EJC paediatric oncologyPub Date : 2026-06-01Epub Date: 2026-05-20DOI: 10.1016/j.ejcped.2026.100523
Emma C. Foster-Thomas , Koen B. Krommenhoek , Shermaine Pan , Craig W. Barclay , Angela Davey , Lucy S.C. Davies , Peter Sitch , SMILE Consortium , Thomas Melichar , Marinka L.F. Hol , Marianne Aznar
{"title":"Contouring and dose extraction of the maxilla, mandible and teeth on head and neck cancer radiotherapy planning scans","authors":"Emma C. Foster-Thomas , Koen B. Krommenhoek , Shermaine Pan , Craig W. Barclay , Angela Davey , Lucy S.C. Davies , Peter Sitch , SMILE Consortium , Thomas Melichar , Marinka L.F. Hol , Marianne Aznar","doi":"10.1016/j.ejcped.2026.100523","DOIUrl":"10.1016/j.ejcped.2026.100523","url":null,"abstract":"<div><h3>Background and Purpose</h3><div>Despite their vulnerability to radiation-induced damage, the maxilla and dentition are not routinely contoured in head and neck (HN) radiotherapy (RT), preventing accurate dose assessment and establishment of dose-effect relationships. This literature review aimed to: (1) identify published dentofacial contouring approaches, (2) synthesise current methods, and (3) where feasible, compare practices in paediatric and adult RT.</div></div><div><h3>Materials and Methods</h3><div>MEDLINE and Embase were searched for studies that contoured the maxilla, mandible, or teeth in HN RT. Reference lists were manually screened.</div></div><div><h3>Results</h3><div>Thirty-one (2003–2025) papers met inclusion criteria. Manual contouring predominated (77.4%), with substantial heterogeneity across all structures. Only seven studies (22.6%) contoured the maxilla. Tooth contouring varied from individual segmentation to variable groupings. Eight studies (25.8%) reported dental professional involvement.</div></div><div><h3>Conclusion</h3><div>Dentofacial contouring lacks standardisation and predominantly relies on manual techniques. This variability impedes dose assessment and cross-study comparisons. Standardised international guidelines for the contouring of the maxilla, mandible and teeth are necessary to ensure uniformity and enable comparability between studies.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100523"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148167366","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}
EJC paediatric oncologyPub Date : 2026-06-01Epub Date: 2026-05-30DOI: 10.1016/j.ejcped.2026.100528
Anne Maas , Valeriia Balaeva , Maëlle de Ville de Goyet , Jelica Predojevic , Jelena Roganovic , Charlotte Demoor-Goldschmidt , Amandine Bertrand , Päivi Lähteenmäki , Mira Pomrén , Eugenie Werbenko , Beate Timmermann , Magdalena Balcerek , Miklós Garami , Zsuzsanna Jakab , Monica Muraca , Sara Oberti , Hanne C. Lie , Kristen E.T. Thornton , Lorna Zadravec Zaletel , Katharina Roser , Gisela Michel
{"title":"Information and support needs of childhood, adolescent, and young adult cancer survivors (CAYACS) across europe and associations with patient activation and health-related quality of life: Results from the e-QuoL project","authors":"Anne Maas , Valeriia Balaeva , Maëlle de Ville de Goyet , Jelica Predojevic , Jelena Roganovic , Charlotte Demoor-Goldschmidt , Amandine Bertrand , Päivi Lähteenmäki , Mira Pomrén , Eugenie Werbenko , Beate Timmermann , Magdalena Balcerek , Miklós Garami , Zsuzsanna Jakab , Monica Muraca , Sara Oberti , Hanne C. Lie , Kristen E.T. Thornton , Lorna Zadravec Zaletel , Katharina Roser , Gisela Michel","doi":"10.1016/j.ejcped.2026.100528","DOIUrl":"10.1016/j.ejcped.2026.100528","url":null,"abstract":"<div><h3>Purpose</h3><div>This study assessed the prevalence and type of information and support needs among European childhood, adolescent, and young adult cancer survivors (CAYACS), their socio-demographic and clinical predictors, and associations with patient activation and health-related quality of life (HRQoL).</div></div><div><h3>Methods</h3><div>The e-QuoL Needs Study is a cross-sectional survey conducted in 15 European countries. Adult CAYACS (≥18 years, diagnosed before age 25 years) completed the modified Childhood Cancer Survivor Study–Needs Assessment Questionnaire (CCSS-NAQ), Patient Activation Measure® (PAM®), and PROMIS Global Health scales (HRQoL). Logistic and linear regression models were used to examine associations between socio-demographic and clinical characteristics, patient activation, and HRQoL.</div></div><div><h3>Results</h3><div>Overall, 571 CAYACS who completed ≥ 50% of the items in at least one CCSS-NAQ domain were included (71% female; 75% in the age group 18–35 years; 47% diagnosed >10 years ago). In all domains, except spirituality, most participants (62–90%) reported at least one need, with the highest prevalence found for cancer-related health information (90%), psycho-emotional consequences (87%), and health system concerns (84%). Females, survivors residing in Eastern Europe, and those with neurocognitive late effects were most likely to report needs (<em>p</em> < 0.05). Re<em>p</em>orting needs was associated with lower patient activation and poorer mental and physical HRQoL.</div></div><div><h3>Conclusions</h3><div>Information and support needs remain highly prevalent among European CAYACS, even long after diagnosis. Routine assessment of individual needs and targeted information strategies tailored to survivor groups identified as having higher needs may help reduce regional inequities, improve patient activation, and optimize HRQoL.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100528"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148167374","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}
EJC paediatric oncologyPub Date : 2026-06-01Epub Date: 2026-02-06DOI: 10.1016/j.ejcped.2026.100489
Britta Vormoor , Joshua Savage , Caroline Kristunas , Sarah Johnson , Mauricio Nicolás Ferrao Blanco , Geoff Shenton , Donna Lancaster , Anna Castleton , Adele K. Fielding , Anne-Louise Latif , Nick Morley , Marion Strullu , Ruta Tuckuviene , Pamela Kearns , Claire Jennings , Shelby Barnett , Gareth J. Veal , Julie Irving , Lucinda Billingham , Tobias Menne , Josef Vormoor
{"title":"Responses can be achieved with a combination of the MEK inhibitor selumetinib and dexamethasone in patients with relapsed/refractory RAS-pathway mutated acute lymphoblastic leukemia: Results of a parallel cohort, dose-finding and expansion phase I/II trial","authors":"Britta Vormoor , Joshua Savage , Caroline Kristunas , Sarah Johnson , Mauricio Nicolás Ferrao Blanco , Geoff Shenton , Donna Lancaster , Anna Castleton , Adele K. Fielding , Anne-Louise Latif , Nick Morley , Marion Strullu , Ruta Tuckuviene , Pamela Kearns , Claire Jennings , Shelby Barnett , Gareth J. Veal , Julie Irving , Lucinda Billingham , Tobias Menne , Josef Vormoor","doi":"10.1016/j.ejcped.2026.100489","DOIUrl":"10.1016/j.ejcped.2026.100489","url":null,"abstract":"<div><h3>Background</h3><div>Relapsed/refractory acute lymphoblastic leukemia (ALL) remains a major clinical challenge. We have previously shown that relapsed leukemias frequently carry RAS-pathway activating</div><div>mutations that could be targeted by MEK-inhibitors in combination with glucocorticosteroids.</div></div><div><h3>Methods</h3><div>Based on these pre-clinical results, we designed a phase I/II trial to evaluate the safety and preliminary efficacy of dexamethasone in combination with the MEK-inhibitor selumetinib for the treatment of relapsed/refractory RAS-pathway mutated ALL. The trial recruited both children and adults. Treatment consisted of oral selumetinib and dexamethasone in 28-day cycles.</div></div><div><h3>Results</h3><div>Initial study participants experienced serious adverse events due to infections, with three deaths from sepsis and pneumonia. Urgent safety measures were therefore introduced. This included reduction of the dexamethasone dose and frequency and the introduction of mandatory infectious prophylaxis. Nevertheless, twelve patients were recruited (four children, all B-immunophenotype; eight adults, 6/8 T-ALL). Nine patients were evaluable for response of whom four achieved a morphological complete remission after four weeks of treatment. Two of the responding patients (T- immunophenotype) remained stable on the combination for three and five cycles, respectively. The leukemic blasts of one responding patient were further characterized at time of progression, revealing persistence of the original NRAS mutation and upregulation of cell cycle/division genes as a potential targetable resistance mechanism. The study was stopped due to poor recruitment highlighting the challenges of academic multi-national early phase clinical trials in rare patient populations.</div></div><div><h3>Conclusion</h3><div>The combination of MEK-inhibitors with corticosteroids merits further investigation in RAS-pathway activated acute lymphoblastic leukemia, particularly T-ALL.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100489"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146173451","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}
EJC paediatric oncologyPub Date : 2026-06-01Epub Date: 2026-02-18DOI: 10.1016/j.ejcped.2026.100493
Luisa Queiró , Caroline C.C. Hulsker , Leendert H.J. Looijenga , Ronald R. de Krijger , Marijn A. Scheijde-Vermeulen , Pascal J.H. Kusters , Annemieke S. Littooij , Jeanette van Leeuwen , Arthur J.A.T. Braat , Annelies M.C. Mavinkurve-Groothuis
{"title":"[18F]FDG-avid nodal gliomatosis associated with ovarian immature teratoma – The importance of histological confirmation","authors":"Luisa Queiró , Caroline C.C. Hulsker , Leendert H.J. Looijenga , Ronald R. de Krijger , Marijn A. Scheijde-Vermeulen , Pascal J.H. Kusters , Annemieke S. Littooij , Jeanette van Leeuwen , Arthur J.A.T. Braat , Annelies M.C. Mavinkurve-Groothuis","doi":"10.1016/j.ejcped.2026.100493","DOIUrl":"10.1016/j.ejcped.2026.100493","url":null,"abstract":"<div><div>Gliomatosis is a rare condition mainly associated with ovarian teratomas. It is characterized by the presence of mature glial tissue in extraovarian sites and most frequently found in the peritoneum (gliomatosis peritonei). Gliomatosis in the lymph nodes (nodal gliomatosis) has rarely been reported. We discuss a case of a 4-year-old girl with immature ovarian teratoma with concomitant gliomatosis peritonei and nodal gliomatosis, detected with magnetic resonance imaging (MRI) and Fluorine-18 fluorodeoxyglucose positron-emission tomography/computed tomography ([<sup>18</sup>F]FDG PET/CT) as [<sup>18</sup>F]FDG-avid lesions. Histologic examination showed mature well differentiated glial tissue. We aim to highlight the importance of histopathologic assessment of [<sup>18</sup>F]FDG positive lesions that may initially appear metastatic.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100493"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147384839","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":"Current pattern of care in brain and near-brain tumor radiotherapy for infants and young children: A survey on age restrictions, treatment conditions and decision-making within SIOP Europe","authors":"Kristina Gaus , Petter Brandal , Martina Stickan-Verfürth , Heike Thomas , Stefanie Schulze Schleithoff , Rolf-Dieter Kortmann , Yasmin Lassen-Ramshad , Thankamma Ajithkumar , Geert O. Janssens , Henriette Magelssen , Beate Timmermann","doi":"10.1016/j.ejcped.2026.100521","DOIUrl":"10.1016/j.ejcped.2026.100521","url":null,"abstract":"<div><h3>Introduction</h3><div>Radiotherapy (RT) in children under 36 months of age with brain and near-brain tumors remains clinically challenging and harmonized age-related recommendations across Europe are lacking. This study aimed to evaluate current RT practice patterns and age-related decision-making in this population.</div></div><div><h3>Methods</h3><div>A web-based survey among members of the European Society for Paediatric Oncology (SIOPE) assessed institutional experience, lower age limits for RT, technical and disease-related determinants, protocol adherence and follow-up practices.</div></div><div><h3>Results</h3><div>Fifty-two professionals from 23 countries participated. All participants reported treating children under 36 months with RT, although only 58% irradiated children under 18 months and 27% under 12 months. Eighty-two percent worked in centers to which young children were referred and advanced RT techniques were widely available (90%). While international study protocols were commonly applied, only 52% reported having institutional standards for dose and volume constraints differing from those for older children. Lower age limits for general RT were reported by 56% of participants and typically ranged between 12 and 36 months. Craniospinal irradiation was avoided by 87% of these respondents in children under 36 months. Preserving cognitive function was the most important factor in age-related decisions (98%), followed by healthy development (75%). Treatment decisions were predominantly multidisciplinary (98%). Parental involvement required greater attention.</div></div><div><h3>Conclusion</h3><div>RT in children under 36 months is applied cautiously and heterogeneously across Europe. Individual decisions are driven by anticipated toxicities, technical feasibility and disease-related prognosis. Harmonized age-specific RT guidelines and improved long-term follow-up structures are needed to ensure safe and evidence-based care.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100521"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148167362","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}
EJC paediatric oncologyPub Date : 2026-06-01Epub Date: 2026-01-13DOI: 10.1016/j.ejcped.2026.100484
Kim P.J. Schellekens , Andrea Ferrari , Michael T. Meister , C. Michel Zwaan , Susanne A. Gatz , Max M. van Noesel , Michela Casanova , Reineke A. Schoot
{"title":"Multi-tyrosine kinase inhibitors in paediatric soft tissue sarcoma – A review","authors":"Kim P.J. Schellekens , Andrea Ferrari , Michael T. Meister , C. Michel Zwaan , Susanne A. Gatz , Max M. van Noesel , Michela Casanova , Reineke A. Schoot","doi":"10.1016/j.ejcped.2026.100484","DOIUrl":"10.1016/j.ejcped.2026.100484","url":null,"abstract":"<div><div>Neoadjuvant systemic treatment for paediatric patients with soft tissue sarcomas (STS) consists of conventional chemotherapy, predominantly ifosfamide and doxorubicin, with a limited role for innovative therapies. Many multi-tyrosine kinase inhibitors (mTKIs) have been developed in the past decades for the treatment of various types of malignancies. Over a decade ago, the mTKI pazopanib was authorized by the EMA and FDA for the treatment of adults with STS. Paediatric development of innovative treatments often follows years after the first marketing authorisations in adults has been obtained. To date, a series of clinical trials have been conducted investigating the value of mTKIs in paediatric patients with STS. Nevertheless, the positioning of mTKIs in the treatment of paediatric STS remains unclear and selection of mTKIs for clinical trials is more often made on grounds of availability rather than on mechanism of action, efficacy data, or toxicity profile. In this manuscript, we provide an overview of studies evaluating mTKIs in paediatric, adolescent, and adult STS patients, aiming to assist in prioritization of future clinical trials in paediatric STS. We performed a systematic literature review with the purpose of collecting data on activity and safety. The results of 107 relevant clinical trials, including 23 trials conducted in the paediatric population and 84 trials in the adult population, were critically assessed and summarized (Appendix 1). Accelerating the development of innovative treatments in patients with such ultra-rare diseases will require innovative clinical trial designs, and international collaboration is necessary. Considering the age distribution of STS, collaboration between paediatric oncologists and medical oncologists should be considered.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"7 ","pages":"Article 100484"},"PeriodicalIF":0.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145978274","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}
EJC paediatric oncologyPub 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
{"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}
EJC paediatric oncologyPub 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
{"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}
EJC paediatric oncologyPub Date : 2026-06-01Epub Date: 2026-02-20DOI: 10.1016/j.ejcped.2026.100498
Ana Carolina Xavier , Oussama Abla
{"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}
EJC paediatric oncologyPub 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
{"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}