with 36,729 matched individuals from the general population, and 9210 siblings. Using a mediation analysis framework allowing for multiple mediators, we estimated interventional direct and indirect effects, using risk differences (RD) with 95 % confidence intervals (CI).</div></div><div><h3>Results</h3><div>By the age of 30, the RD of health-related unemployment between survivors and the general population was 4.61 % (95 %CI 3.98–5.21); of this, 21 % was attributed to the mediating pathway through somatic diseases, a smaller proportion (6 %) was mediated through educational attainment, and only a fraction through psychiatric disorders. The proportion mediated through somatic diseases increased with age at diagnosis, whereas the pathway through educational attainment was important among survivors diagnosed at younger ages.</div></div><div><h3>Conclusions</h3><div>This three-country wide study suggests that somatic disease burden and educational attainment drive some of the increased risk of health-related unemployment among childhood cancer survivors, and the importance of these mediators varies by age at cancer diagnosis. These findings underscore the need for comprehensive survivorship care to prevent or mitigate late effects and barriers that could interfere with later possibilities for employment.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100490"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146173515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rare non-Hodgkin lymphomas in childhood and adolescence: From rare to recognized entities","authors":"Andishe Attarbaschi","doi":"10.1016/j.ejcped.2026.100497","DOIUrl":"10.1016/j.ejcped.2026.100497","url":null,"abstract":"","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100497"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147384843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Large B-cell lymphoma with IRF4-rearrangement (LBCL-IRF4+) among children, adolescents and young adults (CAYA), a narrative review","authors":"Minke HW Huibers ,&nbsp;Marijn Scheijde-Vermeulen ,&nbsp;Jan Loeffen ,&nbsp;Margreet Veening ,&nbsp;Auke Beishuizen ,&nbsp;Friederike Meyer-Wentrup ,&nbsp;Melanie M. Hagleitner","doi":"10.1016/j.ejcped.2026.100495","DOIUrl":"10.1016/j.ejcped.2026.100495","url":null,"abstract":"<div><div>Outcomes for pediatric non-Hodgkin lymphoma (NHL) have improved markedly over the past decade; however, reducing therapy-related toxicity while maintaining excellent survival remains a major challenge. This narrative review, conducted using a PCC (Population–Concept–Context) framework, summarizes current evidence on large B-cell lymphoma with <em>IRF4-</em>rearrangement (LBCL-IRF4⁺) in children, adolescents, and young adults (CAYA) below 30 years of age. Focusing on clinical presentation, pathological and molecular features, treatment strategies, and outcomes, based on literature searches in PubMed, Embase, and Web of Science performed in November 2025. LBCL-IRF4⁺ is a rare subtype, and evidence among these age groups is largely derived from small retrospective series. Available data indicate that patients with low-stage disease and/or follicular growth patterns may be candidates for treatment de-escalation, while those with advanced-stage or atypical presentations should be evaluated for underlying immunologic predispositions. This review underscores the importance of including LBCL-IRF4⁺ in future clinical trials to optimize therapy intensity and explore opportunities for safe de-escalation in selected patient groups.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100495"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147384844","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Primary central nervous system lymphomas in children and adolescents","authors":"Oussama Abla ,&nbsp;Ana Carolina Xavier","doi":"10.1016/j.ejcped.2026.100496","DOIUrl":"10.1016/j.ejcped.2026.100496","url":null,"abstract":"<div><div>Primary central nervous system lymphoma (PCNSL) is a rare and aggressive neoplasm usually of B-cell origin. Its occurrence is associated with age, with PCNSL affecting more often elderly patients. In adults, PCNSL is a non-germinal center B-cell lymphoma characterized by C5/MCD/MYD88 gene signature, and HLA I/II expression alterations. The disease behaviour differs in children, adolescent, and young adults (CAYA) or in those with an inborn or acquired immune dysregulation/dysfunction, with greater variety of histologic subtypes. Also, different genetic signatures are observed in younger patients (<em>MYD88</em> wildtype) or in those with EBV+ tumours, including higher frequency of abnormalities in checkpoint inhibitor axes, suggesting different tumour microenvironment compositions and immune escape mechanisms. Children, adolescents and young adults (CAYA) with PCNSL demonstrate more favourable outcomes, likely reflecting a superior response to therapy, whereas outcomes in adult patients remain comparatively poor. Focused research that specifically includes CAYA PCNSL is necessary to uncover additional unique molecular features and association with the immune system in an “immune-privileged” site such as the CNS. These efforts will likely lead to better diagnostic methods, prognostication and optimization of treatment protocols in children, and adolescents with PCNSL.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100496"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147384837","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Randomized, open-label, phase 2 study evaluating abemaciclib in combination with irinotecan and temozolomide in patients with relapsed or refractory Ewing sarcoma","authors":"Alba Rubio-San-Simon ,&nbsp;Marilena Cesari ,&nbsp;Melissa Bear ,&nbsp;Cristina Mata ,&nbsp;Ayumu Arakama ,&nbsp;Holly Knoderer ,&nbsp;Amanda Sykes ,&nbsp;Jonathan Boss ,&nbsp;Gertjan van Hal ,&nbsp;Antoine Italiano","doi":"10.1016/j.ejcped.2026.100525","DOIUrl":"10.1016/j.ejcped.2026.100525","url":null,"abstract":"<div><h3>Introduction</h3><div>Ewing sarcoma (EWS) is a malignant tumor of primarily bone and soft tissue in children and young adults. The CDK4/6 pathway has emerged as a potential therapeutic target in EWS. Irinotecan and temozolomide are commonly used treatments for relapsed/refractory (R/R) EWS. This Phase 2 study evaluated the potential benefit of adding abemaciclib, a CDK4/6 inhibitor, to irinotecan and temozolomide to treat R/R EWS.</div></div><div><h3>Methods</h3><div>Patients aged 1 to &lt; 40 years with R/R EWS were randomized 2:1 to receive abemaciclib plus irinotecan and temozolomide (Arm A) or irinotecan and temozolomide (Arm B). The primary endpoint was progression-free survival (PFS) assessed by a blinded independent review committee (BIRC). Secondary endpoints included PFS by investigator assessment, overall survival (OS), objective response rate (ORR), disease control rate (DCR), safety, and pharmacokinetics (PK).</div></div><div><h3>Results</h3><div>Forty-six patients were enrolled (median of two prior lines of therapy). The median PFS by BIRC was 2.8 (Arm A) and 2.9 (Arm B) months. The hazard ratio corresponding to PFS by BIRC was 0.64 (95% confidence interval: 0.28–1.45; nominal p-value = 0.28). ORR was 18.8% (Arm A) and 30.8% (Arm B), and DCR was 56.3% (Arm A) and 53.8% (Arm B). Safety profiles were consistent with the known toxicities of the individual agents; however, the abemaciclib arm showed a higher incidence of high-grade adverse events and required more dose modifications of irinotecan and temozolomide.</div></div><div><h3>Conclusions</h3><div>Abemaciclib did not meaningfully improve PFS when added to irinotecan and temozolomide in R/R EWS. Further exploration of alternative strategies is warranted.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100525"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148167360","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk factors for relapse and guidance for follow-up of extracranial immature teratomas in children: A systematic review","authors":"Dana G.M. Peters ,&nbsp;Caroline C.C. Hulsker ,&nbsp;Leendert H.J. Looijenga ,&nbsp;Ronald R. de Krijger ,&nbsp;Annemieke S. Littooij ,&nbsp;Jeanette van Leeuwen ,&nbsp;Aart J. Klijn ,&nbsp;Monica Terenziani ,&nbsp;Cecile Faure-Conter ,&nbsp;Gabriele Calaminus ,&nbsp;Annelies M.C. Mavinkurve-Groothuis","doi":"10.1016/j.ejcped.2025.100322","DOIUrl":"10.1016/j.ejcped.2025.100322","url":null,"abstract":"<div><h3>Objective</h3><div>This systematic review was performed to identify risk factors for recurrence of extracranial immature teratomas (ITs) and to provide recommendations for post-operative follow-up.</div></div><div><h3>Methods</h3><div>The search focused on risk factors for recurrent disease and/or follow-up management of extracranial IT in the paediatric population. The initial search, conducted across three databases, yielded 2131 articles. Ultimately six articles were selected for inclusion in this review, comprising a total of 379 patients.</div></div><div><h3>Results</h3><div>The six studies included were heterogeneous, comprising both retrospective and prospective designs, and were conducted across various countries and continents. The majority of patients were diagnosed with an ovarian IT and were thus female. In addition, the majority of ITs were classified as grade 3 and/or stage 1. Although, the treatment approach was predominantly surgical resection, five articles incorporated adjuvant chemotherapy for more advanced ITs. A total of 29 recurrences were documented between two- and 36-months post-treatment, with the highest incidence of recurrence occurring within the first year after treatment. Higher stage (stage II-III), higher grade (grade 3) and incomplete surgery were identified as risk factors for extracranial IT recurrence. The overall survival rates were excellent in all studies. Based on the findings our recommended follow-up guidelines are differentiated according to patients at risk and IT localisation.</div></div><div><h3>Conclusions</h3><div>This review offers insight into the risk factors associated with recurrence and provides recommendations for follow-up in paediatric extracranial IT patients. Further research is required to explore the identified associations for recurrence, with the aim of providing higher-level evidence that will further enhance the recommended follow-up regimen.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100322"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145121022","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"THE ROLE OF GENETIC COUNSELORS IN BONE MARROW FAILURE: A CASE-BASED APPROACH TO SRP72 AND DHX34 VARIANTS OF UNCLEAR SIGNIFICANCE","authors":"Passant Shaker,&nbsp;Nathan Gray,&nbsp;Michelle Boals,&nbsp;Kelsey Ray,&nbsp;Melvanique Hale,&nbsp;Sara Lewis,&nbsp;Alyssa Kennedy,&nbsp;Marcin Wlodarski","doi":"10.1016/j.ejcped.2025.100374","DOIUrl":"10.1016/j.ejcped.2025.100374","url":null,"abstract":"","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100374"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145428560","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"CANCER AND CANCER PREDISPOSITION SYNDROMES IN A POLISH INHERITED THROMBOCYTOPENIA COHORT","authors":"Bartosz Urbański ,&nbsp;Katarzyna Bąbol-Pokora ,&nbsp;Szymon Janczar ,&nbsp;Magdalena Cienkusz ,&nbsp;Michalina Jezierska ,&nbsp;Anna Tomoń ,&nbsp;Magdalena Bartnik ,&nbsp;Grażyna Wróbel ,&nbsp;Monika Richert-Przygońska ,&nbsp;Grażyna Karolczyk ,&nbsp;Katarzyna Adamczewska-Wawrzynowicz ,&nbsp;Maciej Sójka ,&nbsp;Wojciech Młynarski","doi":"10.1016/j.ejcped.2025.100376","DOIUrl":"10.1016/j.ejcped.2025.100376","url":null,"abstract":"","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100376"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145428562","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"REFRACTORY CYTOPENIA OF CHILDHOOD WITH NORMAL KARYOTYPE: LONG-TERM RESULTS OF AN OBSERVATIONAL APPROACH","authors":"Beatrice Drexler ,&nbsp;Stephan Schwarz-Furlan ,&nbsp;Irith Baumann ,&nbsp;Martina Rudelius ,&nbsp;Peter Noellke ,&nbsp;Dirk Lebrecht ,&nbsp;Senthilkumar Ramamoorthy ,&nbsp;Natalia Rotari ,&nbsp;Axel Karow ,&nbsp;Shinsuke Hirabayashi ,&nbsp;Fabian Beier ,&nbsp;Yvonne Lisa Behrens ,&nbsp;Gudrun Gohring ,&nbsp;Reinhard Kalb ,&nbsp;Marcin W. Wlodarski ,&nbsp;Brigitte Strahm ,&nbsp;Miriam Erlacher ,&nbsp;Charlotte M. Niemeyer ,&nbsp;Ayami Yoshimi","doi":"10.1016/j.ejcped.2025.100330","DOIUrl":"10.1016/j.ejcped.2025.100330","url":null,"abstract":"","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100330"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145428506","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"SINGLE-CELL AND SPATIAL TRANSCRIPTOMICS OF PEDIATRIC MYELODYSPLASTIC SYNDROME WITH REFRACTORY CYTOPENIA OF CHILDHOOD REVEAL A DISRUPTED BONE MARROW NICHE DRIVING IMPAIRED HEMATOPOIESIS","authors":"Patrycja Fryzik,&nbsp;Li-Ting Chen,&nbsp;Evelyn S. Hanemaaijer,&nbsp;Ireen J. Kal,&nbsp;Tito Candelli,&nbsp;Brigit M. te Pas,&nbsp;Nina Epskamp,&nbsp;Valerie de Haas,&nbsp;Marijn Scheijde-Vermeulen,&nbsp;Wim J. de Jonge,&nbsp;Thanasis Margaritis,&nbsp;Mirjam Belderbos","doi":"10.1016/j.ejcped.2025.100331","DOIUrl":"10.1016/j.ejcped.2025.100331","url":null,"abstract":"","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100331"},"PeriodicalIF":0.0,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145428507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}