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Background: Osteosarcoma is the most common malignant bone tumor in children and adolescents. Conventional chemotherapy remains unsatisfactory due to drug toxicity and resistance issues. Therefore, there is an urgent need to develop more effective treatments for advanced osteosarcoma. In the current study, we focused on evaluating the anticancer efficacy of avermectin B1, a novel avermectin analog, against osteosarcoma cells. Methods: The half-inhibitory concentration of avermectin B1 was calculated in three osteosarcoma cell lines. Then, functional experiments were conducted to evaluate the effects of avermectin B1 on cell proliferation, the cell cycle, apoptosis and autophagy. Moreover, the AMPK/ULK1 signaling pathway was detected by Western blot assay. Finally, the in vivo effect of avermectin B1 on tumor growth and metastasis was investigated using the xenograft mouse model. To examine the role of the AMPK/ULK1 pathway, an AMPK-specific inhibitor (dorsomorphin) was used in combination with avermectin B1. Results: Avermectin B1 inhibited the proliferation of osteosarcoma cells in a dose-dependent manner based on CCK8 and colony formation assays. Then, it was found to inhibit migration and invasion by wound healing assay and cell migration and invasion assay. In addition, avermectin B1 induced osteosarcoma cell apoptosis and autophagy. In vivo, avermectin B1 effectively inhibited osteosarcoma cell growth and pulmonary metastasis. Mechanistically, avermectin B1 activated the AMPK/ULK1 pathway to exert antitumor activity in vitro and in vivo. Dorsomorphin significantly attenuated the Avermectin B1-induced antitumor activities. Conclusion: Our study suggests that avermectin B1 is a potential agent to treat osteosarcoma cells through the AMPK/ULK1 signaling pathway. [ABSTRACT FROM AUTHOR]

Pancreatic masses are extremely rare in pediatric patients, with limited data available. This lack of data makes the diagnosis and management of these tumors in children extremely challenging. Therefore, we aimed to describe the presentations, clinical course, and outcomes of children with pancreatic tumors at our center. A retrospective analysis was performed of all pediatric patients diagnosed with pancreatic masses between 2003 and 2022 in an academic freestanding children's hospital. Data including demographics, clinical presentation, workup, management, and subsequent morbidity and mortality were collected and aggregated. Furthermore, we reviewed cases of pancreatic tumor resections in the National Surgical Quality Improvement Program – Pediatric (NSQIP-P) database to identify common adverse outcomes and measures for quality improvement. In total, 17 patients were identified at our institution. Diagnoses included solid pseudopapillary (n = 9), gastrinoma (n = 1), rhabdomyosarcoma (n = 2), pancreatoblastoma (n = 2), and insulinoma (n = 1). Two patients did not have a histopathologic diagnosis and were excluded from subsequent analysis. Overall, 12 patients underwent surgical intervention, with the most common procedures being pancreaticoduodenectomy and distal pancreatectomy, and all 12 were known to be alive at last contact. There were 3 deaths, all due to complications related to metastatic disease. Furthermore, 30-day postoperative outcomes in the NSQIP-P dataset for pancreatic surgeries in pediatric patients are excellent, with negligible morbidity and no mortalities after the index surgery. Conclusions: Children with pancreatic tumors amenable to surgical resection appear to have adequate long-term survival. Short-term outcomes at diagnosis are excellent and mainly appear to be influenced by the presence of metastatic disease at initial presentation. What is Known: • Pancreatic masses are a rare entity in children with limited data on their presentation, management and surgical outcomes. • Solid Pseudopapillary tumors are one of the most common pancreatic tumors in children with a fair prognosis after surgical intervention. What is New: • Surgical management of pediatric patients with pancreatic tumors is safe and effective in patients who do not have aggressive tumor types or metastatic disease. • Our case series provides a notable cohort of these pancreatic tumors with insight into the presentation, management and outcomes of five of these tumor types. [ABSTRACT FROM AUTHOR]

Mature ovarian dermoid cysts (ODCs) are the most common benign ovarian tumors diagnosed in children. However, there is minimal data on management of ODCs in premenarchal patients. This study assesses characteristics associated with expectant (EM) vs surgical (SM) management in premenarchal patients and the growth rate of ODCs in EM patients at a single institution. Forty-four premenarchal patients, either post-surgical with pathologically-confirmed ODC or having radiologic findings consistent with ODCs, were included. Data collected included demographics, cyst characteristics, imaging findings, presence of symptoms, surgical procedure performed, and ovarian torsion occurrence. Patient age at diagnosis was similar between groups (SM: 8.8 vs EM: 8.0, P =.55). At presentation, 36 patients (82%) underwent SM and 8 (18%) underwent EM. There was a significant difference in cyst size between groups (SM: 8.9 cm vs EM: 3.6 cm, P =.004). Of SM patients, 30% underwent oophorectomy vs cystectomy, with a significant difference in ODC size between procedures (11.8 cm vs 7.7 cm, P =.016). Of EM patients, 75% had at least one and 60% had three follow-up ultrasounds, with average follow-up timeframes of 3.7 and 27 months respectively. Average yearly ODC growth rate for the latter group was 0.8 cm. The average yearly growth rate of ODCs in premenarchal patients within our institution was slower than in older cohorts, and both age and cyst size played significant roles in determining surgical procedure. Continued study on EM in premenarchal ODCs will help define parameters for recommending SM vs EM in this population. [ABSTRACT FROM AUTHOR]

Purpose We aimed to document the acceptability (enrollment rate) and feasibility (phone call delivery rate) of implementing a behavioral PA intervention over 12 weeks, in addition to documenting its effects on patient-reported outcomes and physical functioning. This study also describes the costs of carrying out a behavioral PA intervention. A total of 40 participants were randomized in a 1:1 ratio. The tailored behavioral PA intervention was developed based on the most recent PA guidelines in pediatric oncology and on the COM-B framework to enact PA behavior changes. The prescription (frequency, intensity, time and type (FITT)) was adjusted each week during the weekly support calls. The control group did not receive the intervention. 26 males and 14 females (13.6 years old on average and 2.9 years post-cancer treatment on average) participated in our study. The acceptability rate was 90.9% and the feasibility rate was > 85%. We found that 85% improved PA frequency, 80% improved PA intensity, 100% improved PA time, and 50.0% achieved the recommended PA guidelines. No adverse events were reported over the duration of the intervention. Physical function improved with longer 6-minute walk distances in the intervention group (465.8 ± 74.5 m) than in the control group (398.7 ± 92.9 m) (p = 0.016). PROs scores for all participants were within the limits of the normal range. The estimated cost per participant of carrying out this intervention was USD $126.57. Our 12-week behavioral PA intervention, based on the COM-B framework, was found to be acceptable, feasible and safe in childhood cancer survivors. This study is an important step in the right direction to make exercise standard practice in pediatric oncology. Clinical trial registration: NCT04947709. [ABSTRACT FROM AUTHOR]

Background: Neuroblastoma (NB) is one of the most frequent parenchymal tumors among children, with a high degree of heterogeneity and wide variation in clinical presentation. Despite significant therapeutic advances in recent years, long-term survival in high-risk patients remains low, emphasizing the urgent need to find new biomarkers and construct reliable prognostic models. Methods: In this study, data from neuroblastoma samples in the ArrayExpress database were utilized to identify key gene modules and pivotal genes associated with NB prognosis by weighted gene co-expression network analysis (WGCNA). The Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) functional enrichment analysis was performed using the DAVID database. Based on these hub genes, survival prognosis models were constructed and validated on an independent validation set in the Gene Expression Omnibus (GEO) database. Differences in biological functions and immune microenvironments and the sensitivity to pharmacological and immunotherapeutic treatments of patients in the high- and low-risk groups were examined by gene set enrichment analysis (GSEA) and immune infiltration analysis. Results: WGCNA identified 14 gene modules and screened the module with the highest relevance to the International Neuroblastoma Staging System (INSS), containing 60 pivotal genes. GO and KEGG analyses demonstrated that these pivotal genes were mainly implicated in biological processes and signaling pathways including DNA replication, cell division, mitotic cell cycle, and cell cycle. Based on Lasso regression and COX regression analysis, a prognostic model containing DHFR, GMPS and E2F3 was constructed, and the RiskScore was significantly correlated with the 1-, 3- and 5-year survival of the patients. GSEA and immune infiltration analyses revealed significant differences in the levels of cell cycle-related pathways and immune cell infiltration between the high and low RiskScore groups. In particular, patients in the high-risk group are less likely to benefit from immunotherapy and may be better suited for treatment with drugs such as Oxaliplatin and Alpelisib. Conclusion: This research systematically identified biomarkers related to NB prognosis and developed a reliable prognostic model applying WGCNA and multiple bioinformatics methods. The model has important application value in predicting patients' prognosis, evaluating drug sensitivity and immunotherapy effect, and provides new ideas and directions for precise treatment of neuroblastoma. [ABSTRACT FROM AUTHOR]

Simple Summary: Patient activation is a very important psychological construct to examine in individuals who have chronic conditions, because it assesses the one's confidence in managing their own health and care. For childhood cancer survivors, continued follow-up is imperative to monitor late effects conditions; yet many do not adhere to surveillance guidelines. Therefore, investigating this construct could highlight risk factors in the population that contribute to low activation. Furthermore, examining the long-term impact of patient activation on psychological health as well as its contribution to health behavior could provide a reasonable target for interventions to enhance health outcomes in survivors. Background: Patient activation describes a willingness to take action to manage health and is associated with health outcomes. The purpose of this study was to characterize patient activation and its association with psychological outcomes and health behaviors in childhood cancer survivors. Methods: Participants were from the St. Jude Lifetime Cohort Study (SJLIFE). Activation levels (1–4, 4 = highest activation) were measured with the Patient Activation Measure (PAM). Psychological outcomes and health behaviors were obtained via self-report. Cognitive function was assessed by trained examiners. ANOVA or chi-squared tests were utilized to assess group-level differences in activation. Multivariable regression models were used to assess associations between PAM scores and outcomes of interest. Results: Among 2708 survivors and 303 controls, more survivors endorsed lower activation levels than the controls (11.3 vs. 4.7% in level 1) and fewer survivors endorsed the highest level of activation than the controls (45.3 vs. 61.5% in level 4). Not endorsing depression (OR: 2.37, 95% CI 1.87–2.99), anxiety (OR: 2.21, 95% CI 1.73–2.83), and somatization symptoms (OR: 1.99, 95% CI 1.59–2.50), general fear (OR: 1.45, 95% CI 1.23–1.71) and body-focused (OR: 2.21, 95% CI 1.83–2.66), cancer-related worry, and physical (OR: 2.57, 95% CI 2.06–3.20) and mental (OR: 2.08, 95% CI 1.72–2.52) HRQOL was associated with higher levels of activation. Lower activation was associated with not meeting physical activity guidelines (OR: 2.07, 95% CI 1.53–2.80). Conclusions: Survivors endorsed lower activation levels than peers. Interventions to improve physical and psychological health outcomes could leverage these results to identify survivors who benefit from support in patient activation. [ABSTRACT FROM AUTHOR]

Simple Summary: Acupuncture is becoming a popular complementary treatment in pediatric cancer care for managing symptoms like nausea and vomiting that result from cancer treatments. This review aims to evaluate how effective and safe acupuncture and similar techniques are for children with cancer. By analyzing data from multiple studies, the authors found that acupuncture may help reduce nausea and vomiting in young cancer patients. However, there is not enough evidence to make conclusions about its effects on number of vomiting episodes, need for anti-nausea medication, or fatigue. More high-quality research is needed to confirm these benefits and ensure the safety of acupuncture for children undergoing cancer treatment. Background/Objectives: Oncology acupuncture is emerging as a complementary treatment in pediatric cancer care centers. It is valued for its potential to manage symptoms associated with cancer and treatment toxicities without increasing polypharmacy. The aim of this review is to evaluate the efficacy and safety of acupuncture and related techniques in alleviating symptoms of cancer treatment in pediatric oncology patients. Methods: A comprehensive search was conducted across nine databases, including PubMed, Cochrane Library, and CNKI, up to June 2023. Inclusion criteria focused on randomized and quasi-randomized controlled trials involving pediatric oncology patients undergoing needle acupuncture or related techniques. Study selection and data extraction were independently performed by pairs of authors. Results were analyzed narratively, and meta-analysis was performed when possible. Results: Results suggest that acupuncture may help manage symptoms such as nausea and vomiting in pediatric oncology patients. However, the quality of evidence was generally low, and further research is required to substantiate these findings. Conclusions: Acupuncture shows promising results as a complementary treatment for reducing nausea and vomiting in pediatric oncology. However, current evidence is insufficient to draw conclusions for other outcomes, such as the number of vomiting episodes, reduction in antiemetic medication use, or fatigue. High-quality, rigorously designed studies are necessary to better understand the clinical relevance and safety of acupuncture in this vulnerable population. [ABSTRACT FROM AUTHOR]

Simple Summary: The incidence and mortality rates of cancer in the Surveillance, Epidemiology, and End Results (SEER) Program for adolescent and young adult (AYA) patients show distinct patterns among early-onset cancers. For some cancers, AYA cancer rates varied by age group, sex, race, ethnicity and geography. Monitoring the rates and time trends of AYA cancer emphasizes the distinct health concern for this age group. (1) Background: The incidence rate of early onset-cancer (<50) has increased since 1995. Among younger people, cancers in AYAs (aged 15–39 y) are often biologically distinct tumors from those treated in the pediatric and older adult population. The current study describes trends in the United States for the most recent years including the first year of the COVID-19 epidemic. We aimed to describe the recent incidence and mortality trends of cancers in AYAs (aged 15–39 y). (2) Methods: We used data from the Surveillance, Epidemiology, and End Results (SEER 22) from 1 January 2016 to 31 December 2021. Age-adjusted incidence and mortality rates were assessed by SEER*Stat 8.4.3 for major cancer types by sex, race/ethnicity, age, and metropolitan/nonmetropolitan status. Time trends of age-adjusted incidence and mortality rates were examined by sex and metropolitan/nonmetropolitan status. (3) Results: Age-adjusted overall cancer incidence and mortality rates were stable during this study period. The age-adjusted incidence rates declined significantly for ependymoma, melanoma, carcinomas of lung, bronchus, and trachea, unspecified malignant neoplasms, and non-Hodgkin's lymphoma. Significant increases were found for gastrointestinal tract cancers and non-Kaposi sarcomas. The age-adjusted mortality rate decreased for acute myeloid leukemia, melanoma, carcinomas of liver and intrahepatic bile ducts, kidney and, in women, leukemia. For some cancers, rates differed by sex, race, ethnicity, and geography. Monitoring the rates and time trends of AYA cancer emphasizes the distinct health concern for this age group. [ABSTRACT FROM AUTHOR]

The article presents a study on oral health assessment of newly diagnosed oncology patients by dental practitioners. Topics discussed include the aims of the study, the materials and methods used in the study, and the need for improvements in communication with oncology wards as shown in the percentage of patients who received dental referrals.

Aim: Childhood cancer survival has improved significantly with advances in multimodal treatment. However, treatment-related long-term side effects, including dental developmental abnormalities, remain a concern. The aim of this study was to investigate permanent tooth development and associated risk factors in childhood cancer survivors who were treated before the age of six years, focusing on non-leukemia cases. Materials and Methods: This study was conducted on childhood cancer cases treated with chemotherapy and/or radiotherapy at a single center. Patients diagnosed before the age of six and who were twelve or above at the time of assessment were included. Dental examinations, including Decayed, Missing, Filled Teeth index and radiographic assessments, were performed. Statistical analyses were performed in order to identify associations between treatment modalities and dental findings. Results: Of 914 patients, 90 met the inclusion criteria, with 35 participating in this study. The mean age at diagnosis was four years, with a mean follow-up duration of ten years. Root shortening, hypoplasia, and hypodontia were observed to be common dental anomalies. There was a significant association between alkylating agents, vinca alkaloid plus alkylating agents and root shortening (p<0.001, p<0.001). No significant differences in dental findings were found based on gender, tumor site, or other treatment modalities (p<0.05). As the age at diagnosis increased, the risk of root shortening (p=0.026) and the frequency of hypodontia significantly increased with the duration after diagnosis (p=0.048). Conclusion: Childhood cancer survivors treated during early dentition periods are at risk of dental developmental anomalies, with alkylating agents showing the most significant impact. This emphasizes the necessity of providing these patients and their families with information on the importance of maintaining oral and dental health during and after cancer treatment. [ABSTRACT FROM AUTHOR]

Objective: The study investigated maternal exposure to heavy metals from industrial sources during pregnancy as potential risk factors for childhood cancer. Methods: Cases ages 0-19 were identified from California Cancer Registry. Controls (20:1 ratio) were randomly selected from California Birth Registry, frequency-matched by birth year (1998-2016). We estimated maternal exposure to lead, nickel, and cobalt in ambient air from the Toxics Release Inventory. We examined "ever/never" and "high/low" exposures categorized by median exposure. Models were adjusted for maternal age, race/ethnicity, method of payment for prenatal care, neighborhood socioeconomic status, and urban/rural residence. Results: Among highly exposed persons, lead was associated with an increased teratoma risk (adjusted odds ratio [aOR]: 1.52; 95% confidence interval [CI]: 0.97, 2.37), whereas nickel was associated with an increased rhabdomyosarcoma risk (aOR: 1.45; 95% CI: 1.03, 2.04). Cobaltwas associated with an increased glioma risk (aOR: 2.25; 95% CI: 1.39, 3.65) among ever-exposed persons. Inverse associations were found between Wilms tumor and nickel among the ever exposed and highly exposed (ever: aOR: 0.75; 95% CI: 0.59, 0.96; high: aOR: 0.64; 95% CI: 0.45, 0.93). Conclusions: Findings suggest that air pollution from heavy metals released by industrial sources may elevate childhood cancer risk. [ABSTRACT FROM AUTHOR]

Simple Summary: Survivors of childhood cancer are predisposed to a range of late effects of treatment, including high symptom burden. Symptom burden may vary over time. The aim of this prospective pilot study was to assess the feasibility of collecting daily symptoms from adult survivors of childhood cancer using mobile health (mHealth) technology to evaluate symptom fluctuation and associations with future health-related quality of life (HRQOL). The 41 survivors included in this study had high adherence to symptom reporting (83%) and HRQOL reporting (95%). Variability of daily symptom burden differed from person-to-person (74%), day-to-day (18%), and month-to-month (8%). Additionally, a higher symptom burden was associated with poorer HRQOL in the future. Daily assessment of symptoms using mHealth technology revealed fluctuations in symptomology and the association between symptom burden and HRQOL. This method of symptom assessment is valuable for improving our understanding of symptom dynamics and sources of variability. Background: Cancer therapies predispose survivors to a high symptom burden. This study utilized mobile health (mHealth) technology to assess the feasibility of collecting daily symptoms from adult survivors of childhood cancer to evaluate symptom fluctuation and associations with future health-related quality-of-life (HRQOL). Methods: This prospective study used an mHealth platform to distribute a 20-item cancer-related symptom survey (5 consecutive days each month) and an HRQOL survey (the day after the symptom survey) over 3 consecutive months to participants from the Childhood Cancer Survivor Study. These surveys comprised a PROMIS-29 Profile and Neuro-QOL assessed HRQOL. Daily symptom burden was calculated by summing the severity (mild, moderate, or severe) of 20 symptoms. Univariate linear mixed-effects models were used to analyze total, person-to-person, day-to-day, and month-to-month variability for the burden of 20 individual symptoms. Multivariable linear regression was used to analyze the association between daily symptom burden in the first month and HRQOL in the third month, adjusted for covariates. Results: Out of the 60 survivors invited, 41 participated in this study (68% enrollment rate); 83% reported their symptoms ≥3 times and 95% reported HRQOL in each study week across 3 months. Variability of daily symptom burden differed from person-to-person (74%), day-to-day (18%), and month-to-month (8%). Higher first-month symptom burden was associated with poorer HRQOL related to anxiety (regression coefficient: 6.56; 95% CI: 4.10–9.02), depression (6.32; 95% CI: 3.18–9.47), fatigue (7.93; 95% CI: 5.11–10.80), sleep (6.07; 95% CI: 3.43–8.70), pain (5.16; 95% CI: 2.11–8.22), and cognitive function (–6.89; 95% CI: –10.00 to –3.79) in the third month. Conclusions: Daily assessment revealed fluctuations in symptomology, and higher symptom burden was associated with poorer HRQOL in the future. Utilizing mHealth technology for daily symptom assessment improves our understanding of symptom dynamics and sources of variability. [ABSTRACT FROM AUTHOR]

Simple Summary: Genomic rearrangements are chromosomal abnormalities that alter the arrangement of genes and are important for diagnosing multiple cancer types and guiding therapy selection. However, current diagnostic tests may not detect all genomic rearrangements. Hi-C sequencing is a promising new method for detecting genomic rearrangements. First, we examined whether Hi-C can detect known genomic rearrangements in pediatric leukemia and sarcoma specimens. Next, we evaluated whether Hi-C can detect genomic rearrangements that were not found using standard diagnostic testing. Hi-C showed complete agreement with other diagnostic methods in identifying known rearrangements. Hi-C also detected previously unknown genomic rearrangements in 5/11 pediatric leukemia cases that could impact diagnosis, prognosis, or treatment choices. These data suggest Hi-C could be beneficial for medical diagnostic testing of pediatric cancers, but more extensive clinical validation is needed. Hi-C sequencing is a DNA-based next-generation sequencing method that preserves the 3D genome conformation and has shown promise in detecting genomic rearrangements in translational research studies. To evaluate Hi-C as a potential clinical diagnostic platform, analytical concordance with routine laboratory testing was assessed using primary pediatric leukemia and sarcoma specimens. Archived viable and non-viable frozen leukemic cells and formalin-fixed paraffin-embedded (FFPE) tumor specimens were analyzed. Pediatric acute myeloid leukemia (AML) and alveolar rhabdomyosarcoma (A-RMS) specimens with known genomic rearrangements were subjected to Hi-C to assess analytical concordance. Subsequently, a discovery cohort consisting of AML and acute lymphoblastic leukemia (ALL) cases without known genomic rearrangements based on prior clinical diagnostic testing was evaluated to determine whether Hi-C could detect rearrangements. Using a standard sequencing depth of 50 million raw read-pairs per sample, or approximately 5X raw genomic coverage, we observed 100% concordance between Hi-C and previous clinical cytogenetic and molecular testing. In the discovery cohort, a clinically relevant gene fusion was detected in 45% of leukemia cases (5/11). This study provides an institutional proof of principle evaluation of Hi-C sequencing to medical diagnostic testing as it identified several clinically relevant rearrangements, including those that were missed by current clinical testing workflows. [ABSTRACT FROM AUTHOR]

Primary central nervous system tumors are the most frequent solid tumors in children, accounting for over 40% of all childhood brain tumor deaths, specifically high-grade gliomas. Compared with pediatric low-grade gliomas (pLGGs), pediatric high-grade gliomas (pHGGs) have an abysmal survival rate. The WHO CNS classification identifies four subtypes of pHGGs, including Grade 4 Diffuse midline glioma H3K27-altered, Grade 4 Diffuse hemispheric gliomas H3-G34-mutant, Grade 4 pediatric-type high-grade glioma H3-wildtype and IDH-wildtype, and infant-type hemispheric gliomas. In recent years, we have seen promising advancements in treatment strategies for pediatric high-grade gliomas, including immunotherapy, CAR-T cell therapy, and vaccine approaches, which are currently undergoing clinical trials. These therapies are underscored by the integration of molecular features that further stratify HGG subtypes. Herein, we will discuss the molecular features of pediatric high-grade gliomas and the evolving landscape for treating these challenging tumors. [ABSTRACT FROM AUTHOR]

Objective: Differentiated thyroid cancer (DTC) is the most common pediatric endocrine cancer but studies are scarce. Latest recommendations advocate for an individualized risk-based approach to select patients for additional therapy. Lymphovascular invasion is not considered, despite being a well-known risk factor in the adult population. The aim of this study was to describe the outcomes of a cohort of DTC patients diagnosed at pediatric age and to evaluate the impact of lymphovascular invasion on the risk of persistence/ recurrence. Methods: A retrospective study of patients diagnosed with DTC at pediatric age from 2010 to 2022 at a single center was performed. All patients had total thyroidectomy. Radioactive iodine therapy (RAI) was used in selected patients. The response to therapy and occurrence of persistent/recurrent disease were evaluated. Results: A total of 21 DTC were diagnosed, mostly papillary thyroid carcinoma (PTC) (81.0%, n=17). Six patients (28.6%) had nodal involvement and one (4.8%) had lung metastasis at the time of the diagnosis. Lymphovascular invasion was present in 11 patients (52.4%). After surgery, 13 patients (61.9%) underwent RAI. The mean follow-up time was 5.7±3.1 years. In total, 6 patients (31.6%) experienced persistent/recurrent disease during the follow-up time. Among PTC patients, persistent/recurrent disease was more frequent in the presence of lymphovascular invasion [55.6% (5/9) vs. 0.0% (0/6), p=0.031]. Conclusion: An individualized risk-based approach is recommended. Our study suggests that lymphovascular invasion may be associated with a higher risk of persistence/recurrence and should therefore be considered for decision making in children and adolescents with PTC. [ABSTRACT FROM AUTHOR]

Objective: The survival rates of children with acute lymphoblastic leukemia (ALL) have improved over the years, but infections remain a significant cause of morbidity and mortality. Chemotherapy has a range of harmful side effects including the loss of protective antibodies against vaccine-preventable diseases. The objective of this study was to evaluate the serological status of pediatric ALL cases before and after intensive chemotherapy. Materials and Methods: Children treated and followed for ALL at Dokuz Eylül University were included in this retrospective crosssectional study. Antibody levels against hepatitis A, hepatitis B, and rubella were routinely assessed at both the time of diagnosis and 6 months after completion of chemotherapy. Measles, mumps, and varicella antibody levels were evaluated at only 6 months after treatment. Results: Seventy-eight children who completed chemotherapy for ALL were enrolled in the study. All participants had non-protective antibody levels for at least one of the diseases. The highest seropositivity rate was found for hepatitis A (55.1%) and the lowest for measles (17.9%) after chemotherapy. Overall, 50.7%, 30.6%, and 45.7% of the patients significantly lost their humoral immunity against hepatitis B, hepatitis A, and rubella, respectively. Patients in the higher-risk group for ALL had lower seropositivity rates than patients of the other risk groups. There were statistically significant relationships between the protective antibody rates for hepatitis A and varicella and the ages of the patients. Except for hepatitis A vaccination, pre-chemotherapy vaccination did not affect post-chemotherapy serology. On the other hand, all children with a history of varicella before diagnosis showed immunity after chemotherapy. Conclusion: Patients with ALL, including those previously fully vaccinated, are at great risk of infection due to the decrease in protective antibody levels after chemotherapy. There is a need for routine post-chemotherapy serological testing and re-vaccination based on the results obtained. [ABSTRACT FROM AUTHOR]

Background: Nutri Crackle rusk, developed using an innovative pigmented variety of black rice flour, possesses a rich nutritional profile with essential amino acids, dietary fibers, and antioxidants, offering unique health benefits beyond traditional rice varieties. Establishing healthy eating habits during childhood cancer treatment is crucial for long-term well-being. Mindful consumption, incorporating Nutri Crackle rusk instead of empty-calorie snacks, can significantly improve the quality of life of childhood cancer survivors during their survivorship period. Materials and Methods: The novel Nutri Crackle rusk using black rice flour was developed under proper hygienic conditions. Semi-trained panel and consumer panel members evaluated the organoleptic properties of the developed product. Nutrient components of the formulated Nutri Crackle rusk were examined through an organoleptic assessment. Results: The nutrient composition of the formulated Nutri Crackle rusk was analyzed using a standard procedure. Sensory evaluation indicated positive feedback, with an overall acceptability score of 8.01. Nutrient analysis revealed a well-rounded profile, emphasizing energy provision (464.285 kcal), (11.97 g protein), (11.29 g fiber), and essential micronutrients (zinc, iron, and vitamins B1 and B2). Nutri Crackle rusk is a favorable and potentially beneficial snack for this vulnerable group. Conclusion: The study signifies that the novel healthy Nutri Crackle rusk is rich in fiber, zinc, iron, protein, vitamin B1, and vitamin B2. Intake of the developed Nutri Crackle rusk could be a healthy alternative for pediatric cancer patients and cancer survivors. [ABSTRACT FROM AUTHOR]

Purpose: Various surgical nuances of the telovelar approach have been suggested. The necessity of removing the posterior arch of C1 to accomplish optimal tumor exposure is still debated. Therefore, we report on our experience and technical details of the fourth ventricular tumor resection in a modified prone position without systematic removal of the posterior arch of C1. Methods: A retrospective analysis of all pediatric patients, who underwent a fourth ventricular tumor resection in the modified prone position between 2012 and 2021, was performed. Results: We identified 40 patients with a median age of 6 years and a M:F ratio of 25:15. A telovelar approach was performed in all cases. In 39/40 patients, the posterior arch of C1 was not removed. In the remaining patient, the reason for removing C1 was tumor extension below the level of C2 with ventral extension. Gross or near total resection could be achieved in 34/39 patients, and subtotal resection in 5/39 patients. In none of the patients, a limited exposure, sight of view, or range of motion caused by the posterior arch of C1 was encountered, necessitating an unplanned removal of the posterior arch of C1. Importantly, in none of the cases, the surgeon had the impression of a limited sight of view to the most rostral parts of the fourth ventricle, which necessitated a vermian incision. Conclusion: A telovelar approach without the removal of the posterior arch of C1 allows for an optimal exposure of the fourth ventricle provided that critical nuances in patient positioning are considered. [ABSTRACT FROM AUTHOR]

The purpose of this study was to investigate the risk factors for delayed chemotherapy–induced vomiting (DCIV) in pediatric oncology patients. We collected data on pediatric patients from a tertiary care pediatric hospital in an Asian urban center. We analyzed the risk factors for DCIV in patients by univariate analysis and logistic regression. Patients were grouped according to age by the Youden index, and differences in clinical features between the high-risk and low-risk groups were calculated. In the univariate analysis, the number of chemotherapy days, pH, and blood glucose levels were significantly associated with DCIV. In the logistic regression analysis, patient age was an independent risk factor (odds ratio [OR] = 1.013, 95% confidence interval [CI] = 1.005-1.021, P =.002). Children in the high-risk group had a higher grade of vomiting (P <.05). Age is an important risk factor for DCIV in pediatric patients, with older children tending to experience more frequent and more severe vomiting. [ABSTRACT FROM AUTHOR]

Background: Timely and relevant information helps parents to cope when a child is diagnosed with cancer. However, obtaining and understanding information is not a straightforward process for parents. Objectives: This article aims to explain paediatric cancer parents' information behaviour related to the care of their child. Methods: Qualitative in‐depth interviews were conducted with fourteen Malaysian paediatric cancer parents and eight healthcare professionals who worked with paediatric cancer patients. Reflexivity and inductive approaches were used to interpret the data to identify meaningful themes and subthemes. Results: Three themes about how paediatric cancer parents interact with information emerged: Acquiring information, internalising information, and using information. Information may be actively sought or passively acquired. Cognitive and affective aspects influence how information is internalised into meaningful knowledge. Knowledge then leads to further action including further information gathering. Conclusion: Paediatric cancer parents need health literacy support to meet their information needs. They require guidance in identifying and appraising suitable information resources. Development of suitable supporting materials is needed to facilitate parents' ability to comprehend information related to their child's cancer. Understanding parents' information behaviour could assist healthcare professionals in providing information support in the context of paediatric cancer. [ABSTRACT FROM AUTHOR]

Anatomical models have key applications in radiotherapy, notably to help understand the relationship between radiation dose and risk of developing side effects. This review analyses whether age-specific computational phantoms, developed from healthy subjects and paediatric cancer patient data, are adequate to model a paediatric population. The phantoms used in the study were International Commission on Radiological Protection (ICRP), 4D extended cardiac torso (XCAT) and Radiotherapy Paediatric Atlas (RT-PAL), which were also compared to literature data. Organ volume data for 19 organs was collected for all phantoms and literature. ICRP was treated as the reference for comparison, and percentage difference (P.D) for the other phantoms were calculated relative to ICRP. Overall comparisons were made for each age category (1, 5, 10, 15) and each organ. Statistical analysis was performed using Microsoft Excel (version 16.59). The smallest P.D to ICRP was for Literature (-17.4%), closely followed by XCAT (26.6%). The largest was for RT-PAL (88.1%). The rectum had the largest average P.D (1,049.2%) and the large bowel had the smallest (2.0%). The P.D was 122.6% at age 1 but this decreased to 43.5% by age 15. Linear regression analysis showed a correlation between organ volume and age to be the strongest for ICRP (R2 = 0.943) and weakest for XCAT (R2 = 0.676). The phantoms are similar enough to ICRP for potential use in modelling paediatric populations. ICRP and XCAT could be used to model a healthy population, whereas RT-PAL could be used for a population undergoing/after radiotherapy. [ABSTRACT FROM AUTHOR]

Statement of the Problem: Chemotherapy-induced mucositis is the most common complication during cancer treatment. This complication can lead to pain, increased risk of infection and malnutrition. Therefore, it is important to find a solution to reduce the severity and duration of side effects. Purpose: This study aimed to assess the efficacy of Mucosamin spray as an adjunct for prevention of oral mucositis in children under chemotherapy. Materials and Method: This parallel-design clinical trial evaluated 48 patients aged 5 to 15 years with leukemia and lymphoma presenting to the Hematology Department of Mofid and Mahak Hospitals. The patients were randomly divided into two groups (n=24). Before starting chemotherapy, all patients received oral hygiene instructions (toothbrushing without flossing). Patients in both groups were requested to use a mouthwash composed of nystatin, aluminum-magnesium hydroxide suspension (aluminum MgS), and diphenhydramine for 1 min every morning on a daily basis starting the day before treatment. Patients in the intervention group were also requested to spray their entire oral mucosa with Mucosamin spray 3 times a day in addition to using the mouthwash. Patients were requested to refrain from eating and drinking for 1h after spraying. The patients were clinically examined by a senior dental student once every other day for 20 days regarding the occurrence, severity, and duration of oral mucositis. Data were analyzed using the Chi-square and Mann-Whitney tests, Kaplan-Meier survival analysis, and log rank test. Results: No significant difference was noted between the two groups in the incidence, severity, or time of development of mucositis (p> 0.05). The 7-day non-recovery percentage was 72.7% (SE= 13.4) and 25.0% (SE= 15.3) in the control and test groups, respectively, indicating shorter duration (faster healing) of mucositis in the intervention group (p= 0.01). Conclusion: Within the limitations of this study, it seems that prophylactic application of Mucosamin spray can shorten the course of oral mucositis, in case of its occurrence. [ABSTRACT FROM AUTHOR]

(1) Context: Cancer triggers significant changes in family dynamics. It is noteworthy that coping and problem-solving skills, particularly in situations involving cancer in children and adolescents, have not been adequately explored in the context of family adaptation. This study aims to analyze the effectiveness of family interventions in coping to improve problem-solving skills in parents and/or caregivers of children and adolescents during and after oncological treatment. (2) Methods: This is a scoping review following the recommendations of the Joanna Briggs Institute and the Systematic Reviews and Meta-Analyses Extension for Scoping Review (PRISMA-ScR), from 2014 to 2024, in the databases LILACS, CINAHL, SCOPUS, Web of Science, and PUBMED. (3) Results: Forty-five studies were eligible. Coping strategies were categorized as follows: (1) positive attitudes (including a sense of courage and hope, family support to enhance resilience, and future planning), (2) caregiver empowerment (involving acceptance of diagnosis, emotional distancing, and coping through religiosity), and (3) communication skills (encompassing professional communication, horizontal dialogue with healthcare teams, and sincere communication with friends and family). (4) Conclusions: Over time, families develop coping and problem-solving strategies that influence changes in family functioning patterns, aiding them in accepting, reinterpreting, and reframing ideas and feelings associated with neoplasia. [ABSTRACT FROM AUTHOR]

Thromboembolism (TE) complicates the course of pediatric cancer in a considerable number of cases. Cancer-related TE is attributed to an interaction of the underlying malignancy, the effects of therapy, and a possible thrombophilia predisposition. More specifically, recognized risk factors include a very young age and adolescence, non-O blood group, type and site of cancer, inherited thrombophilia, presence of central venous catheter, and type of chemotherapy. TE in children with cancer most commonly occurs in their extremities. In the absence of evidence-based guidelines for the management of thrombotic complications in pediatric oncology patients, TE management follows general recommendations for the management of pediatric TEs. Given the limitations of conventional anticoagulant therapy, direct oral anticoagulants could provide an alternative; however, their safety and efficacy in children with cancer remain to be seen. As for thromboprophylaxis, numerous studies have been conducted, albeit with conflicting results. Although the survival of pediatric oncology patients has significantly improved in recent years, morbidity due to cancer-related TE remains, underlying the need for large multicenter trials investigating both TE management with currently available agents and primary prevention. [ABSTRACT FROM AUTHOR]

Gastrointestinal stromal tumors (GISTs) are rare mesenchymal neoplasms that primarily affect adults, with pediatric cases constituting only 0.5–2.7% of the total. Pediatric GISTs present unique clinical, genetic, and pathological features that distinguish them from adult cases. This literature review aims to elucidate these differences, emphasizing diagnostic and therapeutic challenges. We discuss the resistance of pediatric GISTs to conventional chemotherapy and highlight the importance of surgical intervention, especially in emergency situations involving intra-abdominal bleeding. The review also explores the molecular characteristics of pediatric GISTs, including rare mutations such as quadruple-negative wild-type GIST with an FGF3 gene gain mutation. To illustrate these points, we conclude with a case from our clinic involving a 15-year-old female with multiple CD117-positive gastric GISTs and a quadruple-negative wild-type genetic profile who required urgent surgical intervention following a failed tumor embolization. This case underscores the critical need for early diagnosis and individualized therapeutic strategies combining oncologic and surgical care to improve outcomes in pediatric GIST patients. [ABSTRACT FROM AUTHOR]

Mandibular brown tumors are often radiographically and histologically indistinguishable from cherubism, aneurysmal bone cyst, giant cell granuloma, and odontogenic fibroma. Laboratory findings of hyperparathyroidism (HPT) aid in directing therapy. We present a case of a 14-year-old male with a brown tumor of the mandible associated with secondary HPT due to vitamin D deficiency. Core biopsy exhibited a proliferation of plump spindle cells and osteoclast-like giant cells within variably fibrous stroma. Parathyroid hormone (PTH) levels were elevated to 288 pg/mL (normal 8.5-77.1 pg/mL) and vitamin D was low at a level of 6.9 ng/mL (normal > 20.0 ng/mL). Six months after the introduction of weekly vitamin D supplementation, PTH and vitamin D levels improved but remained abnormal with PTH of 86.8 pg/mL and vitamin D of 9.3 ng/mL. Alongside an improvement in laboratory indices, the tumor character stabilized and became less clinically friable; however, the burden remained unchanged. At 9 months, the patient underwent surgical debulking of the tumor with local soft tissue coverage. A review of pediatric literature was performed, among which 16 patients were identified. Most children who developed brown tumors were found to have secondary HPT and ultimately 44% underwent some degree of surgical debridement of the mass. [ABSTRACT FROM AUTHOR]

Background: With the widespread use of computed tomography (CT), the detection rate of pulmonary nodules in children has gradually increased. Due to the lack of epidemiological evidence and clinical guideline on pulmonary nodule treatment in children, we aimed to provide a reference for the clinical diagnosis and management of pediatirc pulmonary nodules. Methods: This retrospective study collected consecutive cases from April 2012 to July 2021 in the Shanghai Children's Medical Center. The sample included children with pulmonary nodules on chest CT scans and met the inclusion criteria. All patients were categorized into tumor and non-tumor groups by pre-CT clinical diagnosis. Nodule characteristics between groups were analyzed. To establish a clinical assessment model for the benign versus malignant pulmonary nodules, patients who have been followed-up for three months were detected and a decision tree model for nodule malignancy prediction was constructed and validated. Results: The sample comprised 1341 patients with an average age of 7.2 ± 4.6 years. More than half of them (51.7%) were diagnosed with malignancies before CT scan. 48.3% were diagnosed with non-tumor diseases or healthy. Compared to nontumor group, children with tumor weremore likely to havemultiple nodules in both lungs, with larger size and often be accompanied by osteolytic or mass lesions. Based on the decision tree model, patients' history of malignancies, nodules dia meter size=5mm, and specific nodule distribution (multiple in both lungs, multiple in the right lung or solitary in the upper or middle right lobe) were important potential predictors for malignity. In the validation set, sensitivity, specificity and AUC were 0.855, 0.833 and 0.828 (95%CI: 0.712-0.909), respectively. Conclusion: This study conducted a clinical assessment model to differentiate benignity and malignancy of pediatric pulmonary nodules. We suggested that a nodule's diameter, distribution and patient's history of malignancies are predictable factors in benign or malignant determination. [ABSTRACT FROM AUTHOR]

Backgroud: The study objective was to compare three-dimensional and two-dimensional imaging using computer-assisted systems (CASs) in clinical guidance for preoperative surgical planning for middle hepatic tumors in children. Methods: A retrospective analysis was performed on 23 children who underwent surgery for middle hepatic tumors in our hospital from January 2016 to June 2022. The surgical resection plan was formulated by the operator team using two-dimensional CT images before the operation. Then, the same qualified surgeons conducted an in-depth analysis and formulated the surgical resection scheme for the same pediatric patient using three-dimensional imaging of the middle hepatic tumor. The feasibility of the two schemes was compared and analyzed. Result: All the tumors were successfully removed according to the preoperative method developed using three-dimensional imaging. The postoperative short-term follow-up revealed that all patients were doing well. Preoperative plans were revised in 9 cases after evaluating the three-dimensional images due to the disparity between the original plans and the three-dimensional relationship between the tumor and blood vessels, vascular variation, and the volume of remnant liver. Conclusions: Three-dimensional imaging with a computer-assisted surgery system is superior to two-dimensional imaging in the preoperative planning of pediatric hepatoblastoma. [ABSTRACT FROM AUTHOR]

Background: Ewing sarcoma (ES) is the second most common primary malignant bone tumor in children and adolescents. Despite more intensive chemotherapy regimens and improved local control therapy, there is still a considerable rate of recurrent/progressive disease. Methods: A retrospective study of 50 relapsed/progressive ES patients who were treated at the National Cancer Institute (NCI), Cairo University, during the period from 1st of January 2008 to the end of December 2018, to assess different prognostic variables and disease outcomes. Results: Out of fifty eligible cases, 32 patients (64%) had disease recurrence, and 18 (36%) developed disease progression on treatment. The median follow-up period was 7.4 months. The median overall survival (OS) was 7.5 months, and the cumulative OS was 64% at 6 months and 32.6% at 1 year. The cumulative event-free survival (EFS) was 41.3% at 6 months and 22.3% at 1 year. Patients with disease recurrence had better OS and EFS than patients with disease progression (p = 0.019). Patients who underwent local control at relapse/progression had a significantly better outcome than patients who received chemotherapy only (p < 0.001). Recurrence > 2 years from initial diagnosis was the only independent predictor of better survival outcome. Conclusions: Patients with relapsing/progressive ES portended a poor outcome, with disease progression on treatment faring worse than relapse. Better outcome was observed in patients who experienced recurrence > 2 years after diagnosis, patients with disease recurrence rather than disease progression on treatment, and patients who underwent local control along with intensive chemotherapy. [ABSTRACT FROM AUTHOR]

Background: Parameningeal rhabdomyosarcoma (PM-RMS) is a rare and aggressive soft tissue malignancy that primarily occurs in the head and neck region. The standard treatment approach for RMS involves a multimodal therapy regimen, which includes surgery, chemotherapy, and radiotherapy. However, the routine use of radiotherapy and chemotherapy in young patients with RMS in the head and neck region can lead to adverse effects on dental development and thereby, pose a challenge in planning dental intervention. Case presentation: This case report outlines the dental and facial developmental consequences in a 13-year-old child, who received chemo-radiotherapeutic intervention at the age of 7 years for the management of PM-RMS. Following treatment, the child exhibited significant dental complications, including arrested root growth and restricted mouth opening. Conclusions: This case highlights the necessity for interdisciplinary collaboration between oncologists, dentists, and other healthcare professionals to mitigate the adverse effects on dental health and overall quality of life in patients undergoing chemo-radiotherapy for rhabdomyosarcoma. [ABSTRACT FROM AUTHOR]

Simple Summary: Berlin–Frankfurt–Münster (BFM) protocols are widely used outside clinical trials to treat pediatric acute lymphoblastic leukemia patients. However, local specificities might demand treatment adaptations, like the reduction of high-dose methotrexate due to lack of pharmacokinetic monitoring, the substitution of conventional asparaginase for peg-asparaginase because of the unavailability of the latter, or flow-cytometry-based measurable residual disease instead of a PCR-based one. Here, we report the results of a 22-year period of children treated with BFM protocols in a developing country. The results were somewhat comparable to the BFM reports, and we conclude that BFM protocol adaptations can be safely implemented in developing countries, accounting for local specificities. Introduction: The objective of the current study was to determine the survival probabilities of children and adolescents with acute lymphocytic leukemia treated with adapted Berlin–Frankfurt–Münster (BFM) protocols and compare our results with the original BFM reports. Methods: This retrospective study included 695 patients up to 19 years old treated with adapted BFM protocols between 1997 and 2018 in four hospitals in Rio de Janeiro. The 1997–2007 and 2008–2018 cohorts were analyzed separately. Results: More than half of the patients were stratified into the high-risk BFM classification. Overall and event-free survivals were, in the 1997–2007 period, respectively, 88% and 80% (BFM standard risk group—SRG), 75% and 67% (intermediate risk group—IRG), and 48% and 33% (high-risk group—HRG). The corresponding numbers for the 2008–2018 period were 93% and 84% (SRG), 75% and 63% (IRG), and 64% and 57% (HRG). In the second period, both the OS (HR = 0.71, p = 0.011) and EFS (HR = 0.62, p < 0.001) were higher. Except for the intermediate-risk group, the latter results are comparable to the BFM. Conclusion: The BFM protocol adaptations can be safely implemented in developing countries, accounting for local specificities. [ABSTRACT FROM AUTHOR]

Neuroblastoma, the deadliest solid tumor in children, exhibits alarming mortality rates, particularly among high-risk cases. To enhance survival rates, a more precise risk stratification for patients is imperative. Utilizing proteomic data from 34 cases with or without N-Myc amplification, we identified 28 differentially expressed ubiquitination-related proteins (URGs). From these, a prognostic signature comprising 6 URGs was constructed. A nomogram incorporating clinical-pathological parameters yielded impressive AUC values of 0.88, 0.93, and 0.95 at 1, 3, and 5 years, respectively. Functional experiments targeting the E3 ubiquitin ligase FBXO42, a component of the prognostic signature, revealed its TP53-dependent promotion of neuroblastoma cell proliferation. In conclusion, our ubiquitination-related prognostic model robustly predicts patient outcomes, guiding clinical decisions. Additionally, the newfound pro-proliferative role of FBXO42 offers a novel foundation for understanding the molecular mechanisms of neuroblastoma. [ABSTRACT FROM AUTHOR]

Background: Effective response and reducing the burden of family care for children with cancer is critical, and China currently lacks a specific assessment tool. Aims: This study aimed to translate and validate the Caregiving Burden Scale for Family Caregivers of Children with Cancer (CBSFC-CC) and then test and implement the tool. Methods: According to the Beaton cross-cultural debugging guide, preliminary Chinese version of CBSFC-CC scale was formed, which was suitable for Chinese language environment and clinical context. Exploratory factor analyses (EFA) and confirmatory factor analyses (CFA) were performed to verify structural validity. Convergent validity, discriminant validity and reliability were also conducted. Results: A total of 529 family caregivers of children with cancer participated in the survey. EFA extracts and combines four factors and explained 65.80% of the total variation. CFA proved that all the goodness-of-fit indicators were acceptable. The Cronbach's alpha of the Chinese version of CBSFC-CC was.96, and the test–retest reliability coefficient was.95. Four dimensions and 29 items were identified in the final Chinese version of CBSFC-CC. Conclusion: The chinese version CBSFC-CC is scientifically reasonable and has good reliability and validity, which can be applied to the investigation of the nursing burden of family caregivers of children with cancer in China. [ABSTRACT FROM AUTHOR]

Atypical lipomatous tumors (ALTs) are locally aggressive adipocytic malignancies that frequently occur in middle-aged adults. We report the rare case of an ALT of the thigh that occurred in a 4-year-old girl. Since the tumor was initially diagnosed as a lipoblastoma by incisional biopsy, marginal resection was performed. Histopathological findings of the surgical specimen revealed the proliferation of mature and variously sized adipocytes, as well as ectopic ossification; these features differ from the typical findings of lipoblastoma. Immunohistochemical findings showed nuclear positivity for a murine double minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) and negativity for pleomorphic adenoma gene 1 (PLAG1). Fluorescence in situ hybridization showed abnormal amplification of the MDM2 gene. The patient was thus finally diagnosed as having an ALT. No signs of local recurrence or metastasis were noted 1 year postoperatively. This case is instructive in the differential diagnosis of primary adipocytic tumors. Lipoblastomas are the most common adipocytic tumors in children, but if a tumor is located in the deep tissue or imaging findings are not typical, the possibility of ALT should be considered and immunohistochemistry for MDM2 and CDK4 should be added. [ABSTRACT FROM AUTHOR]

Purpose: We sought to assess the feasibility, reproducibility, and accuracy of conventional and newer echocardiographic measures of right ventricular (RV) systolic function in adolescent and young adult childhood cancer survivors treated with anthracyclines. Methods: Echocardiography and cardiac magnetic resonance imaging (CMR) were acquired ≤60 days apart in prospectively recruited survivors and RV functional measures were quantitated by blinded observers. Repeat quantitation was performed in a subset to evaluate reproducibility. For each echocardiographic measure, Spearman correlations with CMR measures were calculated, and values in participants with CMR RV ejection fraction (RVEF) ≥48% and RVEF <48% were compared using two sample Wilcoxon rank‐sum tests. Results: Among 58 participants, mean age was 18.2 years (range 13.1–25.2) and five participants had CMR RVEF <48%. Intra‐ and inter‐observer coefficients of variation were 8.2%–10.1% and 10.5%–12.0% for adjusted automated strain measures, and 5.2%–8.7% and 2.7% for 3D RVEF, respectively. No echocardiographic measures were significantly correlated with CMR RVEF; only tricuspid annular plane systolic excursion was correlated with CMR RV stroke volume (r =.392, p =.003). Participants with RV dysfunction had worse automated global longitudinal strain (−20.3% vs. −23.9%, p =.007) and free wall longitudinal strain (−23.7% vs. −26.7%, p =.09). Conclusions: Echocardiographic strain and 3D RV function measurements were feasible and reproducible in at‐risk childhood cancer survivors. Although not associated with CMR RVEF in this population with predominantly normal RV function, automated strain measurements were more abnormal in participants with RV dysfunction, suggesting potential clinical utility of these measures. [ABSTRACT FROM AUTHOR]

Introduction: To identify the differentially expressed genes of acute myeloid leukaemia (AML) and construct and verify a survival prognosis model combined with patient survival information. Methods: The TARGET database was searched to identify differentially expressed peripheral blood genes in children with AML and healthy children. A gene set functional analysis and pathway analysis were performed using gene ontology and the KEGG pathway. A prognostic model for children with AML was constructed using univariate Cox, LASSO Cox regression and multivariate Cox regression analyses. Time‐dependent receiver operating characteristic (ROC) curves were adopted to assess the predictive capacity of the prognostic models. Results: In total, 1640 differentially expressed genes were screened (1119 upregulated and 521 downregulated genes). The differentially expressed genes were mainly involved in nutrient metabolism and cytochrome P450 metabolism. Six key genes related to the prognosis of AML, FAM157A, GPR78, IRX5, RP4‐800G7.1, RP11‐179H18.5 and RP11‐61N20.3, were identified. Kaplan–Meier curves indicated that 3‐year and 5‐year overall survival was significantly higher in the low‐risk group than in the high‐risk group. The area under the ROC curve was 0.722. At different stages of AML, FAM157A and RP4‐800G7.1 exhibited significant differences in expression. The expression levels of FAM157A were significantly decreased in AML, whereas the expression levels of GPR78, IRX5, RP4‐800G7.1, RP11‐179H18.5 and RP11‐61N20.3 were significantly increased in AML. Conclusion: A prognosis‐related gene model of AML was successfully constructed, and the expression levels of the model genes varied with AML stage. [ABSTRACT FROM AUTHOR]

CONTEXT: There is an increasing population of childhood cancer survivors (CCS) at risk for treatment-related toxicities, including skeletal morbidities. Bone mineral density (BMD) is a proxy for bone health and reductions are associated with osteoporosis and fractures. OBJECTIVE: To investigate bone health in CCS by conducting a systematic review and meta-analysis of BMD after completed treatments. DATA SOURCES: We searched Medline, Embase, Cochrane, and Web of Science in May 2019 and updated in May 2023. STUDY SELECTION: Studies reporting BMD Z-scores measured with dual-energy x-ray absorptiometry in CCS after treatment completion. DATA EXTRACTION: We performed a pooled analysis of studies reporting BMD Z-scores and thereafter we analyzed studies comparing BMD in survivors and healthy controls. All analyses were performed based on the site of BMD measurement. RESULTS: Of 4243 studies, 84 were included (N = 8106). The mean time off-treatment across the studies ranged from 2 months to 24 years. The overall pooled mean Z-score was -0.57 (95% confidence interval [CI] -0.59 to -0.55) in the whole-body, -0.84 (95% CI -0.86 to -0.83) in the lumbar spine, -0.79 (95% CI -0.81 to -0.77) in the femoral neck and -0.14 (95% CI -0.18 to -0.11) in the total hip. When comparing survivors with controls, BMD was significantly lower in survivors at all sites. LIMITATIONS: English publications, study-level meta-analysis. CONCLUSIONS: We showed a significant reduction of BMD Z-scores in CCS. Given the increased fracture risk already within -1 SD, these results emphasize the need for BMD surveillance and secondary prevention in CCS. [ABSTRACT FROM AUTHOR]

BACKGROUND AND OBJECTIVES: Conversations about dignity are fundamental to person-centered care in pediatrics, yet practical language strategies to promote and support dignity remain understudied. To address this gap, we aimed to identify and characterize language used by pediatric oncologists to recognize and affirm dignity across advancing illness. METHODS: In this longitudinal prospective study, we audio-recorded serial disease reevaluation encounters between pediatric oncologists, children with cancer, and families across 24 months or until the child's death. Using a hybrid deductive--inductive qualitative approach, we defined dignity language a priori on the basis of existing descriptions of dignity in the literature and then conducted a content analysis to refine the definition specific to pediatric cancer care before coding serial medical encounters. Thematic frequencies were reported by using descriptive statistics. RESULTS: A total of 91 discussions at timepoints of disease progression were audio-recorded for 36 patients and their families. No dignity language was identified in nearly half (45%) of "bad news" encounters, and the time spent by the oncologist engaging in dignity language represented a minority (<7%) of overall recorded dialogue. Within coded dialogue, we characterized 3 key themes upholding dignity language (empowerment, autonomy, respect). CONCLUSIONS: Opportunities exist to improve dignity communication in childhood cancer, and the authors propose a conceptual model ("Lend an EAR") to guide dignity-based communication in pediatric cancer. Future research should emphasize patient and parent perspectives on language to support dignity for children with advanced cancer, with stakeholder-driven refinement of the Lend an EAR model before integration and testing in communication skills training programs. [ABSTRACT FROM AUTHOR]

Purpose:Although fertility preservation for patients with childhood and adolescent cancer is considered standard of care in the high-resource settings, it is rarely offered in low-resource settings. This study explores the experiences and perspectives of oncology health care professionals in Uganda to identify contextual barriers and facilitators to addressing oncofertility in low-resource settings. Methods: Using ground theory, we conducted in-depth face-to-face interviews of health care professionals managing pediatric patients at the Uganda Cancer Institute (UCI). Using a systematic, semi-structured interview guide, participants were asked open-ended questions about their understanding of fertility preservation and their perspectives on implementing this care at their institution. Although all the eligible health care providers were interviewed, interview transcripts were uploaded into NVivo version 12 and openly coded as per theoretical requirements. Codes were refined into categories and later into structured themes. Results: Twelve health care professionals were interviewed. Most participants identified as female (n = 9). Their role in the medical team varied from nurses (n = 6), medical officers (n = 3), pediatric oncologists (n = 2), and pediatric oncology fellow (n = 1). Six themes were noted as follows: (1) importance of information, (2) importance of future fertility, (3) inadequate consideration to future fertility, (4) communication barriers, (5) inadequate knowledge, and (6) resource barriers. Conclusion: Although health care providers at the UCI face contextual barriers to addressing future fertility among patients with pediatric cancer, they value preserving fertility in this population. Future initiatives that aim to introduce oncofertility care in low-resource settings should prioritize educating providers and building capacity to meet the oncofertility needs in this setting. [ABSTRACT FROM AUTHOR]

Purpose: Social anxiety disorder (SAD) remains an understudied potential link between the cancer experience and adolescent and young adult (AYA) cancer survivors' poor psychosocial outcomes. We investigated the frequency and duration of, as well as factors associated with, symptoms of SAD among AYAs with cancer. Methods: This longitudinal, mixed-methods study involved online surveys (including a validated screening tool for SAD) at recruitment and 6 months later, and a structured clinical interview. Results: Twenty-eight AYAs (aged 12–30 years, <1-year postdiagnosis, 50% male) completed the first survey (M = 6 months postdiagnosis). About 32% reported clinically significant SAD symptoms. Fourteen completed the follow-up survey (M = 12 months postdiagnosis), of which 9 (62%) reported persistent or worse symptoms of SAD significantly associated with emotional distress, physical appearance concerns, negative social cognitions, and depression. Conclusion: A subset of AYAs with cancer may experience clinically significant SAD symptoms that can affect their psychosocial well-being. Further work on how to best identify and support AYAs with SAD is needed. [ABSTRACT FROM AUTHOR]

Purpose: We investigated the health-related quality of life (HRQoL) of an adolescent and young adult (AYA)-aged South African childhood cancer survivor (CCS) cohort. Methods: Participants completed the Minneapolis-Manchester Quality of Life adolescent and adult forms. The overall Cronbach's alpha coefficients were 0.81 (adolescent form) and 0.92 (adult form). The scale-level content validity indexes were acceptable (0.88 and 0.89 for the adolescent and adult forms, respectively). The total domain and overall HRQoL scores were calculated. Results: Sixty-two survivors completed the adolescent form and 30 completed the adult form. The median age was 17.5 years (range 13–34 years), and the median time from diagnosis was 12 years (male:female ratio 1:1.2). Risk factors for poor physical functioning included age at study visit (p = 0.015), solid tumor diagnosis (p = 0.012), radiotherapy (p = 0.021), and surgery (p = 0.006). Six or more late effects impacted most domains negatively; severe late effects (p = 0.020) decreased physical functioning. Lower socioeconomic status was associated with poorer physical (p = 0.006) and cognitive (p = 0.047) functioning. The adult form cohort had poorer psychological (p = 0.014) and social functioning (p = 0.005) and body image (p = 0.016) than the adolescent form cohort. Conclusion: Older age, radiotherapy, surgery, solid tumor diagnosis, and the number and severity of late effects negatively influenced HRQoL in AYA-aged CCSs. A long-term follow-up (LTFU) risk stratification system should include HRQoL status to assist with holistic LTFU care. [ABSTRACT FROM AUTHOR]

Astroblastoma is an uncommon circumscribed glial tumor mostly involving the cerebral hemisphere. The characteristic molecular alteration is meningioma (disrupted in balanced translocation) 1 (MN1) rearrangement. No definite World Health Organization grade has been assigned as both low‐ and high‐grade tumors are known to occur. Tumors in the spine are extremely rare; to date only three cases have been reported in the literature. A vigilant microscopy and ancillary testing aid in diagnosis when the tumors present in unusual locations, as in our case. The prompt differentiation of this tumor from its mimickers is a mandate as modalities of management are different and not clearly established. [ABSTRACT FROM AUTHOR]

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]

Simple Summary: Pediatric, adolescent, and young adult (AYA) cancer patients, especially those with rare cancers, face unique challenges that traditional support systems may not adequately address. This study explores the potential of the metaverse, a virtual shared space, to provide psychological support and foster a sense of community among these patients. By utilizing customizable avatars and facilitating interactions across geographical and temporal barriers, the metaverse enables patients to connect with peers, share experiences, and receive emotional support. The anonymity provided by avatars helps reduce appearance-related anxiety and the stigma associated with cancer treatment. This research highlights the metaverse's potential to transform the delivery of psychosocial support for patients with rare cancers, offering a novel approach to patient care that transcends physical limitations and fosters a global patient community. The findings suggest that integrating virtual spaces into healthcare models can effectively meet the complex needs of pediatric and AYA cancer patients. This study investigated the potential of the metaverse in providing psychological support for pediatric and AYA cancer patients, with a focus on those with rare cancers. The research involved ten cancer patients and survivors from four distinct regions in Japan, who participated in metaverse sessions using customizable avatars, facilitating interactions across geographical and temporal barriers. Surveys and qualitative feedback were collected to assess the psychosocial impact of the intervention. The results demonstrated that the metaverse enabled patients to connect with peers, share experiences, and receive emotional support. The anonymity provided by avatars helped reduce appearance-related anxiety and stigma associated with cancer treatment. A case study of a 19-year-old male with spinal Ewing's sarcoma highlighted the profound emotional relief fostered by metaverse interactions. The findings suggest that integrating virtual spaces into healthcare models can effectively address the unique needs of pediatric and AYA cancer patients, offering a transformative approach to delivering psychosocial support and fostering a global patient community. This innovative intervention has the potential to revolutionize patient care in the digital age. [ABSTRACT FROM AUTHOR]

Background and Objective: Olanzapine (OLZ) containing regimens are approved in adults for chemotherapy-induced nausea and vomiting (CINV) receiving highly emetogenic chemotherapy (HEC) or moderately emetogenic chemotherapy (MEC), and the same has not been approved in the pediatric population. In order to generate data regarding the efficacy and safety of OLZ as an adjunct to the standard of care (SoC) for CINV in pediatric patients receiving HEC/MEC, the review authors performed this systematic review and meta-analysis. Methods: A systematic literature search was performed through the databases Cochrane Library, Pub Med, and clinicaltrials.gov, from inception to September 2023, using keywords: "chemotherapy" and "olanzapine," "nausea" and "vomiting." Randomized clinical trials published in English that analyzed the efficacy and safety of olanzapine as an adjunct to SoC were included. The essential outcomes included in this study were the proportion of patients with no emesis in the acute and delayed phase, patients with no nausea in the acute and delayed phase, the proportion of patients requiring rescue medication, and the proportion of patients with reduced CNS arousal. Results: In the OLZ group, a greater number of patients had no emesis both in the acute and delayed phase (RR = 1.22; 95% CI = 1.09-1.37; P =.0004); and (RR = 1.23; 95% CI = 0.92-1.63; P =.16) respectively. Similarly, a higher number of patients showed no nausea both in the acute and delayed phase (RR = 1.08; 95% CI = 0.78-1.48; P =.66) and (RR = 1.12; 95% CI = 0.79-1.61; P =.52) respectively. The use of rescue medication was significantly less in the OLZ group (RR = 0.62; 95% CI = 0.42-0.91; P =.01). More patients experienced reduced CNS arousal in the OLZ group (RR = 2.97; 95% CI = 2.02-4.38; P <.0001). Conclusions: Olanzapine as an adjunct to the SoC may be effective in acute emesis, which may also reduce the use of rescue medication. Reduced CNS alertness was the significant adverse effect observed. For other endpoints, more studies are required to substantiate its role in CINV. [ABSTRACT FROM AUTHOR]

Purpose: Pediatric spinal cord gliomas (PSGs) are rare in children and few reports detail their imaging features. We tested the association of tumoral grade with imaging features and proposed a novel approach to categorize post-contrast enhancement patterns in PSGs. Methods: This single-center, retrospective study included patients <21 years of age with preoperative spinal MRI and confirmed pathological diagnosis of PSG from 2000-2022. Tumors were classified using the 5th edition of the WHO CNS Tumors Classification. Two radiologists reviewed multiple imaging features, and classified enhancement patterns using a novel approach. Fisher's exact test determined associations between imaging and histological features. Results: Forty-one PSGs were reviewed. Thirty-four were intramedullary, and seven were extramedullary. Pilocytic astrocytoma was the most common tumor (39.02%). Pain and weakness were the most prevalent symptoms. Seven patients (17.07%) died. Cyst, syringomyelia, and leptomeningeal enhancement were associated with tumor grade. Widening of the spinal canal was observed only in low-grade astrocytomas. There was a significant association between tumor grade and contrast enhancement pattern. Specifically, low-grade PSGs were more likely to exhibit type 1A enhancement (mass-like, with well-defined enhancing margins) and less likely to exhibit type 1B enhancement (mass-like, with ill-defined enhancing margins). Conclusion: PSGs display overlapping imaging features, making grade differentiation challenging based solely on imaging. The correlation between tumor grade and contrast enhancement patterns suggests a potential diagnostic avenue, requiring further validation with larger, multicenter studies. Furthermore, Low-grade PSGs display cysts and syringomyelia more frequently, and leptomeningeal enhancement is less common. [ABSTRACT FROM AUTHOR]

BACKGROUND: A major obstacle in translating the therapeutic potential of chimeric antigen receptor (CAR) T cells to children with central nervous system (CNS) tumors is the blood–brain barrier. To overcome this limitation, preclinical and clinical studies have supported the use of repeated, locoregional intracranial CAR T-cell delivery. However, there is limited literature available describing the process for the involvement of an investigational drug service (IDS) pharmacy, particularly in the setting of a children’s hospital with outpatient dosing for CNS tumors. OBJECTIVES: To describe Seattle Children’s Hospital’s experience in clinically producing CAR T cells and the implementation of IDS pharmacy practices used to deliver more than 300 intracranial CAR T-cell doses to children, as well as to share how we refined the processing techniques from CAR T-cell generation to the thawing of fractionated doses for intracranial delivery. METHODS: Autologous CD4+ and CD8+ T cells were collected and transduced to express HER2, EGFR, or B7-H3–specific CAR T cells. Cryopreserved CAR T cells were thawed by the IDS pharmacy before intracranial delivery to patients with recurrent/refractory CNS tumors or with diffuse intrinsic pontine glioma/diffuse midline glioma. RESULTS: The use of a thaw-and-dilute procedure for cryopreserved individual CAR T-cell doses provides reliable viability and is more efficient than typical thaw-and-wash protocols. Cell viability with the thaw-and-dilute protocol was approximately 75% and was always within 10% of the viability assessed at cryopreservation. Cell viability was preserved through 6 hours after thawing, which exceeded the 1-hour time frame from thawing to infusion. CONCLUSION: As the field of adoptive immunotherapy grows and continues to bring hope to patients with fatal CNS malignancies, it is critical to focus on improving the preparatory steps for CAR T-cell delivery. [ABSTRACT FROM AUTHOR]

Schizophrenia (SCZ) and schizoaffective disorder (SHZ) are psychiatric disorders commonly identified in individuals in their late adolescence or early adulthood. Comorbidities are common, though a concurrent diagnosis of leukemia, one of the most frequently occurring cancers of adolescence, has not yet been described in such cases. This case study outlines the clinical presentation, course, and treatment response of two 17-year-old male adolescents whose psychotic disorders complicated their leukemia treatment. The first patient was diagnosed with leukemia and subsequently with SCZ while undergoing leukemia treatment. The second patient was diagnosed with SHZ prior to the onset of leukemia. The case study will follow the methodology of Robert E. Stake (Abma & Stake, 2014), as the two cases share a leukemia diagnosis and the reported mental health impact connected with cancer-directed treatment. Early identification and treatment are critical for both psychotic disorders and cancers, often impacting the long-term prognosis. However, when co-occurring, their interplay can present unique challenges to care. [ABSTRACT FROM AUTHOR]

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