EJC paediatric oncology最新文献

{"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":"2025-09-17","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":"Early discontinuation of empiric antibiotic therapy in children treated for cancer who develop febrile neutropenia: A prospective cohort study","authors":"Smaragda Papachristidou ,&nbsp;Dimitrios Doganis ,&nbsp;Georgia Kourlaba ,&nbsp;George Pantalos ,&nbsp;Sophia Pasparaki ,&nbsp;Margarita Baka ,&nbsp;Apostolos Pourtsidis ,&nbsp;Lydia Kossiva ,&nbsp;Vasiliki Papaevangelou ,&nbsp;Nikolaos Spyridis ,&nbsp;Maria Tsolia","doi":"10.1016/j.ejcped.2025.100321","DOIUrl":"10.1016/j.ejcped.2025.100321","url":null,"abstract":"<div><h3>Introduction</h3><div>Febrile neutropenia (FN) is a life-threatening complication for children with cancer. Early administration of broad–spectrum antibiotics has significantly improved outcome. In many countries, children with culture negative FN remain hospitalized on intravenous antibiotic treatment until neutrophil count recovery. The aim of this study was to explore the safety of short course antibiotic regimens in children with culture negative FN.</div></div><div><h3>Methods</h3><div>A prospective cohort study with 1:1 matched historical controls was conducted in a single center. Children with cancer admitted with a low-risk episode of FN from 2017 until 2020 and met the inclusion criteria, were included in the study. The study was initiated after institutional adoption of an early antibiotic discontinuation protocol for low-risk FN. All children received empiric treatment with cefepime or piperacillin/tazobactam combined with an aminoglycoside. Antibiotics were discontinued after 48 h of defervescence, when blood cultures were negative regardless of neutrophil count.</div></div><div><h3>Results</h3><div>Thirty-six out of 456 FN episodes met inclusion criteria. There were no readmissions due to fever or infection during neutropenia. Median neutrophil count at discontinuation was 0.16 × 10⁹/L. Median length of hospitalization was 2 days, compared with 6 days until neutrophil recovery (p &lt; 0.0001) and 7 days for the control group (p &lt; 0.0001). There was significant reduction of hospital charges compared to controls.</div></div><div><h3>Conclusion</h3><div>This study provides evidence that early discontinuation of antibiotics in oncology patients with a low-risk episode of FN is safe, regardless of neutrophil count. Reduced exposure to antibiotics, shorter hospitalization and lower costs are beneficial secondary outcomes observed in this study.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100321"},"PeriodicalIF":0.0,"publicationDate":"2025-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145098895","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 multi-dimensional approach to recognize genetic predisposition in children with acute lymphoblastic leukemia","authors":"Stefanie V. Junk ,&nbsp;Laura R. Bettini ,&nbsp;Katharina Daugs ,&nbsp;Melina Mescher ,&nbsp;Marjolijn C.J. Jongmans ,&nbsp;Arndt Borkhardt ,&nbsp;Giovanni Cazzaniga ,&nbsp;Roland P. Kuiper ,&nbsp;Jette J. Bakhuizen","doi":"10.1016/j.ejcped.2025.100320","DOIUrl":"10.1016/j.ejcped.2025.100320","url":null,"abstract":"<div><div>Pediatric acute lymphoblastic leukemia (ALL) is the most common childhood cancer, with significant advances in treatment leading to high cure rates. Recent studies have highlighted the importance of genetic predisposition in ALL. Identifying contributing heritable or <em>de novo</em> genetic factors is crucial for potential treatment modifications, early detection of second malignant neoplasms (SMNs) and genetic counseling for surveillance of patients and family members. Multiple syndromes, such as Down syndrome (DS) or Ataxia Telangiectasia (AT), are known to give rise to increased risk for developing ALL. Most of these syndromes can be recognized by the presence of specific clinical features. However, a notable proportion of patients harboring (likely) pathogenic germline variants in cancer predisposition genes (CPGs) can easily be missed due to the absence of these characteristics. Therefore, the diagnosis of cancer predisposition syndromes (CPS) requires multiple approaches that are based on phenotypic characteristics, germline genetic analysis and genomic characterization of the leukemia samples. Despite the recognized benefits, routine screening for germline variants has not yet been implemented in large study groups due to logistical and financial challenges. This review emphasizes the importance of integrating systematic genetic testing into standard care protocols for ALL patients and summarizes current practical considerations for CPS identification in children with ALL.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100320"},"PeriodicalIF":0.0,"publicationDate":"2025-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145049360","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":"Social interactions and psychosocial development of adolescent and young adult childhood cancer survivors: a scoping review of qualitative research","authors":"Asami Ogino ,&nbsp;Naho Sato ,&nbsp;Aya Nakazuru","doi":"10.1016/j.ejcped.2025.100319","DOIUrl":"10.1016/j.ejcped.2025.100319","url":null,"abstract":"<div><div>Adolescent and Young Adult (AYA) childhood cancer survivors (CCSs) sometimes experience disruption and changes in social interactions owing to their childhood cancer experience and subsequent late effects. Additionally, they are undergoing psychosocial development and establishing their social identities. This study aimed to explore previous findings on the qualitative characteristics of developmental and social interactions among AYA-CCSs. This scoping review was conducted based on Arksey and O'Malley's framework and PRISMA-ScR guidelines. CINAHL, MEDLINE, PsycINFO, and Web of Science databases were searched for terms related to social interactions and psychosocial development among AYA-CCSs from January 2004 to May 2022. This review included 22 references from 11 countries published from 2011 to 2022. Content related to AYA social interactions and psychosocial development was coded and classified into 10 categories based on the social well-being model of quality of life for cancer survivors, with 26 subcategories created based on content similarity. Most descriptions fell under the categories of Family and Roles and relationships. In “Roles and Relationships,” it is stated that close interactions with friends help maintain a strong sense of self-identity, whereas the experience of losing friends is a negative experience that affects developmental discrepancies. Therefore, it can be said that relationships in one's surroundings have both positive and negative effects on identity formation during adolescence. Social interaction is an essential factor supporting the psychosocial development of AYA-CCSs. The ability to develop positive relationships with those around them and to find their roles in society may facilitate the development of their identity.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100319"},"PeriodicalIF":0.0,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144917798","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 insights and future directions in systemic therapies for gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) in children and adolescents: a critical review of advancements and challenges","authors":"Michaela Kuhlen ,&nbsp;Katharina Karges ,&nbsp;Marina Kunstreich ,&nbsp;Maximilian Schmutz ,&nbsp;Antje Redlich ,&nbsp;Rainer Claus ,&nbsp;Constantin Lapa","doi":"10.1016/j.ejcped.2025.100318","DOIUrl":"10.1016/j.ejcped.2025.100318","url":null,"abstract":"<div><div>Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) in children and adolescents are rare and biologically heterogeneous. Due to their low incidence, therapeutic strategies are largely adapted from adult protocols, underscoring a critical need for paediatric-specific evidence.</div><div>Surgical resection remains the mainstay of curative treatment for localized disease and should be prioritized before the initiation of systemic therapy whenever feasible. This review synthesizes current knowledge on systemic therapies in paediatric GEP-NENs,</div><div>including somatostatin analogues (SSAs), peptide receptor radionuclide therapy (PRRT), chemotherapy, small molecules (e.g., everolimus, sunitinib), and immune checkpoint inhibitors (ICIs). While SSAs remain the mainstay for well-differentiated, somatostatin receptor (SSTR)-positive tumours, emerging data support the safety and potential efficacy of PRRT in paediatric populations, despite limited prospective evidence. Chemotherapy continues to play a role in high-grade or progressive disease, although responses are variable.</div><div>Supportive therapies, including high-dose proton pump inhibitors (PPIs), are also important in managing functional tumours and can significantly alleviate clinical symptoms in advanced disease.</div><div>Novel approaches, including SSTR antagonists, α- and β-emitting radiopharmaceuticals, and oncolytic virotherapy (e.g., SVV-001), are under active investigation in adults and may inform future paediatric protocols. Resistance mechanisms—particularly to SSAs—highlight the dynamic nature of tumour evolution and the need for individualized strategies.</div><div>These insights underscore the importance of molecular profiling and imaging-based SSTR assessment to guide therapeutic selection, particularly in refractory or complex paediatric cases. Future efforts should prioritize international collaboration, the design of rational combination regimens, and the integration of radiomics, genomics, and biomarker-driven approaches to advance precision medicine in paediatric GEP-NENs.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100318"},"PeriodicalIF":0.0,"publicationDate":"2025-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144748756","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":"Developing patient reported outcome measures for children with cancer: A current perspective","authors":"Madeleine Adams ,&nbsp;Rachel M. Taylor","doi":"10.1016/j.ejcped.2025.100312","DOIUrl":"10.1016/j.ejcped.2025.100312","url":null,"abstract":"<div><div>Patient reported outcomes measures (PROM) are the gold standard for evaluating patient experience and quality of life (QOL), both in research studies and clinical practice. In paediatric oncology, the wide spectrum of disease and treatment strategies means that correct choice of PROM and robust methodology when developing new PROMs is vital to ensuring that the desired outcomes (e.g. pain, functional ability or quality of life) are accurately assessed. Children differ from adults both medically and developmentally meaning that specific factors should be considered including age and developmental ability, use of proxy/observer reports and mode of administration. Best practice guidelines outline the methodology for developing the content of a new PROM as well as assessing psychometric properties. This paper provides a review of the background, current guidelines, and methodology for developing and choosing PROMs for children with cancer.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100312"},"PeriodicalIF":0.0,"publicationDate":"2025-07-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144748605","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":"Local therapy for rhabdomyosarcoma of the bladder and/or prostate without nodal or metastatic spread during the European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS2005 study","authors":"Naima Smeulders ,&nbsp;Florent Guerin ,&nbsp;Mark N. Gaze ,&nbsp;Timothy Rogers ,&nbsp;Sheila Terwisscha van Scheltinga ,&nbsp;Federica De Corti ,&nbsp;Julia Chisholm ,&nbsp;Olga Slater ,&nbsp;Veronique Minard-Colin ,&nbsp;Beatrice Coppadoro ,&nbsp;Ilaria Zanetti ,&nbsp;Ross Craigie ,&nbsp;Gabriela Guillen Burrieza ,&nbsp;Patrizia Dall'Igna ,&nbsp;Raquel Davila Fajardo ,&nbsp;Pei S. Lim ,&nbsp;Cyrus Chargari ,&nbsp;Sophie Espenel ,&nbsp;Ana L. Luis Huertas ,&nbsp;Alexander Cho ,&nbsp;Helene Martelli","doi":"10.1016/j.ejcped.2025.100313","DOIUrl":"10.1016/j.ejcped.2025.100313","url":null,"abstract":"<div><h3>Background</h3><div>During the EpSSG RMS2005 trial, organ-sparing surgery (OSS) with brachytherapy (BT) became the local therapy (LT) of choice for selected patients with bladder-prostate rhabdomyosarcoma (BP-RMS). We compare this LT technique with surgical resection and/or external-beam radiotherapy.</div></div><div><h3>Methods</h3><div>Patients with BP-RMS without nodal or metastatic spread enrolled in RMS2005 were categorized by their LT, differentiating OSS from organ-depleting surgery (ODS) and BT from external-beam radiotherapy (EBRT). Progressive disease, relapse or death were considered events for progression-free survival (PFS) and all deaths for overall survival (OS).</div></div><div><h3>Results</h3><div>The cohort comprised 176 patients, aged 10days-21.8years (median 2.5years). Median follow-up was 6.5years (22months-12.5years): 5year-PFS was 80.3 % (95 %CI:73.6–85.5 %); 5year-OS was 90.7 % (95 %CI:85.3–94.2 %).</div><div>Patients selected for surgery alone or BT with/without OSS (BT+/-OSS) differed significantly in age, tumour size and location from those offered EBRT alone or any other surgery and radiotherapy. Nevertheless, 5year-PFS was similar for the LT groups. However, 5year-OS differed significantly, being highest in patients suitable for surgery alone (100 %; by ODS in 55 %) or BT+ /-OSS (98.1 %; 95 %CI:87.4–99.7 %). Patients with local tumour progression/relapse after EBRT failed salvage: 5year-OS was 81.8 % (95 %CI:58.5–92.8 %) for EBRT alone and 85.3 % (95 %CI:71.6–92.7 %) for surgery and radiotherapy.</div><div>Postponing LT until after chemotherapy cycle 7 did not significantly impact 5year-PFS or OS.</div></div><div><h3>Conclusions</h3><div>The risk of events was similar for different LT modalities; poor salvage after EBRT significantly reduced 5year-OS. Although not feasible for all, BT+ /-OSS offers an excellent prospect of cure, the best chance of organ retention while avoiding EBRT, and may be delayed for chemotherapy responsive tumours.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100313"},"PeriodicalIF":0.0,"publicationDate":"2025-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144711246","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":"Multicentre evaluation of the management of children with high risk medulloblastoma: Real world performance of the SJMB03 and COG-99701 protocols","authors":"John R. Apps ,&nbsp;Jack Goddard ,&nbsp;Shaimaa Sahmoud ,&nbsp;Katherine Green ,&nbsp;Kriti Hedge ,&nbsp;Claire Keeling ,&nbsp;Anoop Cherungonath ,&nbsp;Banuja Srikumar ,&nbsp;Blanche Lumb ,&nbsp;Jennifer Whitby ,&nbsp;James Hayden ,&nbsp;Jenny Gains ,&nbsp;Henry Mandeville ,&nbsp;Lewis Joanne ,&nbsp;Barry Pizer ,&nbsp;John Paul Kilday ,&nbsp;Steven C. Clifford ,&nbsp;Simon Bailey ,&nbsp;Mette Jorgensen ,&nbsp;Fernando Carceller ,&nbsp;Jenny Adamski","doi":"10.1016/j.ejcped.2025.100311","DOIUrl":"10.1016/j.ejcped.2025.100311","url":null,"abstract":"<div><div>Several treatment protocols are used for the treatment of high-risk medulloblastoma (HR-MB). In 2015, the UK Children’s Cancer and Leukaemia Group issued guidance recommending treatment as per the SJMB03 protocol, whilst also recognising that the COG-99701 protocol may be used. Patients were defined as high-risk if metastatic at presentation, large-cell/anaplastic histology, <em>MYC</em> amplification, significant residual disease or <em>MYCN</em> amplification. Recently, the latter two only define high risk if other adverse features are present.</div></div><div><h3>Methods</h3><div>Retrospective multi-centre service evaluation of treatment of HR-MB at five UK centres. Patients were included if treated as per SJMB03 or COG-99701. Patients were excluded if initially treated for standard-risk medulloblastoma and subsequently treated with these protocols due to upstaging or disease progression.</div></div><div><h3>Results</h3><div>58 patients were identified: 26 treated as per SJMB03, 32 as per COG-99701. 5-year OS was 83 % (95 %CI 73–94 %) and 5-year PFS was 65 % (53–80 %). For patients treated as per SJMB03, 5-year OS and PFS were 80 % (65–97 %) and 75 % (60–95 %) respectively; for patients treated as per COG-99701, 5-year OS and PFS were 85 % (73–100 %) and 60 % (43–83 %). There was no significant difference in outcomes between protocols. There was a higher incidence of grade 3/4 ototoxicity (44 % vs 6 %, p = 0.001) and admission to paediatric intensive care (19 % vs 0 %, p = 0.014) in patients treated as per SJMB03 compared to COG-99701.</div></div><div><h3>Conclusion</h3><div>These real-world outcomes are consistent with the published literature on HR-MB patients treated with these protocols within clinical trials, and provide important evidence to inform their use in routine practice.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100311"},"PeriodicalIF":0.0,"publicationDate":"2025-07-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144771505","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":"Preoperative short-term fasting in paediatric cancer care is safe, feasible and activates protective responses","authors":"D.S.J. Komninos ,&nbsp;C.A.J. Oudmaijer ,&nbsp;R.A. Ozinga ,&nbsp;K. Smit ,&nbsp;J.N.M. Ijzermans ,&nbsp;J.H.J. Hoeijmakers ,&nbsp;R.C. Minnee ,&nbsp;M.M. van den Heuvel-Eibrink ,&nbsp;W.P. Vermeij","doi":"10.1016/j.ejcped.2025.100309","DOIUrl":"10.1016/j.ejcped.2025.100309","url":null,"abstract":"<div><h3>Background</h3><div>Paediatric cancer patients often face treatment-induced complications significantly affecting Quality of Life. Short-term fasting (STF) could mitigate therapy-related adverse toxicity by triggering a protective survival response. While STF and other nutritional interventions have shown clinical benefits in adults, application in paediatric cancer remains unexplored. This study aims to explore the safety and feasibility of incorporating STF into the treatment regimen for children with renal cancer, while simultaneously showing a protective transcriptomic signature.</div></div><div><h3>Methods</h3><div>Here we selected children with a localized renal tumour at the Princess Máxima Center. In a randomized controlled setting preceding surgery (FIURTT-study), these children fasted for 10–18 h, depending on age, during which patient/parent experiences and blood measurements were collected. To investigate the STF-induced effects on enhancing postoperative recovery, kidney tissue was used for transcriptome analysis, and compared to a well-defined 2.5-day fasting response in adults.</div></div><div><h3>Results</h3><div>Blood measurements of 13 patients revealed significantly decreased glucose levels after STF, without occurrence of hypoglycaemia. Questionnaires demonstrated that patients and parents experienced no obvious burden and described the intervention as highly feasible. RNA-expression patterns in normal kidney tissue unveiled distinctions between cases with STF and those without, displaying significant difference in relevant pathways linked to activated protective-responses, resembling those in adults.</div></div><div><h3>Conclusion</h3><div>Our results indicate pre-operative STF to be safe and feasible in children with cancer. Also, we observed induction of the survival response at the transcriptional level, suggesting that STF induced a protective gene expression profile already after relatively short fasting periods.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100309"},"PeriodicalIF":0.0,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144631888","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":"Familial risks and incidence rates for childhood nervous system tumors in Sweden","authors":"Kari Hemminki ,&nbsp;Xinjun Li ,&nbsp;Kristina Sundquist ,&nbsp;Jan Sundquist ,&nbsp;Steffen Hirsch ,&nbsp;Jianguang Ji ,&nbsp;Akseli Hemminki ,&nbsp;Asta Försti","doi":"10.1016/j.ejcped.2025.100310","DOIUrl":"10.1016/j.ejcped.2025.100310","url":null,"abstract":"<div><h3>Background</h3><div>Childhood (&lt; 20 years) nervous system tumors manifest in some rare cancer syndromes but how commonly they present with familial clustering between various histological types is not well known at a nation-wide level. Our aim is to enhance understanding of familial risks in childhood nervous system cancers.</div></div><div><h3>Methods</h3><div>We used the Swedish population and cancer data from years 1958–2021 to address familial risks among nervous system cancers when the case was diagnosed before age 20 years but the proband could be diagnosed at any age. Adjusted familial (relative) risks were expressed as standardized incidence ratios (SIRs).</div></div><div><h3>Results</h3><div>Familial childhood cancer cases amounted to 123 in the brain, 15 in the spinal cord and 9 in peripheral nerves. Familial risk for concordant brain cancer was about 2.0 irrespective of proband. Concordant risk of spinal cord cancer was high when mothers (17.92) or siblings were probands (24.91). High familial risk of 34.53 was recorded for hemangioblastoma, and moderate high risks were observed also for schwannoma (4.07), ependymoblastoma (3.48) and female ganglioneuroma (7.72) whereas astrocytoma risks were at the level of common cancers at other sites (1.7–2.0). Hemangioblastoma families appeared not to be related to von Hippel-Lindau syndrome because of lack of pathognomonic signs other than hemangioblastoma in the families.</div></div><div><h3>Conclusion</h3><div>In spite of the low number of childhood nervous system tumors, we observed high familial risks for, probably novel, syndromic hemangioblastoma, and schwannomas for which concordant clusters were found particularly in the spinal cord. Inquiring about a detailed family history at diagnosis of a nervous system cancer in childhood may reveal a syndromic disease due to a constitutional variant amenable to treatment.</div></div>","PeriodicalId":94314,"journal":{"name":"EJC paediatric oncology","volume":"6 ","pages":"Article 100310"},"PeriodicalIF":0.0,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144605535","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}