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OBJECTIVES: Childhood cancer survivors may experience complex health issues during transition and long-term follow-up (LTFU); therefore, high-quality healthcare is warranted. Care coordination is one of the essential concepts in advanced healthcare. Care coordination models vary among childhood cancer survivors in transition and LTFU. This study aimed to identify care coordination models for childhood cancer survivors in transition and LTFU and synthesise essential components of the models. DESIGN: This scoping review was guided by the methodological framework from Arksey and OMalley and was reported with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews. A systematic literature search was conducted on six databases using possible combinations of terms relevant to childhood cancer survivors, transition/LTFU and care coordination model. Data were analysed by descriptive and content analysis. DATA SOURCES: The literature search was first conducted in May 2023 and updated in May 2024. Six databases including Medline, PubMed, Embase, Web of Science, CINAHL and Cochrane Library were searched; meanwhile, a hand search was also conducted. ELIGIBILITY CRITERIA FOR SELECTING STUDIES: Studies relevant to describing any models, interventions or strategies about care coordination of transition or LTFU healthcare services among childhood cancer survivors were included. DATA EXTRACTION AND SYNTHESIS: Two reviewers independently screened and included studies. Basic information as well as care coordination model-related data in the included studies were extracted. Descriptive summary and content analysis were used for data analysis. RESULTS: In the 20 545 citations generated by the search strategy, seven studies were identified. The critical determinants of the models in the included studies were the collaboration of the multidisciplinary team, integration of the navigator role and the provision of patient-centred, family-involved, needs-oriented clinical services. The main functions of the models included risk screening and management, primary care-based services, psychosocial support, health education and counselling, and financial assistance. Models of care coordination were evaluated at patient and clinical levels. Based on this review, core concepts of successful care coordination models for childhood cancer survivors in transition or LTFU were synthesised and proposed as the 3 I framework: individualisation, interaction and integration. CONCLUSION: This scoping review summarised core elements of care coordination models for childhood cancer survivors transition and LTFU. A proposed conceptual framework to support and guide the development of care coordination strategies for childhood cancer survivors transition and LTFU care was developed. Future research is needed to test the proposed model and develop appropriate care coordination strategies for providing high-quality healthcare for childhood cancer survivors transition and LTFU.

Purpose: Evidence-based interventions (EBIs) are essential to improve the well-being and neurocognitive outcomes of pediatric cancer patients; however, considerable barriers hamper the implementation of these tools. The present study assessed health care professionals’ (HCP) perceived barriers and facilitators to the implementation of a specific EBI for pediatric oncology in a standardized manner to define effective solutions and practical recommendations. Methods: An adapted version of the Consolidated Framework for Implementation Research (CFIR) questionnaire was applied to inquire n = 31 HCPs in pediatric oncology about the five domains of implementation. Results: While most ‘intervention characteristics’ were considered beneficial for implementation, various aspects of the ‘inner’ and ‘outer setting’ were considered problematic. The most prevalent barriers included a shortage in resources, poor integration of EBIs into policies and lacking incentives such as user benefits. Concrete proposed and realized steps to facilitate effective implementation include a patient-focused design and continuous evaluation and adaption of the tool, a detailed EBI user manual and application workshops, as well as regular interdisciplinary meetings to improve communication. Regarding the internal and external settings, involving policy makers, establishing psychosocial care in the insurance system and increasing awareness by sharing evidence are essential steps for improved implementation. Conclusion: Based on standardized implementation evaluation, various targeted actions could be defined and implemented to facilitate successful implementation of EBIs in pediatric oncology. The results emphasize that psychosocial care must become an integral part of treatment standards and public health policies to ensure that effective psychosocial interventions for improved wellbeing and neurocognitive skills successfully reach pediatric cancer patients. Trial registration number: ClinicalTrials.gov Identifier: NCT04474678 (July 17th 2020). [ABSTRACT FROM AUTHOR]

Image-guided treatment adaptation is a game changer in oncological particle therapy (PT), especially for younger patients. The purpose of this study is to present a cycle generative adversarial network (CycleGAN)-based method for synthetic computed tomography (sCT) generation from cone beam CT (CBCT) towards adaptive PT (APT) of paediatric patients. Firstly, 44 CBCTs of 15 young pelvic patients were pre-processed to reduce ring artefacts and rigidly registered on same-day CT scans (i.e., verification CT scans, vCT scans) and then inputted to the CycleGAN network (employing either Res-Net and U-Net generators) to synthesise sCT. In particular, 36 and 8 volumes were used for training and testing, respectively. Image quality was evaluated qualitatively and quantitatively using the structural similarity index metric (SSIM) and the peak signal-to-noise ratio (PSNR) between registered CBCT (rCBCT) and vCT and between sCT and vCT to evaluate the improvements brought by CycleGAN. Despite limitations due to the sub-optimal input image quality and the small field of view (FOV), the quality of sCT was found to be overall satisfactory from a quantitative and qualitative perspective. Our findings indicate that CycleGAN is promising to produce sCT scans with acceptable CT-like image texture in paediatric settings, even when CBCT with narrow fields of view (FOV) are employed. [ABSTRACT FROM AUTHOR]

Sandip Patil, Xinye Li, Huirong Mai, Ying Wang, Xue Tang, Sixi Liu, Feiqiu Wen Department of Haematology and Oncology, Shenzhen Children’s Hospital, Shenzhen, Guangdong Province, People’s Republic of ChinaCorrespondence: Sixi Liu; Feiqiu Wen, Email tigar467@126.com; fwen62@163.comBackground: This case report underscores the intricate challenges in managing paediatric patients with acute myeloid leukaemia (AML) undergoing intensive chemotherapy, particularly when complicated by the emergence of multidrug-resistant pathogens such as Carbapenem-Resistant Pseudomonas aeruginosa (CRPA).Case Presentation: An 11-year-old male with AML presented with skin purpura and persistent cough. Clinical and laboratory assessments revealed a high-risk AML profile with genetic mutations, leading to the initiation of intensive chemotherapy per the C-HUANA-AML-2015 protocol. Despite successful disease remission after initial chemotherapy courses, the patient experienced unexpected complications. Notably, septic shock, bone marrow failure, and the emergence of CRPA were encountered during the clinical course. Septic shock occurred following Course B3 chemotherapy, marked by a fever unresponsive to initial antibiotic therapy. Despite negative blood cultures, meropenem and vancomycin were initiated, successfully normalizing temperature. Subsequent challenges included persistent bone marrow suppression, perianal dermatitis, and the identification of CRPA in stool cultures, leading to altered antibiotic therapy guided by minimum inhibitory concentration (MIC) considerations. Whole-genome sequencing (WGS) of the CRPA strain revealed a highly virulent clone (ST-970) with numerous resistance and virulence genes.Conclusion: This case report offers new insights into the complexities of pediatric AML management, with a focus on the emergence of CRPA. The discovery of a high-risk CRPA clone with detailed genomic data underscores the growing challenge of antimicrobial resistance in pediatric oncology. The persistent presence of CRPA and ongoing bone marrow failure highlight the difficulties in managing these complications. This case calls for a reassessment of treatment strategies and encourages further research to improve outcomes in pediatric AML, emphasizing the need for a multidisciplinary approach to address infectious complications and antimicrobial resistance.Keywords: acute myeloid leukaemia, carbapenem-resistant Pseudomonas aeruginosa, paediatric oncology, antimicrobial resistance, superbugs

The association of recognisable neurological conditions with an underlying malignancy is well described. In this review we explore the complex interplay of genetic, environmental and tumour factors which contribute to autoimmunity and paraneoplastic conditions. We review the current understanding of the pathogenesis of well recognised paraneoplastic conditions in children including Opsoclonus myoclonus ataxia syndrome, N-Methyl-D Aspartate receptor encephalitis and limbic encephalitis, and the broad approaches to treatment. Rapid advances in oncological treatment has expanded the arsenal of therapeutic modalities. We explore the broad spectrum of immune therapies in childhood cancer, and the potential neurological complications of these novel therapies, and discuss the fine balance of risk and benefit that these bring. • Sex, age, ethnicity, genetic vulnerability and malignancy are factors associated with brain directed autoimmunity. • In OMAS NMDARE immune and oncological management are required for optimal management of these rare childhood conditions. • Immunotherapy in the treatment of childhood cancers has potential to improve both survival and quality of life. • Immune check-point inhibitors are associated with neurological immune-related adverse events in at least 10 % of patients. [ABSTRACT FROM AUTHOR]

Personalised medicine, facilitated by advancements like 3D printing, may offer promise in oncology. This scoping review aims to explore the applicability of 3D printing for personalised pharmaceutical dosage forms in paediatric cancer care, focusing on treatment outcomes and patient experiences. Following the Joanna Briggs Institute (JBI) methodology, a comprehensive search strategy was implemented to identify the relevant literature across databases including PubMed, Embase, and Web of Science. Three independent reviewers conducted study selection and data extraction, focusing on studies involving paediatric patients under 18 years old and pharmaceutical dosage forms manufactured using 3D printing technology. From 2752 records screened, only six studies met the inclusion criteria, none of which specifically targeted paediatric cancer patients. These studies examined aspects of acceptability, including swallowability, taste, and feasibility of 3D-printed formulations for children. While the studies demonstrated the potential benefits of 3D printing in paediatric medication, particularly in personalised dosing, there is a notable lack of evidence addressing its acceptability in paediatric cancer patients. Further interdisciplinary collaborative research is needed in this area to fully assess preferences and acceptability among children with cancer and their parents or caregivers. [ABSTRACT FROM AUTHOR]

To compare paediatric oncologic vascular access ports located on the anterior thoracic wall to ports on the lower lateral thoracic wall, in terms of perceived port-related hindrance and scar-quality. A cross-sectional survey study including paediatric oncology patients (≥8-<19 yrs), caregivers (in patients <8 yrs), survivors (>22 yrs with only anterior ports) and nurses of the Princess Máxima Center, the Netherlands, was performed. The survey consisted of questions regarding satisfaction, hindrance during daily life, and port position preference. For survivors, scar-quality was assessed using the validated Patient and Observer Scar Assessment Scale (POSAS 2.0); a high score (i.e., a displeasing scar) was defined as a score higher than the third quartile of the median for that question. In total, 147 participants were included; 83 patients/caregivers, 31 survivors, and 33 nurses. Overall, 81 % was satisfied with the position of their port. Satisfaction, hindrance and complications did not differ between anterior and lower lateral ports. For the anterior position, minimal pressure on the port during daily life was a mentioned reason to prefer this position. For the lower lateral position, less visibility of the scar and easiest access were mentioned. Of all survivors with an anterior port scar, one in five had a displeasing scar and all scars observed were widened. Female patients preferred a lower lateral port, and scar-quality was better for left-sided port scars. The port position should be chosen together with patients/caregivers based on the (dis-)advantages of each position, as identified by this study. II. • Satisfaction, hindrance and complications did not differ between anterior and lower lateral ports. • The most ideal port position should therefore be chosen together with patients/caregivers based on the (dis-)advantages of each position. [ABSTRACT FROM AUTHOR]

Due to the promise of transforming healthcare and medicine that Artificial Intelligence (AI) has posed, the number of applications has increased exponentially. These applications range from screening and disease diagnosis to prognosis, treatment planning, and follow-up. In complex topics such as childhood cancer, these techniques are being expanded with the ambition of improving the quality of care by allowing healthcare professionals to make more informed decisions. However, the adequate application of such techniques heavily depends on the data, which creates a set of challenges including collection, bias, and scarcity among others. Furthermore, ethical, legal, and regulatory frameworks increase even more the difficulties to develop AI-powered solutions. In this paper, we present an exhaustive literature review to identify and analyse public datasets targeting two common childhood cancer types, such as neuroblastoma and nephroblastoma. Moreover, the complex context for the development of AI- based software solutions is outlined. It includes the description of the most relevant techniques to address problems associated with data sharing and training. Finally, a set of code snippets is provided to perform exploratory analysis for the available data.

Introduction: Data-driven research has improved paediatric cancer outcomes for children. However, challenges in sharing data between institutions prevent the use of artificial intelligence (AI) to address substantial unmet needs in children diagnosed with cancer. Harmonising collected data can enable the application of AI for a greater understanding of paediatric cancers. The main goal of the paper was to analyse the currently used childhood cancer databases to identify a core of variables able to capture the most relevant data on the diagnosis and treatment of children and adolescents with cancer. Methods: We arbitrarily identified different types of existing databases dedicated to collecting data of patients with solid tumours, Umbrella, FAR-RMS; PARTNER; ERN PAEDCAN Registry; INSTRUCT and INRG; the common data elements for Rare Disease by Joint Research Centre. The different elements of the CRFs were analysed and ranked “essential” and “good to have”. Domains that included a group of variables structurally connected were identified. Each variable was defined by name, data type, description, and permissible values. Results: We identified six structural domains: Patient registration, Personal information, Disease History, Diagnosis, Treatment, and Follow-up and Events. For each of them, “essential” and “good to have” variables were defined. Discussion: Data harmonisation is essential for enhancing integration and comparability in research. By standardizing data formats and variables, researchers can facilitate data sharing, collaboration, and analysis across multiple studies and datasets. Embracing data harmonization practices will advance application of AI, scientific knowledge, improve research reproducibility, and contribute to evidence-based decision-making in various fields.

A chemoport is widely used in paediatric oncology population. Removal is a relatively easy procedure, but difficulty can be encountered in case the catheter is densely adherent to the vascular wall. It is a rare complication and is associated with long indwelling duration and acute lymphoblastic leukaemia (ALL). Forceful traction can lead to vascular injury and high morbidity. Herein, we report a 7-year-old girl with precursor B ALL who had delayed chemoport removal due to the coronavirus disease (COVID-19) pandemic. The removal process was difficult, as the catheter was adherent to the right innominate vein. Out of panic, the surgeon pulled it out forcefully. Fortunately, the catheter and its fragment were successfully retrieved completely and the child was discharged the next day. The management strategy varies and ranges from minimally invasive to open surgery. Leaving a stuck chemoport catheter in situ can be a bailout method or part of conservative management.

Summary: Background: Food restrictions during periods of neutropenia have been widely used in oncology settings to prevent infections. As there is a lack of clearly demonstrated effectiveness, this strategy is being increasingly questioned. Methods: A multi-national panel of 23 individuals was convened to develop a clinical practice guideline (CPG) on the use of food restrictions to prevent infections in paediatric patients with cancer and haematopoietic cell transplantation (HCT) recipients. It included representation from persons with lived experience and physicians, dieticians, nurses, pharmacists and guideline methodologists working in paediatric oncology/HCT or infectious diseases. Panel members (female n = 15; 65%) were from North America (12, 52%), Europe (8, 35%), South America (2, 9%) and Australia (1, 4%). The Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach was used to formulate the CPG recommendations based on a systematic review of randomised controlled trials (RCTs). MEDLINE, MEDLINE in-Process and Embase databases were searched from January 1, 1980, to May 7, 2024, with a broad strategy which combined subject headings and text words relating to neutropenia, infection and diet. Findings: The systematic review, which provided the evidence base for the CPG recommendations, identified 4312 unique citations, of which 52 were retrieved for full-text evaluation. Eight RCTs met the eligibility criteria and informed panel deliberations. Although there was clinical heterogeneity in the food restrictions evaluated, data were consistent in suggesting that food restrictions lack clinically significant benefit in preventing infections. The panel made two conditional recommendations against the use of food restrictions in a) paediatric patients with cancer receiving chemotherapy and b) in the setting of allogeneic and autologous HCT. The panel developed a good practice statement to emphasise the importance of health care organisations and families adhering to local food safety practices. Interpretation: This CPG provides the first evidence-based recommendations on use of food restrictions to prevent infections in children and adolescents undergoing chemotherapy and paediatric haematopoietic cell transplant recipients. Funding: This CPG was funded and developed through the POGO Guidelines Program.

Simple Summary: Large datasets concerning childhood cancers are rare. Therefore, it is important to fully exploit all available data, which are distributed over several resources, including biomaterials, images, clinical trials, and registries. With privacy-preserving record linkage (PPRL), datasets can be merged, without disclosing the patients' identities. Although PPRL is already implemented or described in various settings, use case descriptions are fragmented and incomplete. The present paper gives an overview of current and future use cases of PPRL in childhood cancer. We screened the literature, projects, and trial protocols, analysed a hypothetical patient journey, and discussed use cases with experts. All the identified use cases were structured along six key dimensions. We conclude that PPRL is a key concept in childhood cancer. Therefore, PPRL strategies should already be considered when starting research projects, to avoid distributed data silos, to maximise the knowledge derived from collected data, and, ultimately, to improve outcomes for children with cancer. Large datasets in paediatric oncology are inherently rare. Therefore, it is paramount to fully exploit all available data, which are distributed over several resources, including biomaterials, images, clinical trials, and registries. With privacy-preserving record linkage (PPRL), personalised or pseudonymised datasets can be merged, without disclosing the patients' identities. Although PPRL is implemented in various settings, use case descriptions are currently fragmented and incomplete. The present paper provides a comprehensive overview of current and future use cases for PPRL in paediatric oncology. We analysed the literature, projects, and trial protocols, identified use cases along a hypothetical patient journey, and discussed use cases with paediatric oncology experts. To structure PPRL use cases, we defined six key dimensions: distributed personalised records, pseudonymisation, distributed pseudonymised records, record linkage, linked data, and data analysis. Selected use cases were described (a) per dimension and (b) on a multi-dimensional level. While focusing on paediatric oncology, most aspects are also applicable to other (particularly rare) diseases. We conclude that PPRL is a key concept in paediatric oncology. Therefore, PPRL strategies should already be considered when starting research projects, to avoid distributed data silos, to maximise the knowledge derived from collected data, and, ultimately, to improve outcomes for children with cancer. [ABSTRACT FROM AUTHOR]

Purpose: Long-term follow-up (LTFU) care for childhood cancer survivors (CCSs) is essential to improve and maintain their quality of life. The Survivorship Passport (SurPass) is a digital tool which can aid in the delivery of adequate LTFU care. During the European PanCareSurPass (PCSP) project, the SurPass v2.0 will be implemented and evaluated at six LTFU care clinics in Austria, Belgium, Germany, Italy, Lithuania and Spain. We aimed to identify barriers and facilitators to the implementation of the SurPass v2.0 with regard to the care process as well as ethical, legal, social and economical aspects. Methods: An online, semi-structured survey was distributed to 75 stakeholders (LTFU care providers, LTFU care program managers and CCSs) affiliated with one of the six centres. Barriers and facilitators identified in four centres or more were defined as main contextual factors influencing implementation of SurPass v2.0. Results: Fifty-four barriers and 50 facilitators were identified. Among the main barriers were a lack of time and (financial) resources, gaps in knowledge concerning ethical and legal issues and a potential increase in health-related anxiety in CCSs upon receiving a SurPass. Main facilitators included institutions' access to electronic medical records, as well as previous experience with SurPass or similar tools. Conclusions: We provided an overview of contextual factors that may influence SurPass implementation. Solutions should be found to overcome barriers and ensure effective implementation of SurPass v2.0 into routine clinical care. Implications for Cancer Survivors: These findings will be used to inform on an implementation strategy tailored for the six centres. [ABSTRACT FROM AUTHOR]

INTRODUCTION: As African countries adopt the global goal of improving childhood cancer survival to 60% by 2030, intentional actions are required to improve nursing. This report aims to describe the current status of paediatric oncology nursing in Africa. METHODS: We report on nursing-related aspects of a survey to map paediatric oncology services in Africa (2018-2019), document perceived nursing strengths and weaknesses (2017) and share nurses research priorities (2019). Additionally, we report on a survey to identify topics for a foundation course (2019) and the expressed perspective of African nurses about the status of paediatric oncology nursing across the continent (2022). RESULTS: Only 21% of respondents in the African mapping survey reported having nurses who care for children with cancer at least 75% of the time. Many centres do not have allied health workers like dieticians and play therapists, thus contributing to the nursing burden of care. The main strength of African paediatric oncology nurses was the humanisation of care, while the major weakness was the lack of training follow-up. The top research priorities focused on professional practice and psychosocial support. The Delphi survey identified 57 topic areas grouped into a 12-module curriculum for nurses new to paediatric oncology. The nurses affirmed their dedication to providing compassionate care, however, noted their vulnerability to harm and called for better specialisation, recognition and remuneration. CONCLUSION: This paper amplifies the voice of African paediatric oncology nurses. It illuminates the room for improvement and provides a reference point for future comparison.

Abstract Background Paediatric oncology/haematology patients and their families are confronted with a life-threatening situation for which music therapy can be a cross-linguistic field of action. The creative act of making music together offers the possibility to strengthen competences and make conflicts tangible. Besides its complementing of evidence-based biomedical care, there is little research on the feasibility and efficacy of interactive music therapy including the diagnosed child and their significant others. Methods We conducted an assessor blind, prospective, multicentric feasibility randomized controlled trial (RCT) with subsequent intervention. Including overall 52 child-significant other dyads, INMUT investigates interaction-focused music therapy with cancer-affected children and their significant others (INMUT-KB; n = 21) compared to music therapy only with the child (MUT-K; n = 21) and a wait-list group (WLG; n = 10). The measurement points include the screening for a cancer diagnosis, psychometric baseline (pre-T1), initial assessment (T1/T2), music therapy sessions (T3–T9), final assessment (T10), final psychometric evaluation (post-T10), and 3-month follow-up (cat-T11). Feasibility and acceptability of the (1) research methodology, (2) intervention and (3) estimation of effect sizes will be assessed using qualitative and quantitative data. The proposed primary outcome includes the parent–child interaction (APCI), and the proposed secondary outcomes refer to subjective goal achievement (GAS), quality of life (KINDL), system-related functional level (EXIS), psychosocial stress (BAS), psychosomatic complaints (SCL-9k), and resources (WIRF). We plan to investigate the efficacy of INMUT-KB and MUT-K post-intervention (post-T10) within the RCT design and at 3-month follow-up (cat-T11). Discussion This study will provide insights into the feasibility of INMUT and the final sample needed for a confirmatory RCT. We will reflect on successfully implemented study procedures and, if necessary, provide recommendations for changes considering the design, procedures, measures, and statistical analyses. The discussion will conclude with an evaluation whether a confirmatory RCT is worth the investment of future resources, including the calculated number of child-significant other dyads needed based on the efficacy trends derived from this feasibility study. Trial registration ClinicalTrials.gov: NCT05534282; date of registration: June 23, 2022.

Abstract Background Globally, children with cancer often experience delays in palliative care referral or are infrequently referred. Therefore, we conducted a qualitative study to gain insight from paediatric oncologists into what enables or deters palliative care referral. Strategic solutions to develop integrated palliative care was a critical study theme. In this paper, we have explained and interpreted these strategic solutions through the lens of feedback intervention theory. Methodology The study findings were interpreted using Kumar’s six-step approach that enabled systematic evaluation of a theory’s appropriateness and alignment with the researcher’s paradigm, methodology, and study findings. It also explained how theory informed analysis and elucidated challenges or the development of new models. The feedback intervention theory appraises the discrepancy between actual and desired goals and provides feedback to improve it. Results Strategic solutions generated from the study findings were coherent with the aspects elucidated in theory, like coping mechanisms, levels of feedback hierarchy, and factors determining the effect of the feedback intervention on performance. Paediatric oncologists suggested integrating palliative care providers in the team innocuously, improving communication between teams, relabelling palliative care as symptom control, and working with a skilled and accessible palliative care team. The paper proposes an infinite loop model developed from the study, which has the potential to foster integrated palliative care through excellent collaboration and continuous feedback. Conclusion Applying feedback intervention theory can bridge the gap between actual and desired practice for integrated cancer palliative care in paediatric oncology.

Abstract The gut microbiota of paediatric oncology patients undergoing a conditioning regimen before hematopoietic stem cell transplantation is recently considered to play role in febrile neutropenia. Disruption of commensal microbiota and evolution of opportune pathogens community carrying a plethora of antibiotic-resistance genes play crucial role. However, the impact, predictive role and association of patient´s gut resistome in the course of the therapy is still to be elucidated. We analysed gut microbiota composition and resistome of 18 paediatric oncology patients undergoing hematopoietic stem cell transplantation, including 12 patients developing febrile neutropenia, hospitalized at The Bone Marrow Transplantation Unit of the National Institute of Children´s disease in Slovak Republic and healthy individuals (n = 14). Gut microbiome of stool samples obtained in 3 time points, before hematopoietic stem cell transplantation (n = 16), one week after hematopoietic stem cell transplantation (n = 16) and four weeks after hematopoietic stem cell transplantation (n = 14) was investigated using shotgun metagenome sequencing and bioinformatical analysis. We identified significant decrease in alpha-diversity and nine antibiotic-resistance genes msr(C), dfrG, erm(T), VanHAX, erm(B), aac(6)-aph(2), aph(3)-III, ant(6)-Ia and aac(6)-Ii, one week after hematopoietic stem cell transplantation associated with febrile neutropenia. Multidrug-resistant opportune pathogens of ESKAPE, Enterococcus faecium, Staphylococcus aureus, Klebsiella pneumoniae and Escherichia coli found in the gut carried the significant subset of patient’s resistome. Over 50% of patients treated with trimethoprim/sulfamethoxazole, piperacillin/tazobactam and amikacin carried antibiotic-resistance genes to applied treatment. The alpha diversity and the resistome of gut microbiota one week after hematopoietic stem cell transplantation is relevant predictor of febrile neutropenia outcome after hematopoietic stem cell transplantation. Furthermore, the interindividual diversity of multi-drug resistant opportunistic pathogens with variable portfolios of antibiotic-resistance genes indicates necessity of preventive, personalized approach.

Background: Paediatric oncology/haematology patients and their families are confronted with a life-threatening situation for which music therapy can be a cross-linguistic field of action. The creative act of making music together offers the possibility to strengthen competences and make conflicts tangible. Besides its complementing of evidence-based biomedical care, there is little research on the feasibility and efficacy of interactive music therapy including the diagnosed child and their significant others. Methods: We conducted an assessor blind, prospective, multicentric feasibility randomized controlled trial (RCT) with subsequent intervention. Including overall 52 child-significant other dyads, INMUT investigates interaction-focused music therapy with cancer-affected children and their significant others (INMUT-KB; n = 21) compared to music therapy only with the child (MUT-K; n = 21) and a wait-list group (WLG; n = 10). The measurement points include the screening for a cancer diagnosis, psychometric baseline (pre-T1), initial assessment (T1/T2), music therapy sessions (T3–T9), final assessment (T10), final psychometric evaluation (post-T10), and 3-month follow-up (cat-T11). Feasibility and acceptability of the (1) research methodology, (2) intervention and (3) estimation of effect sizes will be assessed using qualitative and quantitative data. The proposed primary outcome includes the parent–child interaction (APCI), and the proposed secondary outcomes refer to subjective goal achievement (GAS), quality of life (KINDL), system-related functional level (EXIS), psychosocial stress (BAS), psychosomatic complaints (SCL-9k), and resources (WIRF). We plan to investigate the efficacy of INMUT-KB and MUT-K post-intervention (post-T10) within the RCT design and at 3-month follow-up (cat-T11). Discussion: This study will provide insights into the feasibility of INMUT and the final sample needed for a confirmatory RCT. We will reflect on successfully implemented study procedures and, if necessary, provide recommendations for changes considering the design, procedures, measures, and statistical analyses. The discussion will conclude with an evaluation whether a confirmatory RCT is worth the investment of future resources, including the calculated number of child-significant other dyads needed based on the efficacy trends derived from this feasibility study. Trial registration: ClinicalTrials.gov: NCT05534282; date of registration: June 23, 2022. [ABSTRACT FROM AUTHOR]

Background: Globally, children with cancer often experience delays in palliative care referral or are infrequently referred. Therefore, we conducted a qualitative study to gain insight from paediatric oncologists into what enables or deters palliative care referral. Strategic solutions to develop integrated palliative care was a critical study theme. In this paper, we have explained and interpreted these strategic solutions through the lens of feedback intervention theory. Methodology: The study findings were interpreted using Kumar's six-step approach that enabled systematic evaluation of a theory's appropriateness and alignment with the researcher's paradigm, methodology, and study findings. It also explained how theory informed analysis and elucidated challenges or the development of new models. The feedback intervention theory appraises the discrepancy between actual and desired goals and provides feedback to improve it. Results: Strategic solutions generated from the study findings were coherent with the aspects elucidated in theory, like coping mechanisms, levels of feedback hierarchy, and factors determining the effect of the feedback intervention on performance. Paediatric oncologists suggested integrating palliative care providers in the team innocuously, improving communication between teams, relabelling palliative care as symptom control, and working with a skilled and accessible palliative care team. The paper proposes an infinite loop model developed from the study, which has the potential to foster integrated palliative care through excellent collaboration and continuous feedback. Conclusion: Applying feedback intervention theory can bridge the gap between actual and desired practice for integrated cancer palliative care in paediatric oncology. [ABSTRACT FROM AUTHOR]

Optimal nutritional support in childhood cancer relies on the adequate provision of energy. This study investigated the impact of chemotherapy on resting energy expenditure (REE) during the first six months of treatment and the accuracy of predictive equations in calculating said requirements of newly diagnosed children with cancer. REE was measured at diagnosis utilising a validated bioelectrical impedance analysis (BIA) mobile unit and compared with three predictive equations (Schofield 1985, World Health Organization [WHO] 1985 and the Recommended Dietary Allowance [RDA] 1989). Agreement and accuracy of these equations were tested by determining bias and agreement rates and displayed using the Bland–Altman plot. Baseline values were plotted against monthly follow-up measurements over time. Statistical significance was 5% and a priori limits of agreement set between 90% and 110% of measured REE. Forty-three newly diagnosed children with median age 4 years (IQR 2.0–7.6) were measured prior to chemotherapy initiation. Compared with measured REE (mean ± SD) 719.53 ± 206.29 kcal/day, all predictive equations significantly overestimated REE: WHO 1985 (889.75 ± 323.31 kcal/day; 23% overestimation), Schofield 1985 (899.62 ± 336.10 kcal/day; 25% overestimation) and RDA (1647.67 ± 481.06 kcal/day; 129% overestimation) (p < 0.001). Despite significant proportionate bias in all three equations (p < 0.001), the intra-class consistency coefficient showed good reliability for the Schofield 1985 (0.864) and WHO 1985 (0.849) equations. Though statistically significant (chi-square = 23.11, p < 0.003), the overall 1 kcal/kg (1.3%) increase for all cancer types at six months may not be clinically significant. Existing predictive equations are unable to calculate REE accurately at childhood cancer diagnosis, highlighting the need for future investigations into the development of cancer-specific equations. [ABSTRACT FROM AUTHOR]

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The gut microbiota of paediatric oncology patients undergoing a conditioning regimen before hematopoietic stem cell transplantation is recently considered to play role in febrile neutropenia. Disruption of commensal microbiota and evolution of opportune pathogens community carrying a plethora of antibiotic-resistance genes play crucial role. However, the impact, predictive role and association of patient´s gut resistome in the course of the therapy is still to be elucidated. We analysed gut microbiota composition and resistome of 18 paediatric oncology patients undergoing hematopoietic stem cell transplantation, including 12 patients developing febrile neutropenia, hospitalized at The Bone Marrow Transplantation Unit of the National Institute of Children´s disease in Slovak Republic and healthy individuals (n = 14). Gut microbiome of stool samples obtained in 3 time points, before hematopoietic stem cell transplantation (n = 16), one week after hematopoietic stem cell transplantation (n = 16) and four weeks after hematopoietic stem cell transplantation (n = 14) was investigated using shotgun metagenome sequencing and bioinformatical analysis. We identified significant decrease in alpha-diversity and nine antibiotic-resistance genes msr(C), dfrG, erm(T), VanHAX, erm(B), aac(6)-aph(2), aph(3)-III, ant(6)-Ia and aac(6)-Ii, one week after hematopoietic stem cell transplantation associated with febrile neutropenia. Multidrug-resistant opportune pathogens of ESKAPE, Enterococcus faecium, Staphylococcus aureus, Klebsiella pneumoniae and Escherichia coli found in the gut carried the significant subset of patient's resistome. Over 50% of patients treated with trimethoprim/sulfamethoxazole, piperacillin/tazobactam and amikacin carried antibiotic-resistance genes to applied treatment. The alpha diversity and the resistome of gut microbiota one week after hematopoietic stem cell transplantation is relevant predictor of febrile neutropenia outcome after hematopoietic stem cell transplantation. Furthermore, the interindividual diversity of multi-drug resistant opportunistic pathogens with variable portfolios of antibiotic-resistance genes indicates necessity of preventive, personalized approach. [ABSTRACT FROM AUTHOR]

Summary: Background: Taurolidine containing lock solutions (TL) are a promising method for the prevention of central line associated bloodstream infections. Per accident, the TL may not always be aspirated from the central venous catheter (CVC) before blood cultures are obtained. The TL could, unintentionally, end up in a blood culture vial, possibly altering the results. The aim of this study was to investigate the effect of the TLs on the detection of microbial growth in blood culture vials. Methods: Different lock solutions (taurolidine-citrate-heparin (TCHL), taurolidine, heparin, citrate or NaCl) were added to BD BACTECTM blood culture vials (Plus Aerobic/F, Lytic/10 Anaerobic/F or Peds Plus/F) before spiking with Staphylococcus aureus (ATCC 29213 or a clinical strain) or Escherichia coli (ATCC 25922 or a clinical strain) in the presence and absence of blood. Subsequently, blood culture vials were incubated in the BD BACTEC FX instrument with Time-to-positivity (TTP) as primary outcome. In addition, the effect of the TCHL on a variety of other micro-organisms was tested. Discussion: In the presence of taurolidine, the TTP was considerably delayed or vials even remained negative as compared to vials containing heparin, citrate or NaCl. This effect was dose-dependent. The delayed TTP was much less pronounced in the presence of blood, but still notable. Conclusion: This study stresses the clinical importance of discarding TLs from the CVC before obtaining a blood culture.

Inequalities in research and innovations affect childhood cancer survival across Europe. Vilnius University Hospital Santaros Klinikos (VULSK, the coordinator) and eight research-intensive institutions from seven European countries implemented the TREL project (Twinning in Research and Education to improve survival in childhood solid tumours in Lithuania) supported by the Horizon 2020 Widening programme. TREL aimed to enhance translational, clinical, and survivorship research in paediatric CNS, neuroblastoma, and renal tumours to improve future treatment outcomes in Lithuania. From January 2021 to December 2023, 49 VULSK professionals and 55 peers from partner institutions collaborated in this twinning program. Achievements after three years were: nine educational events, the initiation of basic and clinical research on fertility preservation, ten VULSK researchers joining international research groups, six signed agreements to participate in international academic clinical trials and the implementation of the European Survivorship Passport. Thirty patients received individual treatment recommendations following multidisciplinary discussions with experts from partner institutions. Twenty-five rare genetic variants were classified by the twinning bioinformatician teams with direct consequences on patient management. In conclusion, coordination of the Horizon 2020 project enhanced VULKS’s research capacities, networking channels and attractiveness for industry and academia-initiated innovative actions that will improve survival rates in the long run.

Introduction: Scarcity of age appropriate formulations for orally administered medicines for paediatric malignancies is a critical and ongoing issue which necessitates urgent solutions. According to JARC Survey 27 % of oral medicines were never available in child-friendly formulations (Vassal et al., 2021 [2]). Pharmacists from the European Society of Oncology Pharmacy Global (ESOP) and the European Society for Pediatric Oncology (SIOPE) have collaborated to provide a document summarising literature data on this topic and a set of practical instructions on the preparation of extemporaneous oral liquid medicines. Material and methods: Literature review was conducted for the preparation of oral medicines for paediatric cancer, through Pubmed 2.0. A table was drawn up with necessary information for medicine preparation. We adapted the classification model of the molecule stability ranking of the International database of stability for injectable drugs, Stabilis® (Class A–C). Results: A total of 126 articles were selected and analysed. All commercially available marketed liquid oral medications used in paediatric cancer were overviewed using globally accessible drug databases and compiled in a table with appropriate indications. Based on the literature review, 28 formulations for 13 different active ingredients for chemotherapy and 35 formulations for 16 different active ingredients for supportive therapy were compiled. Conclusions: Development of child-appropriate formulations of anticancer medicines by the pharmaceutical industry should be incentivised towards marketing authorisation to enhance accessibility. The results of this study could help facilitate creation of European standards for extemporaneous preparation and persuade researchers in the field of paediatric oncology on the way forward.

Background: Having sufficient knowledge of cancer diagnosis, treatment and late effects in survivors of childhood, adolescent, and young adult (CAYA) cancer is important for effective self-management and optimising health outcomes. Therefore, in collaboration with different stakeholders, the PanCare PLAIN Information Group converted the PanCareFollowUp Recommendations for late effects surveillance into information summaries that are Person-centred, written in Lay language, Accessible, Internationally relevant, and Navigable (PLAIN). Methods: The PanCare PLAIN Information Group, comprising 21 stakeholders from seven European countries, collaborated to provide concise information for survivors and their families. The aim was to deliver PLAIN summaries that are clear and accessible for the majority of survivors, while providing links to additional sources of information. The PLAIN summaries were drafted by the PanCare PLAIN Information Group and subjected to two internal and one external consultation round, the latter involving experts, CAYA cancer survivors and parents/caregivers. Results: In total, 45 PLAIN summaries were developed, each corresponding to one of the PanCareFollowUp Recommendations for late effects surveillance. The summaries provide information about late effects, personal health risks, important symptoms and signs, recommended surveillance strategies, possible referral and treatment options, and self-care. Conclusions: The PLAIN summaries are meant to increase knowledge in survivors and their families, while they may also inform healthcare professionals. Along with their translations, the PLAIN summaries will be made freely available on the PanCare website, with a link provided on the European Network of Youth Cancer Survivors information platform. In addition, they will become and integral part of the Survivorship Passport.

INTRODUCTION: Improved treatment regimens have led to increased survival rates among childhood cancer survivors (CCS), and more than 84% of all children diagnosed with cancer will experience long-term survival or cure. Survivors are susceptible to late effects of cancer treatment often requiring lifelong follow-up care, as many of these conditions can be prevented or mitigated with surveillance. Integrating primary care (PC) and childhood cancer survivorship care can improve follow-up for survivors, however, little integrative research exists. This scoping review aims to: identify and describe existing models of care that integrate PC and childhood cancer survivorship care, examine the effectiveness of these models of care, and characterise the barriers and facilitators for the integration of PC for CCS. METHODS AND ANALYSIS: A comprehensive empirical literature search of three electronic databases (PubMed, CINAHL, and Embase) was employed to identify potentially relevant citations on 1 October 2020. The population, independent variables/intervention, comparator, outcomes, timing, setting and study design/other limiters (PICOTSS) framework was used to inform protocol development. The Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) checklist and explanation will be used to report study findings. The search strategy will be completed again prior to publication to ensure recent empirical research is accounted for. ETHICS AND DISSEMINATION: This research is exempt from Institutional Review Board (IRB) review. Approval from a research ethics board for this study was not required as it does not involve human participants or unpublished secondary data. The findings from this scoping review will be disseminated through peer-reviewed scientific manuscripts, clinical conference presentations, professional networks and digital communications using social media platforms such as Twitter. This study has been registered with Open Science Framework: https://osf.io/92xbg.

A chemoport is widely used in paediatric oncology population. Removal is a relatively easy procedure, but difficulty can be encountered in case the catheter is densely adherent to the vascular wall. It is a rare complication and is associated with long indwelling duration and acute lymphoblastic leukaemia (ALL). Forceful traction can lead to vascular injury and high morbidity. Herein, we report a 7-year-old girl with precursor B ALL who had delayed chemoport removal due to the coronavirus disease (COVID-19) pandemic. The removal process was difficult, as the catheter was adherent to the right innominate vein. Out of panic, the surgeon pulled it out forcefully. Fortunately, the catheter and its fragment were successfully retrieved completely and the child was discharged the next day. The management strategy varies and ranges from minimally invasive to open surgery. Leaving a stuck chemoport catheter in situ can be a bailout method or part of conservative management. [ABSTRACT FROM AUTHOR]

BackgroundThe emergence of COVID-19 pandemic has led to heightened fear and uncertainty among parents of children with cancer. This study was conducted to evaluate the parental perceptions toward effects of COVID-19 infection to children with cancer, determine their stress level and factors contributing to high stress level during the pandemic.MethodsThis cross-sectional study was conducted in three paediatric oncology centres in Malaysia from September 2020 until December 2022. A total of 167 parents were recruited. Parents completed a set of questionnaires to assess their perception on effect of COVID-19 infection to children with cancer and COVID Stress Scale (CSS) to assess the parents’ stress level.ResultsPatients’ mean age at study entry was 8.75 years (SD 4.38). Ninety-one (54.5%) patients were still on active treatment. More than 80% of the parents obtained information regarding COVID-19 infection from mass media and social networking. Fear of their children contracting COVID-19 infection was high especially among patients who were still on treatment. Forty-nine (29.3%) parents were significantly affected by the pandemic leading to loss of job or monthly income. Twenty-nine (17.4%) patients required treatment modification during the pandemic. The median total score for CSS was 78.0 (IQR 25th 64.0; 75th 95.0). Ninety-one (54.5%) respondents were very/extremely stressed based on the CSS scores. Components with high scores were xenophobia (median score 18.0; IQR 25th 13.0, 75th 22.0), fear of danger (median score 17.0; IQR 25th 14.0, 75th 20.0) and contamination fears (median score 16.0; IQR 25th 12.0, 75th 19.0). Lower household income was associated with higher stress level (p = 0.006).ConclusionOur study demonstrated high awareness regarding risk of COVID-19 infection among parents of oncology children. Half of the parents had high stress level, with low household income identified as a factor associated with high stress level.

Background: Studies done in South Africa show that Rhabdomyosarcomas (RMS) comprises 6 % of childhood malignancies. Very few centres in South Africa (SA) have reported their management and outcomes of children with RMS, and as such, it is difficult to compare outcomes and come up with management protocols befitting our environment. Aim: To describe the management and outcomes of children with RMS at Chris Hani Baragwanath Academic Hospital (CHBAH). Setting: The Departments of Paediatric Surgery and Paediatric Oncology. Methods: A retrospective review of clinical records of patients below 18 years of age with RMS, managed from 01 January 2008 to 31 December 2017. Results: Fifty-eight patients had RMS, 77 % embryonal and 21 % alveolar subtypes. Primary tumour site was favourable in 48 %, and unfavourable in 45 %. Thirty-three patients (57%,) had surgery for the primary tumour, whilst 25 patients (43 %) did not have surgery. Post-operative clinical groups were 29 % group I, 9 % group II, 43 % group III, and 19 % group IV. The overall 5-year survival was 55%. Predictors for a good outcome included early disease stage at presentation, favourable site, embryonal subtype, and surgery for the primary tumour. Conclusion: The 5-year survival of 55 % from this study is low when compared to high income countries but is comparable to middle income countries. The main factor contributing to mortality is patients presenting with unresectable advanced disease in unfavourable sites. Surgical resection plays a major role in improving outcomes. Level of evidence: IV

Summary: Background: Medulloblastoma patients with a sub-total surgical resection (STR; >1.5 cm2 primary tumour residuum post-surgery) typically receive intensified treatment. However, the association of STR with poor outcomes has not been observed consistently, questioning the validity of STR as a high-risk disease feature. Methods: We collected extent of resection (EOR) data from 1110 patients (from UK CCLG centres (n = 416, collected between September 1990 and July 2014) and published (n = 694) cohorts), the largest cohort of molecularly and clinically annotated tumours assembled to specifically assess the significance of EOR. We performed association and univariable/multivariable survival analyses, assessing overall survival (OS) cohort-wide and with reference to the four consensus medulloblastoma molecular groups and clinical features. Findings: STR was reported in 20% (226/1110) of patients. Non-WNT (p = 0.047), children

ObjectivesPaediatric oncologists often encounter challenges when seeking compassionate access to off-label therapies for their patients. This study employed implementation science and co-design techniques to develop the ProCure medicines database, with the goal of streamlining the application process and addressing identified barriers in paediatric oncology.MethodsThis study utilised an exploratory qualitative research design. Seventeen healthcare providers, including oncologists, nurse consultants, and allied health professionals, participated in semi-structured interviews guided by the Consolidated Framework for Implementation Research (CFIR) and a visual process map aid. Deductive qualitative data analysis, according to the CFIR constructs, identified key barriers and facilitators. Collaborative design sessions engaged multidisciplinary teams to develop the ProCure beta version.ResultsBarriers to off-label therapy access included resource-intensive applications, time sensitive decision-making, and complex pharmaceutical information. Facilitators included Drug Access Navigators, Molecular Tumour Boards, and a multi-disciplinary approach. ProCure addressed end-user needs by centralising medicines information. Additional features suggested by healthcare providers included blood–brain-barrier penetrability data and successful application examples.ConclusionProCure represents a promising solution to the challenges paediatric oncologists face in accessing off-label therapies. By centralising information, it simplifies the application process, aids decision-making, and promotes a collaborative approach to patient care. The potential of the database to stream and enhance off-label therapy access underscores its relevance in improving paediatric oncology practise. Further research and implementation efforts are warranted to assess ProCure’s real-world impact and refine its features based on user feedback.

Background: The substantial increase in smartphone ownership has led to a rise in mobile health (mHealth) app use. Developing tailored features through mHealth apps creates a pathway to address the health care needs of pediatric patients with cancer and their families who have complex care needs. However, few apps are designed specifically to integrate with pediatric cancer care. Objective: This study reports a systematic search and analysis of mHealth apps available on the Apple App (iOS) and Google Play (Android) stores designed for pediatric cancer through a list of features that serve (1) patients, (2) caregivers, or (3) both audiences. Methods: Following PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, we reviewed apps for pediatric patients with cancer and caregivers available as of January 30, 2024. We searched the Apple App and Google Play stores with a list of keyword combinations focusing on pediatric cancer care. The inclusion criteria were (1) specifically apps targeted toward pediatric patients with cancer, their families, or both; (2) available in either app store; and (3) available in English. Apps were assessed using the Mobile Application Rating Scale (MARS). The MARS is a quality assessment for mHealth apps, including components of engagement, functionality, aesthetics, and informational quality (5-point Likert scale items—1: low and 5: high quality). Results: In total, 22 apps were identified and 17 of those apps were available on both platforms. The most popular features (n=12) were resource sharing, symptom tracking, reminders, care team connections, journaling, community support, medication tracking, data visualizations, and appointment tracking. Features and interfaces were designed for caregivers (n=9) more frequently than the patients (n=7) while a subset of apps created options for both users (n=6). A total of 16 apps received positive reviews (mean 4.4, SD 0.59; Min=3.1, Max=5.0). A small subset (n=3) achieved over 5000 downloads; however, the majority (n=15) had fewer than 500. More than half (n=12) of the apps were not available in English. Apps requested access to a range of device functionalities to operate (mean 2.72, SD 3.13; Min=0, Max=10). Out of 22, a total of 17 apps were publicly accessible. The mean MARS scores for the apps ranged from 1.71 (SD 0.75) to 4.33 (SD 0.82). Overall, apps scored high on functionality (mean 3.72, SD 0.54) but low on engagement (mean 3.02, SD 0.93). Conclusions: Our review highlights the promising yet underdeveloped potential of mHealth apps in pediatric oncology care, underscoring the need for more inclusive, comprehensive, and integrative digital health solutions. Future developments should actively involve key stakeholders from the pediatric oncology community, including patients, families, and health care professionals, to ensure the apps meet specific needs while addressing linguistic and cultural barriers. [ABSTRACT FROM AUTHOR]

Simple Summary: Central nervous system (CNS) tumours are highly common solid neoplasms in children and adolescents. Survival remains low in many countries, including Spain. While some studies have shown a rise in the incidence of these tumours in Europe, others have not. This study, the first in Spain, focused on two questions: (1) Is the incidence of CNS tumours increasing in Spanish children and adolescents? and (2) Has the survival of these patients improved? We analysed incidence in Spain across the period 1983–2007 and survival from 1991 to 2005, according to the International Childhood Cancer Classification. The incidence results revealed a stabilisation in children's overall incidence trend since the early 1990s similar to that of Southern Europe. Overall survival was lower than that in Europe, without any improvement from 1991 to 2005. Our results provide a baseline for assessing current incidence and the achievements of paediatric oncology with regard to CNS tumours in children and adolescents. Background: Central nervous system (CNS) neoplasms are highly frequent solid tumours in children and adolescents. While some studies have shown a rise in their incidence in Europe, others have not. Survival remains limited. We addressed two questions about these tumours in Spain: (1) Is incidence increasing? and (2) Has survival improved? Methods: This population-based study included 1635 children and 328 adolescents from 11 population-based cancer registries with International Classification of Childhood Cancer Group III tumours, incident in 1983–2007. Age-specific and age-standardised (world population) incidence rates (ASRws) were calculated. Incidence time trends were characterised using annual percent change (APC) obtained with Joinpoint. Cases from 1991 to 2005 (1171) were included in Kaplan–Meier survival analyses, and the results were evaluated with log-rank and log-rank for trend tests. Children's survival was age-standardised using: (1) the age distribution of cases and the corresponding trends assessed with Joinpoint; and (2) European weights for comparison with Europe. Results: ASRw 1983–2007: children: 32.7 cases/106; adolescents: 23.5 cases/106. The overall incidence of all tumours increased across 1983–2007 in children and adolescents. Considering change points, the APCs were: (1) children: 1983–1993, 4.3%^ (1.1; 7.7); 1993–2007, −0.2% (−1.9; 1.6); (2) adolescents: 1983–2004: 2.9%^ (0.9; 4.9); 2004–2007: −7.7% (−40; 41.9). For malignant tumours, the trends were not significant. 5-year survival was 65% (1991–2005), with no significant trends (except for non-malignant tumours). Conclusions: CNS tumour incidence in Spain was found to be similar to that in Europe. Rises in incidence may be mostly attributable to changes in the registration of non-malignant tumours. The overall malignant CNS tumour trend was compatible with reports for Southern Europe. Survival was lower than in Europe, without improvement over time. We provide a baseline for assessing current paediatric oncology achievements and incidence in respect of childhood and adolescent CNS tumours. [ABSTRACT FROM AUTHOR]

This paper presents a case of a 16-year-old boy with dyspnoea and chest pain after injury with changes in the lung observed on the radiograph that may have suggested post-traumatic fluid. Further examinations revealed atelectasis that was caused by compression of the left lung by fluid that originated from pulmonary metastases of the tumour located in the left testicle. Vigilance should be maintained during the physical examination and history taking. Initial symptoms may suggest a traumatic aetiology, while the cause could be different and an acute or chronic process unrelated to trauma should be considered. [ABSTRACT FROM AUTHOR]

Background: We previously demonstrated in a large multicentre point prevalence study (PPS) a marked variability across German and Austrian centres regarding the management of fever and neutropenia (FN) in children, and a high rate of inappropriate treatments compared to recommendations in the German national FN guidelines. Methods: We analysed local FN standard operating procedures (SOPs) of participating centres and rated their concordance with the German national FN guidelines. To this end, we defined items derived from the German national FN guidelines that we considered essential for any local FN SOP, and assigned points per items. The items comprised “basic requirements of a SOP”; “risk analysis”; “diagnostic approach”; and “use of antibiotics including dosing recommendations”, including sub-categories. Results: Of the 30 participating centres’ SOPs, 29 were of sufficient granularity for detailed analysis. Only 19/29 (66%) and 20/29 (69%) of the SOPs provided a definition of fever and of neutropenia, respectively. The top scoring sub-categories were “empiric treatment” (mean percentage 69%), “laboratory investigations” (62.4%), and “SOP basics” (59.7%). The worst scoring sub-categories were “definitions” (37.7%), “risk analysis” (32.3%), and “outpatient treatment” (15.7%). Conclusions: The majority of the local FN SOPs demonstrated a lack of concordance with the German national guidelines on the management of paediatric FN. These discrepancies may explain the high rate of inappropriate antimicrobial treatments in our previous PPS. Our data indicate that local SOPs should be better adapted to national guidelines, and national guidelines should be conceived with the feedback of end-users, thereby anticipating barriers and facilitating acceptance.

Osteonecrosis (ON) is a common disabling complication of treatment for patients with acute lymphoblastic leukaemia (ALL). Reported incidence rates range from 1% to 61% and multiple possible risk factors have been identified. This review explored existing evidence to provide new perspectives and recommendations for future interdisciplinary research. PEDro, CINAHL, AMED, EMBSAE, OVID, EMCARE databases were systematically searched from their inception to March 2022. Published original research reporting the incidence rates of osteonecrosis in patients aged 10–25 with ALL were included. Study reporting quality was assessed against appropriate reporting guidelines (STROBE, CONSORT and CROSS). All relevant data reporting incidence rates and risk factors were extracted for narrative synthesis. 3146 report titles were screened, with 34 studies included (n = 12,056) (30 observational cohort studies, three randomised trials, and one questionnaire study). The median study quality reporting score was 68% (IQR 64–82%). Median overall incidence rate of ON was 51.8% (IQR 41.4–58.9%) and 15.65% (IQR 9.2–24.2%) for asymptomatic and symptomatic patient screening respectively. Five possible risk factor categories were identified: sex assigned at birth, age, ethnicity, steroid regimen, and genotype. The female sex and white ethnicity were consistently reported as risk factors independently associated with an increased risk of osteonecrosis in all studies. A heterogenous body of literature with moderate reporting quality identified a high incidence rate of osteonecrosis in patients with ALL. Future research investigating the efficacy of stratified treatments that focus on reducing the risk of osteonecrosis through modification of steroid regimen particularly in females of white ethnicity is needed. Obtaining multidisciplinary consensus with regards to screening methodologies and intervention outcomes may also help to improve evidence synthesis in this area. This may in turn facilitate early diagnosis and improve long term patient outcomes through treatment regimen modification and possible prevention of ON progression.

Abstract Background More effective incentives are needed to motivate paediatric oncology drug development, uncoupling it from dependency on adult drug development. Although the current European and North‐American legislations aim to promote drug development for paediatrics and rare diseases, children and adolescents with cancer have not benefited as expected from these initiatives and cancer remains the first cause of death by disease in children older than one. Drug development for childhood cancer remains dependent on adult cancer indications and their potential market. The balance between the investment needed to execute a Paediatric Investigation Plan (PIP) in Europe and an initial Paediatric Study Plan (iPSP) in the US, coupled with the potential financial reward has not been sufficiently attractive to incite the pharmaceutical industry to develop drugs for rare indications such as childhood cancer. Methods We propose changes in the timing and nature of the rewards within the European Paediatric Medicine Regulation (PMR) and Regulation on Orphan Medicinal Products (both currently under review), which would drive earlier initiation of paediatric oncology studies and provide incentives for drug development specifically for childhood indications. Results We suggest modifying the PMR to ensure mechanism‐of‐action driven mandatory PIP and reorganization of incentives to a stepwise and incremental approach. Interim and final deliverables should be defined within a PIP or iPSP, each attracting a reward on completion. A crucial change would be the introduction of the interim deliverable requiring production of paediatric data that inform the go/no‐go decisions on whether to take a drug forward to paediatric efficacy trials. Conclusion Additionally, to address the critical gap in the current framework where there is a complete lack of incentives to promote paediatric‐specific cancer drug development, we propose the introduction of early rewards in the Orphan Regulation, with a variant on the US‐Creating Hope Act and its priority review vouchers.

Abstract Background Research is crucial to improve treatment, survival and quality of life for children with cancer. However, recruitment of children for research raises ethical challenges. The aim of this study was to explore and describe ethical values and challenges related to the recruitment of children with cancer for research, from the perspectives and experiences of healthcare professionals in the Swedish context. Another aim was to explore their perceptions of research ethics competence in recruiting children for research. Methods An explorative qualitative study using semi-structured interviews with key informants. Seven physicians and ten nurses were interviewed. Interviews were analysed using inductive qualitative content analysis. Results The respondents’ ethical challenges and values in recruitment mainly concerned establishing relationships and trust, meeting informational needs, acknowledging vulnerability, and balancing roles and interests. Ensuring ethical competence was raised as important, and interpersonal and communicative skills were highlighted. Conclusion This study provides empirical insight into recruitment of children with cancer, from the perspectives of healthcare professionals. It also contributes to the understanding of recruitment as a relational process, where aspects of vulnerability, trust and relationship building are important, alongside meeting informational needs. The results provide knowledge on the complexities raised by paediatric research and underpin the importance of building research ethics competence to ensure that the rights and interests of children with cancer are protected in research.

Abstract Background Despite complete resection of mature ovarian teratoma, there remains a risk of metachronous contralateral disease with implications for further surgery and fertility. Current estimates of this risk are wide and practice regarding surveillance varies. We aimed to identify the incidence of metachronous contralateral disease in girls presenting with unilateral mature ovarian teratoma and to describe current follow-up. Methods Retrospective case note review was performed for all girls (

Paediatric oncology patients who develop severe chemotherapy-induced toxicity that requires dose reduction, delay or termination of treatment are at risk of decreased treatment efficacy. Previous research has provided evidence that genetic variants in TPMT, NUDT15, UGT1A1 and DPYD are associated with toxicity of anticancer drugs. This led to pharmacogenetic guidelines that are integrated into clinical practice in paediatric oncology. Recently, novel genetic variants have been associated with a higher risk of developing chemotherapy-induced toxicity. In this case series, we selected 21 novel variants and genotyped these in nine patients with excessive chemotherapy-induced toxicity using whole exome sequencing or micro-array data. We observed that six out of nine patients carried at least one variant that, according to recent studies, potentially increased the risk of developing methotrexate- or vincristine-induced toxicity. As patient-derived genetic data are becoming widely accessible in paediatric oncology, these variants could potentially enter clinical practice to mitigate chemotherapy-induced toxicity. [ABSTRACT FROM AUTHOR]

Background: This systematic review has been conducted with the aim of characterizing cognitive deficits and analyzing their frequency in survivors of paediatric Central Nervous System tumours. Materials and methods: All literature published up to January 2023 was retrieved searching the databases "PubMed", "Cochrane", "APA PsycInfo" and "CINAHL". The following set of pre-defined inclusion criteria were then individually applied to the selected articles in their full-text version: i) Retrospective/prospective longitudinal observational studies including only patients diagnosed with primary cerebral tumours at = 21 years (range 0-21); ii) Studies including patients evaluated for neuro-cognitive and neuro-psychological deficits from their diagnosis and/or from anti-tumoral therapies; iii) Studies reporting standardized tests evaluating patients' neuro-cognitive and neuro-psychological performances; iv) Patients with followups = 2 years from the end of their anti-tumoral therapies; v) Studies reporting frequencies of cognitive deficits. Results: 39 studies were included in the analysis. Of these, 35 assessed intellectual functioning, 30 examined memory domains, 24 assessed executive functions, 22 assessed attention, 16 examined visuo-spatial skills, and 15 explored language. A total of 34 studies assessed more than one cognitive function, only 5 studies limited their analysis on a single cognitive domain. Attention impairments were the most recurrent in this population, with a mean frequency of 52.3% after a median period post-treatment of 11.5 years. The other cognitive functions investigated in the studies showed a similar frequency of impairments, with executive functions, language, visuospatial skills and memory deficits occurring in about 40% of survivors after a similar post-treatment period. Longitudinal studies included in the systematic review showed a frequent decline over time of intellectual functioning. Conclusions: Survivors of paediatric Central Nervous System tumours experience cognitive sequelae characterized by significant impairments in the attention domain (52.3%), but also in the other cognitive functions. Future studies in this research field need to implement more cognitive interventions and effective, but less neurotoxic, tumour therapies to preserve or improve neurocognitive functioning and quality of life of this population. [ABSTRACT FROM AUTHOR]