Pub Date : 2026-06-01Epub Date: 2026-02-01DOI: 10.1016/j.ejcped.2026.100487
Markus G. Seidel , Michael H. Albert , Andishe Attarbaschi , Siobhan O. Burns , Eleonora Gambineri , Antonis Kattamis , Antonio Marzollo , Maria Ester Bernardo , Fabio Candotti , Carmelo Rizzari
The three scientific societies ESID, EHA, and SIOPe jointly organized their first focus meeting entitled “The Interplay of Inborn Errors of Immunity with Hematology,” which took place in Vienna, Austria, in November 2025 and was met with great success. The sessions addressed a wide range of topics at the interface of these overlapping fields, such as interpretation and integration of histopathological and genetics results, mechanisms of immune dysregulation and tumor predisposition, current diagnostic and therapeutic measures, and combined novel insights from biological mechanisms with clinical challenges. This brief report highlights and summarizes key aspects of the scientific program.
{"title":"The interplay of inborn errors of immunity with hematology – Report of the joint focus meeting of ESID, EHA, and SIOPE, Vienna, Austria","authors":"Markus G. Seidel , Michael H. Albert , Andishe Attarbaschi , Siobhan O. Burns , Eleonora Gambineri , Antonis Kattamis , Antonio Marzollo , Maria Ester Bernardo , Fabio Candotti , Carmelo Rizzari","doi":"10.1016/j.ejcped.2026.100487","DOIUrl":"10.1016/j.ejcped.2026.100487","url":null,"abstract":"<div><div>The three scientific societies ESID, EHA, and SIOPe jointly organized their first focus meeting entitled “The Interplay of Inborn Errors of Immunity with Hematology,” which took place in Vienna, Austria, in November 2025 and was met with great success. The sessions addressed a wide range of topics at the interface of these overlapping fields, such as interpretation and integration of histopathological and genetics results, mechanisms of immune dysregulation and tumor predisposition, current diagnostic and therapeutic measures, and combined novel insights from biological mechanisms with clinical challenges. This brief report highlights and summarizes key aspects of the scientific program.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100487"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146173516","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}
Pub Date : 2026-06-01Epub Date: 2026-02-10DOI: 10.1016/j.ejcped.2026.100491
Caroline C.C. Hulsker , Emma van der Gaag , Annelies M.C. Mavinkurve-Groothuis , Marc H.W. Wijnen , Ronald R. de Krijger , Leendert H.J. Looijenga , Henrike E. Karim-Kos , Alida F.W. van der Steeg
Background
Since 2018, cancer care for patients up to 18 years is concentrated in the Princess Máxima Center for Pediatric Oncology (Máxima). In this retrospective population-based study, we evaluated the diagnosis and treatment of malignant extracranial germ cell tumors (GCTs) presenting at our institution and elsewhere in the Netherlands between 2018 and 2022.
Methods
The files of patients with malignant extracranial GCTs diagnosed in patients aged 0–18 years, between 2018 and 2022, were retrieved from Netherlands Cancer Registry (NCR). Patient, tumor and treatment characteristics were compared in and outside the Máxima.
Results
A total of 122 cases were identified, comprising 89 gonadal (51 testicular, 38 ovarian) and 33 extragonadal GCTs. More than half of the patients (70/122, 57 %) were treated in the Máxima. Testicular GCTs were predominantly treated outside the Máxima (37/51, 73 %). Ovarian and extragonadal GCTs were mainly seen in the Máxima (31/38, 82 % and 25/33, 76 %, respectively). Patients treated outside the Máxima were older (median age 17 years outside versus 8.5 years in the Máxima), were of male gender more often (40 versus 23 boys), and received adjuvant chemotherapy less often (15 versus 35 patients). In the study period, only four patients died from their GCT (three in the Máxima, one not).
Conclusions
Since centralization, more than half of pediatric malignant extracranial GCT patients in the Netherlands have been treated in the Máxima, particularly prepubertal children, those with ovarian tumors, higher-stage disease, and complex extragonadal cases. Through a shared care center structure that fosters collaborative multidisciplinary meetings and the recent establishment of specialized care pathways, a high standard of care for pediatric malignant extracranial GCT patients in the Netherlands is ensured.
{"title":"Five years of centralized pediatric oncology care in the Netherlands: Evaluation of diagnosis and treatment of children and adolescents with a malignant extracranial germ cell tumor","authors":"Caroline C.C. Hulsker , Emma van der Gaag , Annelies M.C. Mavinkurve-Groothuis , Marc H.W. Wijnen , Ronald R. de Krijger , Leendert H.J. Looijenga , Henrike E. Karim-Kos , Alida F.W. van der Steeg","doi":"10.1016/j.ejcped.2026.100491","DOIUrl":"10.1016/j.ejcped.2026.100491","url":null,"abstract":"<div><h3>Background</h3><div>Since 2018, cancer care for patients up to 18 years is concentrated in the Princess Máxima Center for Pediatric Oncology (Máxima). In this retrospective population-based study, we evaluated the diagnosis and treatment of malignant extracranial germ cell tumors (GCTs) presenting at our institution and elsewhere in the Netherlands between 2018 and 2022.</div></div><div><h3>Methods</h3><div>The files of patients with malignant extracranial GCTs diagnosed in patients aged 0–18 years, between 2018 and 2022, were retrieved from Netherlands Cancer Registry (NCR). Patient, tumor and treatment characteristics were compared in and outside the Máxima.</div></div><div><h3>Results</h3><div>A total of 122 cases were identified, comprising 89 gonadal (51 testicular, 38 ovarian) and 33 extragonadal GCTs. More than half of the patients (70/122, 57 %) were treated in the Máxima. Testicular GCTs were predominantly treated outside the Máxima (37/51, 73 %). Ovarian and extragonadal GCTs were mainly seen in the Máxima (31/38, 82 % and 25/33, 76 %, respectively). Patients treated outside the Máxima were older (median age 17 years outside versus 8.5 years in the Máxima), were of male gender more often (40 versus 23 boys), and received adjuvant chemotherapy less often (15 versus 35 patients). In the study period, only four patients died from their GCT (three in the Máxima, one not).</div></div><div><h3>Conclusions</h3><div>Since centralization, more than half of pediatric malignant extracranial GCT patients in the Netherlands have been treated in the Máxima, particularly prepubertal children, those with ovarian tumors, higher-stage disease, and complex extragonadal cases. Through a shared care center structure that fosters collaborative multidisciplinary meetings and the recent establishment of specialized care pathways, a high standard of care for pediatric malignant extracranial GCT patients in the Netherlands is ensured.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100491"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146173517","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}
Pub Date : 2026-06-01Epub Date: 2026-02-20DOI: 10.1016/j.ejcped.2026.100498
Ana Carolina Xavier , Oussama Abla
Paediatric nodal marginal zone lymphoma (PNMZL) is a rare low-grade mature B-cell non-Hodgkin lymphoma (NHL) that primarily affects male adolescents. It has been considered a distinct entity from the nodal marginal zone lymphoma (NMZL), given distinct epidemiology and outcomes. There is strong evidence suggesting that PNMZL overlaps with paediatric-type follicular lymphoma (PTFL) histologically, and genetically with recurrent alterations affecting MAP2K1, TNFRSF14, and IRF8. Given its indolent course, most patients with PNMZL will present with limited stage usually involving the head and neck areas, with excellent outcomes with observation-only or complete surgical resection approaches. The known elevated risk of transformation into a high-grade lymphoma seen in adults with NMZL rarely occurs in PNMZL. Importantly, patients with PNMZL, especially those with extranodal involvement, should be investigated for an inborn or acquired error of immunity, or chronic inflammatory diseases. Dedicated studies designed to include paediatric patients with rare types of NHL, such as PNMZL, will help unveiling additional molecular characteristics, leading to optimization of diagnosis and treatment.
{"title":"Paediatric nodal marginal zone lymphoma","authors":"Ana Carolina Xavier , Oussama Abla","doi":"10.1016/j.ejcped.2026.100498","DOIUrl":"10.1016/j.ejcped.2026.100498","url":null,"abstract":"<div><div>Paediatric nodal marginal zone lymphoma (PNMZL) is a rare low-grade mature B-cell non-Hodgkin lymphoma (NHL) that primarily affects male adolescents. It has been considered a distinct entity from the nodal marginal zone lymphoma (NMZL), given distinct epidemiology and outcomes. There is strong evidence suggesting that PNMZL overlaps with paediatric-type follicular lymphoma (PTFL) histologically, and genetically with recurrent alterations affecting <em>MAP2K1</em>, <em>TNFRSF14</em>, and <em>IRF8</em>. Given its indolent course, most patients with PNMZL will present with limited stage usually involving the head and neck areas, with excellent outcomes with observation-only or complete surgical resection approaches. The known elevated risk of transformation into a high-grade lymphoma seen in adults with NMZL rarely occurs in PNMZL. Importantly, patients with PNMZL, especially those with extranodal involvement, should be investigated for an inborn or acquired error of immunity, or chronic inflammatory diseases. Dedicated studies designed to include paediatric patients with rare types of NHL, such as PNMZL, will help unveiling additional molecular characteristics, leading to optimization of diagnosis and treatment.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100498"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147384838","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}
Pub Date : 2026-06-01Epub Date: 2026-03-07DOI: 10.1016/j.ejcped.2026.100500
Milāna Bloha , Dārta Valdovska , Anna Raita , Liene Smane , Santa Kursīte , Elīna Dimiņa
Introduction
Health-related quality of life (HRQoL) assessment is an essential component of paediatric oncology care. The Paediatric Quality of Life Inventory (PedsQL) 3.0 Cancer Module is widely used to evaluate disease-specific HRQoL in children aged 2–18 years. This study assessed the reliability of the Latvian version of the module and examined HRQoL across age, diagnosis, and therapy intensity subgroups.
Methods
A methodological, descriptive, cross-sectional study was conducted at the Children’s Clinical University Hospital (CCUH), in Riga, from February to July 2025. Patients (2–18 years) and their parents completed the PedsQL 3.0 Cancer Module (self-report and proxy-report questionnaires). Socio-demographic and clinical data were obtained from the medical records.
Results
Seventy-five children and their families were included. Internal consistencies ranged from 0.28 to 0.94, with most domains demonstrating acceptable reliability. A tendency of lower internal consistency in self-reports was observed among younger patients. No significant differences emerged between self- and proxy-reports. The range of p-values for non-significant differences in patient proxy-reports was 0.076–1.000. Lower HRQoL scores were noted for procedural anxiety in the youngest cohorts, communication and worry in 8–12-year-olds, nausea and cognitive problems in adolescents. Proxy-reports showed significantly lower physical appearance scores for central nervous system tumours versus haematological malignancies. In self-reports, higher treatment intensity was associated with lower procedural anxiety scores.
Conclusions
The Latvian PedsQL 3.0 Cancer Module is a reliable tool for the assessment of HRQoL in paediatric oncology patients. Subgroup differences were modest, supporting its broad applicability in clinical and research settings.
{"title":"Assessment of health-related quality of life with the PedsQL 3.0 Cancer Module in Latvian paediatric oncology patients","authors":"Milāna Bloha , Dārta Valdovska , Anna Raita , Liene Smane , Santa Kursīte , Elīna Dimiņa","doi":"10.1016/j.ejcped.2026.100500","DOIUrl":"10.1016/j.ejcped.2026.100500","url":null,"abstract":"<div><h3>Introduction</h3><div>Health-related quality of life (HRQoL) assessment is an essential component of paediatric oncology care. The Paediatric Quality of Life Inventory (PedsQL) 3.0 Cancer Module is widely used to evaluate disease-specific HRQoL in children aged 2–18 years. This study assessed the reliability of the Latvian version of the module and examined HRQoL across age, diagnosis, and therapy intensity subgroups.</div></div><div><h3>Methods</h3><div>A methodological, descriptive, cross-sectional study was conducted at the Children’s Clinical University Hospital (CCUH), in Riga, from February to July 2025. Patients (2–18 years) and their parents completed the PedsQL 3.0 Cancer Module (self-report and proxy-report questionnaires). Socio-demographic and clinical data were obtained from the medical records.</div></div><div><h3>Results</h3><div>Seventy-five children and their families were included. Internal consistencies ranged from 0.28 to 0.94, with most domains demonstrating acceptable reliability. A tendency of lower internal consistency in self-reports was observed among younger patients. No significant differences emerged between self- and proxy-reports. The range of p-values for non-significant differences in patient proxy-reports was 0.076–1.000. Lower HRQoL scores were noted for procedural anxiety in the youngest cohorts, communication and worry in 8–12-year-olds, nausea and cognitive problems in adolescents. Proxy-reports showed significantly lower physical appearance scores for central nervous system tumours versus haematological malignancies. In self-reports, higher treatment intensity was associated with lower procedural anxiety scores.</div></div><div><h3>Conclusions</h3><div>The Latvian PedsQL 3.0 Cancer Module is a reliable tool for the assessment of HRQoL in paediatric oncology patients. Subgroup differences were modest, supporting its broad applicability in clinical and research settings.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100500"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147384841","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}
Pub Date : 2026-06-01Epub Date: 2026-02-06DOI: 10.1016/j.ejcped.2026.100490
Hanna Mogensen , Javier Louro , Thomas Maltesen , Line Kenborg , Henrik Hasle , Anna Sällfors Holmqvist , Sirpa Heinävaara , Maria Feychting , Line Elmerdahl Frederiksen , Friederike Erdmann
Background
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.
Methods
In a Nordic register-based cohort, we identified 8825 survivors of childhood cancer diagnosed age < 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).
Results
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.
Conclusions
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.
{"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 , Javier Louro , Thomas Maltesen , Line Kenborg , Henrik Hasle , Anna Sällfors Holmqvist , Sirpa Heinävaara , Maria Feychting , Line Elmerdahl Frederiksen , 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 < 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}
Pub Date : 2026-06-01Epub Date: 2026-02-20DOI: 10.1016/j.ejcped.2026.100497
Andishe Attarbaschi
{"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}
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 IRF4-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.
儿童非霍奇金淋巴瘤（NHL）的预后在过去十年中有显著改善；然而，减少治疗相关的毒性，同时保持良好的生存仍然是一个主要的挑战。这篇叙述性综述使用PCC （Population-Concept-Context）框架进行，总结了目前在30岁以下儿童、青少年和年轻人（CAYA）中发生irf4重排（LBCL-IRF4⁺）的大b细胞淋巴瘤的证据。重点关注临床表现、病理和分子特征、治疗策略和结果，基于2025年11月在PubMed、Embase和Web of Science进行的文献检索。LBCL-IRF4⁺是一种罕见的亚型，这些年龄组的证据主要来自小型回顾性研究。现有数据表明，低期疾病和/或卵泡生长模式的患者可能是降级治疗的候选者，而晚期或非典型表现的患者应评估潜在的免疫倾向。这篇综述强调了在未来的临床试验中包括LBCL-IRF4⁺的重要性，以优化治疗强度，并在选定的患者组中探索安全降压的机会。
{"title":"Large B-cell lymphoma with IRF4-rearrangement (LBCL-IRF4+) among children, adolescents and young adults (CAYA), a narrative review","authors":"Minke HW Huibers , Marijn Scheijde-Vermeulen , Jan Loeffen , Margreet Veening , Auke Beishuizen , Friederike Meyer-Wentrup , 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}
Pub Date : 2026-06-01Epub Date: 2026-02-20DOI: 10.1016/j.ejcped.2026.100496
Oussama Abla , Ana Carolina Xavier
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 (MYD88 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.
{"title":"Primary central nervous system lymphomas in children and adolescents","authors":"Oussama Abla , 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}
Pub Date : 2025-12-01Epub Date: 2025-09-17DOI: 10.1016/j.ejcped.2025.100322
Dana G.M. Peters , Caroline C.C. Hulsker , Leendert H.J. Looijenga , Ronald R. de Krijger , Annemieke S. Littooij , Jeanette van Leeuwen , Aart J. Klijn , Monica Terenziani , Cecile Faure-Conter , Gabriele Calaminus , Annelies M.C. Mavinkurve-Groothuis
Objective
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.
Methods
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.
Results
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.
Conclusions
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.
{"title":"Risk factors for relapse and guidance for follow-up of extracranial immature teratomas in children: A systematic review","authors":"Dana G.M. Peters , Caroline C.C. Hulsker , Leendert H.J. Looijenga , Ronald R. de Krijger , Annemieke S. Littooij , Jeanette van Leeuwen , Aart J. Klijn , Monica Terenziani , Cecile Faure-Conter , Gabriele Calaminus , 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}
Pub Date : 2025-12-01Epub Date: 2025-11-04DOI: 10.1016/j.ejcped.2025.100374
Passant Shaker, Nathan Gray, Michelle Boals, Kelsey Ray, Melvanique Hale, Sara Lewis, Alyssa Kennedy, Marcin Wlodarski
{"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, Nathan Gray, Michelle Boals, Kelsey Ray, Melvanique Hale, Sara Lewis, Alyssa Kennedy, 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}
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