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Context Children with cancer are at risk of poor nutritional status during treatment and into survivorship. Objectively measured taste perception and self-reported food hedonics are 2 factors that may influence food intake. Objective This 2-armed systematic review examined whether chemotherapy and radiotherapy affect (1) taste perception and (2) hedonic experiences of children and survivors of childhood cancer. Data Source A 2-armed systematic literature search was conducted in the Medline, CINAHL, Embase, and PsychInfo database until June 2022. The effects of cancer treatment on objective taste perception or food hedonics (ie, food liking or aversion and appetite) were examined. Data Extraction Peer-reviewed articles published in English of studies that included children (aged <18 years) or survivors of childhood cancer (any age) were reviewed. Risk of bias was determined using the Evidence Analysis Library by the Academy of Nutrition and Dietetics. Data Analysis A total of 1417 articles in the taste search arm and 3862 articles in the hedonics search arm were identified. Of these, 9 and 4 articles were eligible for review, respectively. Cancer treatment had highly variable effects on taste perception during treatment and into survivorship. Learned food aversions were experienced by children receiving chemotherapy treatment and liking of meats and salty foods by children with cancer was affected. The impact of treatment on appetite varied. Conclusions Cancer treatment did not uniformly affect taste perception. Food liking may be negatively affected, and learned food aversions may develop during cancer treatment. To establish the clinical relevance of childhood cancer treatment on taste perception and food hedonics, more research is required. Systematic Review Registration PROSPERO registration no.CRD42020207127. [ABSTRACT FROM AUTHOR]

The article discusses the use of the BRAF inhibitor dabrafenib for the treatment of pilocytic astrocytoma with the BRAF V599ins mutation in an 8-year-old boy. The case report details the patient's symptoms, diagnosis, treatment, and response to dabrafenib therapy, showing significant improvement in neurological deficits and tumor size reduction. The study highlights the effectiveness of molecular targeted therapy over conventional chemotherapy for tumors with rare mutations like BRAF V599ins, emphasizing the importance of thorough molecular testing for appropriate treatment decisions. [Extracted from the article]

PURPOSE: Our study aimed to explore real-world treatment scenarios for children and adolescents with neurotrophic tropomyosin receptor kinase (NTRK)–fused tumors, emphasizing access, responses, side effects, and outcomes. PATIENTS AND METHODS: Pooled clinical data from 17 pediatric cases (11 soft-tissue sarcomas, five brain tumors, and one neuroblastoma) treated with larotrectinib and radiologic images for 14 patients were centrally reviewed. Testing for gene fusions was prompted by poor response to treatment, tumor progression, or aggressiveness. RESULTS: Six different NTRK fusion subtypes were detected, and various payment sources for testing and medication were reported. Radiologic review revealed objective tumor responses (OR) in 11 of 14 patients: Complete responses: two; partial responses: nine; and stable disease: three cases. Grades 1 or 2 Common Terminology Criteria for Adverse Events adverse effects were reported in five patients. Regarding the entire cohort's clinical information, 15 of 17 patients remain alive (median observation time: 25 months): four with no evidence of disease and 11 alive with disease (10 without progression). One patient developed resistance to the NTRK inhibitor and died from disease progression while another patient died due to an unrelated cause. CONCLUSION: This real-world study confirms favorable agnostic tumor OR rates to larotrectinib in children with NTRK-fused tumors. Better coordination to facilitate access to medication remains a challenge, particularly in middle-income countries like Brazil. [ABSTRACT FROM AUTHOR]

Aims: The concordance of adolescents with cancer and caregivers was examined, and the core elements of transition readiness were identified. Methods: In this cross‐sectional study, 196 adolescent–caregiver dyads completed the Chinese version of Self‐Management and Transition to Adulthood with Rx = Treatment Questionnaire and its parent version between March 2023 and August 2023. Intraclass correlation coefficients, paired t‐tests and network analysis were used for data analysis. Results: Caregivers reported slightly lower scores for transition readiness than adolescents (3.28 vs. 3.32). Healthcare engagement and provider communication were core elements in transition readiness networks. At the dyad level, agreement between adolescents' and caregivers' transition readiness ranged from poor to fair (intraclass correlation coefficients 0.103–0.486), and a significant difference in structure was found between the two networks. Conclusions: Caregivers tended to underestimate adolescents' transition readiness. Attaining better concordance between adolescents and family caregivers is critical to aligning roles and responsibilities in the transition process. Implications for Paediatric Cancer Care: This study extends the evidence on the variation in adolescents' transition readiness, clarifying the complex associative relationships among the elements of transition readiness, which can be potential pathways for improving transition readiness. Second, this study is the first to assess transition readiness from a dyad's perspective. The findings highlighted the patient–caregiver incongruence in rating patients' transition readiness, suggesting that targeted dyadic interventions should be developed and implemented to improve patient–caregiver transition readiness concordance, facilitate effective communication and mutuality between patients and caregivers, and contribute to their collaboration during the transition of adolescents and optimization of outcomes. What problem did the study address?: Increased long‐term survival rates of survivors of paediatric cancer highlighted the significant need for care continuity.Transitional readiness is an important predictor of adolescent survivor's ability to adapt to a long‐term survival period.Assessments of adolescents' transition readiness are limited and overlook the synergies between family caregivers and adolescents in the transition period. What Were the Main Findings?: The levels of agreement on rating transition readiness varied from poor to fair among adolescent–caregiver dyads, and caregivers tended to underestimate adolescents' transition readiness.The findings highlighted the patient–caregiver incongruence in rating patients' transition readiness.Targeted dyadic interventions should be developed and implemented to improve patient–caregiver's transition readiness concordance, and facilitate effective communication and mutuality between patients and caregivers. Reporting Method: Study methods and results reported in adherence to the STROBE checklist. Patient or Public Contribution: No patients or members of the public were involved in the study. Contribution to the Wider Global Clinical Community: The main findings introduce pathways for improving transition readiness, which can enhance healthcare transition among other medical populations. [ABSTRACT FROM AUTHOR]

The promising results seen in the treatment of refractory hematologic malignancies with tisagenlecleucel (Kymriah), the pioneering Chimeric Antigen Receptor (CAR) T-cell immunotherapy, has stimulated a fast growth in research and clinical testing of novel CAR-T constructs, targets, and various generations of CAR T-cells. Pharmacists may receive inquiries about active clinical trials or may be contributing to the care of patients participating in these studies. This briefing discusses the on-going clinical trials that explore novel CAR T-cell immunotherapies in pediatric refractory malignancies of hematologic and solid organ origins. It can be valuable in assisting practitioners in navigating the rapidly evolving field of CAR T-cell immunotherapies. [ABSTRACT FROM AUTHOR]

Background: Neuroblastoma (NB), the most common extracranial solid tumor in children, is featured by high malignancy and poor prognosis. Flavokawain A (FKA), a novel chalcone isolated from the roots of the kava plant, has been identified to exert the tumor-inhibiting properties in various cancers. The present study was formulated to tell about the anticarcinogenic effects of FKA against NB and to thoroughly elucidate the intrinsic molecular mechanisms. Methods: In this work, for functional experiments, SK-N-SH cells were treated with various concentrations (0, 12.5, 25, 50 μM) of FKA in order to expound the tumor-inhibiting functions of FKA on proliferative ability, clone-forming ability, apoptosis, cell cycle arrest, migratory ability, invasive ability, EMT and in vitro angiogenesis of NB cells. Moreover, to probe into the intrinsic molecular mechanisms underlying the tumor-inhibiting functions of FKA in NB cells, FKA-treated SK-N-SH cells were co-treated with ERK activator LM22B-10 for rescue experiments. Results: In our current work, it was verified that FKA treatment suppressed the proliferative and clone-forming abilities of NB cells, facilitated NB cell apoptosis, arrested NB cell cycle as well as inhibited NB cell migration, invasion, EMT and in vitro angiogenesis in a dose-dependent manner. What's more, molecular docking predicted the compound-protein interaction between FKA and ERK and biotin pull-down assay validated the binding of FKA to ERK. FKA targeted on ERK and acted as an inhibitor of ERK to inactivate ERK/VEGF/MMPs signaling pathway. Treatment with ERK activator LM22B-10 partially abolished the tumor-inhibiting functions of FKA in NB. Conclusion: Overall, FKA may suppress the malignant behaviors of NB cells depending on inactivation of ERK/VEGF/MMPs signaling pathway. [ABSTRACT FROM AUTHOR]

Background: Multiple differentials exist for pediatric liver tumors under 2 years. Accurate imaging diagnosis may obviate the need for tissue sampling in most cases. Objective: To evaluate the imaging features and diagnostic accuracy of computed tomography (CT) in liver tumors in children under 2 years. Methods: Eighty-eight children under 2 years with treatment naive liver neoplasms and baseline contrast-enhanced CT were included in this institutional review board approved retrospective study. Two blinded onco-radiologists assessed these tumors in consensus. Findings assessed included enhancement pattern, lobulated appearance, cystic change, calcifications, central scar-like appearance, and metastases. The radiologists classified the lesion as hepatoblastoma, infantile hemangioma, mesenchymal hamartoma, rhabdoid tumor, or indeterminate, first based purely on imaging and then after alpha-fetoprotein (AFP) correlation. Multivariate analysis and methods of comparing means and frequencies were used for statistical analysis wherever applicable. Diagnostic accuracy, sensitivity, and positive predictive values were analyzed. Results: The mean age of the sample was 11.4 months (95% CI, 10.9–11.8) with 50/88 (57%) boys. The study included 72 hepatoblastomas, 6 hemangiomas, 4 mesenchymal hamartomas, and 6 rhabdoid tumors. Presence of calcifications, multilobular pattern of arterial enhancement, lobulated morphology, and central scar-like appearance was significantly associated with hepatoblastomas (P-value < 0.05). Fourteen out of eighty-eight lesions were called indeterminate based on imaging alone; six lesions remained indeterminate after AFP correlation. Pure radiology-based diagnostic accuracy was 81.8% (95% CI, 72.2–89.2%), which increased to 92.1% (95% CI, 84.3–96.7%) (P-value > 0.05) after AFP correlation, with one hepatoblastoma misdiagnosed as a rhabdoid tumor. If indeterminate lesions were excluded for biopsy, the accuracy would be 98.8% (95% CI, 93.4–99.9%). Conclusion: CT had high accuracy for diagnosing liver neoplasms in the under 2-year age population after AFP correlation. Certain imaging features were significantly associated with the diagnosis of hepatoblastoma. A policy of biopsying only indeterminate lesions after CT and AFP correlation would avoid sampling in the majority of patients. [ABSTRACT FROM AUTHOR]

Background: Wilms tumor (WT) is the most common malignant renal tumor in children. This study investigated the clinical features, pathological findings, and outcomes of children with malignant renal tumors in Southern Iran. Factors associated with recurrence and mortality were assessed. Methods: Electronic files of children with malignant renal tumors from 2009 to 2023 were reviewed. The 5-year overall survival (OS) and event-free survival (EFS) were reported. Results: Eighty-three patients (44 males) with a median age of 40 months (range: 3-122) were included. WT was the most common pathological variant (94%). Anaplasia was found in 17.3% of patients. Upfront chemotherapy followed by nephrectomy was performed in 54.2% of the patients. Ten patients (12%) experienced relapse, and five patients (6%) died during the 14-year follow-up. The 5-year OS and EFS were 90.75% (95% CI, 78.64–96.16%) and 81.9% (95% CI, 70.10-89.38%), respectively, and were comparable between the two treatment strategies (upfront chemotherapy vs. upfront nephrectomy). Metastasis and residual disease were associated with relapse, whereas tumor recurrence was the only predictive factor of survival. Conclusion: WT is a curable disease with excellent outcomes if diagnosed and treated promptly. The timing of nephrectomy does not affect OS and EFS. Patients with low-stage tumors and those with complete surgical excision are at a lower risk of tumor recurrence. Relapse is the primary risk factor for death. [ABSTRACT FROM AUTHOR]

Neuroblastoma is the most common extracranial solid tumor in children, and a leading cause of childhood cancer deaths. All neuroblastomas arise from neural crest-derived sympathetic neuronal progenitors, but numerous mutations, the most common of which is MYCN amplification, give rise to these lesions. Epigenetic aberrations also play a role in oncogenesis and tumor progression. To better understand biologic diversity of neuroblastomas, we performed joint single-nucleus ATAC sequencing and single-nucleus RNA sequencing on six neuroblastoma cell lines, three of which are MYCN amplified. After standard filtering for high-quality nuclei, we obtained chromatin accessibility and transcript abundance data from 41,733 neuroblastoma tumor cells. Preliminary analysis reveals significant diversity in chromatin landscape and gene expression across neuroblastoma cell lines. This dataset is a valuable resource for studying the transcriptional and epigenetic mechanisms of this deadly childhood disease. [ABSTRACT FROM AUTHOR]

Objective: Pediatric posterior fossa tumors pose diagnostic challenges due to their diverse histopathological features and variable clinical presentations. Conventional magnetic resonance imaging (MRI) serves as the initial diagnostic tool; however, additional modalities, such as diffusion-weighted imaging (DWI), are essential for refining tumor classification. This retrospective single-center study aimed to evaluate the diagnostic utility of apparent diffusion coefficient (ADC) parameters in distinguishing between the most common pediatric posterior fossa tumors. Materials and Methods: Fifty-nine patients under the age of 18 (27 females and 32 males) with histopathologically diagnosed primary posterior fossa tumors underwent pre-treatment conventional and diffusion MRI. Apparent diffusion coefficient values were measured from solid tumor regions and normal cerebellar parenchyma, with subsequent calculation of tumor/normal cerebellar ADC ratios. Results: The median ADC values for pilocytic astrocytomas (PAs) were 1786.2 ×10−6 mm² /s, ependymomas 1144.9 × 10−6 mm² /s, and for medulloblastomas 666.1 × 10−6 mm2 /s were significantly different (P < .001 for all three). Similarly, the median ADC ratios demonstrated discriminatory potential, with PAs showing the highest ratio (2.46), followed by ependymomas (1.55) and medulloblastomas (0.89) (P < .001 for all three). Receiver operating characteristic analysis revealed distinct ADC cutoffs and ratios for differentiating all tumor types from each other. Conclusion: Despite limitations, such as a small cohort size and different MRI protocols, our results show that ADC metrics are especially useful for distinguishing between the most common pediatric posterior fossa tumors. We recommend that future studies integrate advanced imaging techniques and larger cohorts to improve diagnostic accuracy. [ABSTRACT FROM AUTHOR]

ABSTRACT: Aims: This study aims to review our experience of treating ovarian masses in children with an emphasis on clinical presentation, diagnosis, treatment, and outcome. Methods: We retrospectively reviewed the electronic medical records of all patients below 18 years of age who underwent surgical treatment for ovarian masses at our institute between 2009 and 2023. Study variables included demography, clinical presentation, physical findings, tumor markers, radiologic features, operative details, histopathology, follow-up status, and overall survival. Results: During the study period, 30 patients with a mean age of 10.07 years (range: 15 days–18 years) underwent surgical treatment for ovarian masses. Nonneoplastic ovarian masses were seen in 5 (16.7%) patients, whereas 25 (83.3%) patients had benign (10 [33.3%], borderline 3 [10%], or malignant 12 [40%]) ovarian neoplasms. The most common clinical presentation in the benign group was abdominal pain (n = 6), whereas painless abdominal mass (n = 6) was the predominant complaint in children with malignant tumors. A functional ovarian mass presenting with precocious puberty or virilization was seen in 5 (16.7%) patients. On imaging, nonneoplastic and benign lesions had a mean size of 4.33 (range: 3.1–6) cm and 12.63 (range: 2.8–28) cm, respectively, whereas borderline and malignant masses had a mean tumor size of 22.5 (range: 6.5–32) cm and 12.55 (range: 3.5–18.7) cm, respectively (P < 0.05). The cystic component was identified in all nonneoplastic and benign tumors, whereas the solid component was present in all borderline and malignant lesions (P < 0.05). Tumor markers such as serum alpha-fetoprotein and beta-human chorionic gonadotropin were raised in 8 (66.67%) of malignant tumors, whereas markers were normal in all benign lesions and borderline malignant lesions and 4 (33.33%) of malignant tumors. Lactate dehydrogenase (LDH) was also raised in all malignant masses (n = 12), whereas it was normal in all benign and borderline malignant masses (n = 18). In 6 (20%) patients with nonneoplastic and benign masses with maximum tumor size <6 cm, the laparoscopic approach was adopted, whereas open surgery was preferred in the rest of the patients. At a mean follow-up of 53.5 (range: 4–117) months, all patients are alive and disease free. Conclusions: Preoperative imaging characteristics (tumor size and solid component) and raised tumor markers may help us to differentiate between benign and malignant ovarian pathologies. The overall prognosis of pediatric ovarian tumors seems to be favorable. [ABSTRACT FROM AUTHOR]

Simple Summary: Proton beam radiation therapy is one of the major treatment modalities used for cancer treatment, including brain tumors. This treatment modality is distinct from other radiation options because of its ability to deliver radiation to tumor targets while sparing healthy tissue. Brainstem toxicity is a rare but important complication can arise due to exposure to radiation; hence, in this systematic review and meta-analysis, we aimed to explore the risk of brainstem toxicity in pediatric brain tumor patients undergoing proton beam radiation, focusing on quantifying its incidence and severity. Eleven articles were considered eligible in our study, and the results yielded an overall brainstem toxicity incidence of 1.8%, ranging in severity, with Grade 1 brainstem toxicity (asymptomatic) being the most common. This study revealed a low incidence of symptomatic brainstem toxicity and related mortality among pediatric brain tumor patients undergoing proton beam radiation, which could support the idea of it having a good toxicity profile and possibly re-enforces the need for more comprehensive primary studies regarding this radiation modality in brain tumor patients to uncover unknowns and help us understand the grey areas of this topic. Background: Proton beam radiation therapy (PBRT) is an advanced cancer treatment modality that utilizes the distinctive physical properties of protons to precisely deliver radiation to tumor targets while sparing healthy tissue. This cannot be obtained with photon radiation. In this systematic review and meta-analysis, we aimed to comprehensively assess the risk of brainstem toxicity in pediatric brain tumor patients undergoing PBRT. Methods: With adherence to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a predetermined search strategy was used to identify eligible articles from PubMed, Web of Science, Scopus, and Cochrane Library through July 2024. Results: The current study included a total of 11 eligible articles. The pooled prevalence of patients who suffered from brainstem toxicity was 1.8% (95% CI: 1%, 2.6%). The pooled prevalences of patients with Grade 1 to Grade 5 brainstem toxicity were found to be 10.6% (95% CI: 8.8%, 30%), 1.5% (95% CI: 0.6%, 2.5%), 0.7% (95% CI: 0.3%, 1.1%), 0.4% (95% CI: 0.1%, 0.7%), and 0.4% (95% CI: 0.1%, 0.8%), respectively, with an overall pooled prevalence of 0.7% (95% CI: 0.4%, 1%). Conclusions: This study revealed a relatively low incidence of symptomatic brainstem toxicity and its related mortality in the pediatric population undergoing PBRT. However, further research is encouraged to study the broader effects of PBRT and to explore various factors that may influence the risk of brainstem toxicity in patients treated with PBRT. [ABSTRACT FROM AUTHOR]

Simple Summary: The purpose of this pediatric cancer research investigation was to assess how radiation therpay in the first primary malignannt neoplasm predispose to Second Primary Thyroid Maligancy (SPTM), as well as therapeutics and mortality associated with SPTM. This study applied a retrospective cohort design, as non-experiment translational epidemiologic investigation, utilizing the USA National Cancer Institute, Surveillance Epidemiology and End Results (SEER) data in the assessment of risk determinants and predisposing factors in SPTM, as well as racial differentials in SPTM cumulative incidence and mortality. Specifically, radiation utilization in first primary malignancy among children predisposed to SPTM, indicative of the need to assess exposure effect of other carcinogens prior to radiation therapy in first primary malignant neoplasm, hence SPTM marginilaization. Objective: To assess racial and sex variances in second primary thyroid malignancy (SPTM) cumulative incidence and temporal trends and the radiation exposure effect in pediatric SPTM. Materials and Methods: A retrospective cohort study, a non-experimental epidemiologic design, was used to assess the cumulative incidence (CmI) and temporal trends as well as the exposure effect of radiation in SPTM among children, 0–19 years, in the Surveillance, Epidemiology, and End Results (SEER) registry, National Cancer Institute (NCI), USA. Percent change (PC) and annual percent change (APC) were used to examine CmI rates and temporal trends, while chi-square statistics and binomial regression models were used to examine variable distribution by race and to determine the exposure effect of radiation on SPTM as well as mortality, respectively. Results: The frequency of pediatric thyroid cancer was (n = 3457) between 1973 and 2013/14, while the PC was 151.2 for all races but 99.8 among whites. Of all pediatric thyroid cancers diagnosed during this period, SPTM accounted for 3% (n = 99). Compared to whites, blacks/AA were 60% less likely to present with SPTM, adjusted risk ratio, aRR = 0.40, 99% CI 0.06–2.47, while other races were 18% more likely to develop SPTM, aRR = 1.18, 99% CI 0.48–2.87. Additionally, females relative to males were 63% less likely to be diagnosed with SPTM, aRR = 0.37, 99% CI 0.22–0.61. With respect to urbanicity, compared to children in rural areas, those in urban areas were 21% less likely to develop SPTM, aRR = 0.79, 99% CI, 0.12–5.35, while children in metropolitan areas were 40% less likely to develop SPTM, aRR = 0.60, 99% CI, 0.10–3.59. Although imprecise, there was a 5% increased risk of SPTM, with radiation as an exposure effect, aRR = 1.05, 99% CI 1.01–1.75. Conclusions: There are increasing temporal trends in pediatric SPTM with blacks relative to whites having observed lower incidence, despite an increasing percent change among blacks/AA, indicative of the disproportionate burden of this malignant neoplasm. SPTM risk was higher among males and in rural areas, while radiation as a risk for SPTM was clinically and biologically meaningful, albeit an observed statistically insignificant inference due to sampling variability, requiring intervention mapping in radiation exposure margination among children. [ABSTRACT FROM AUTHOR]

Simple Summary: There has been little improvement in Ewing sarcoma survival rates over the past several decades and new therapeutic options are desperately needed. Histone deacetylases (HDACs) are known to play a role in cancer development and progression. We show that HDAC2 is overexpressed in Ewing sarcoma cells and that an HDAC inhibitor, Panobinostat, is cytotoxic to Ewing sarcoma cells both alone and when combined with standard of care. Mechanistically, we show that Panobinostat increases the DNA damage caused by standard of care leading to the death of the Ewing sarcoma cells. We believe that HDAC inhibition combined with standard-of-care chemotherapeutics may be an effective treatment option for Ewing sarcoma patients. Background: The survival rate of patients with Ewing sarcoma (EWS) has seen very little improvement over the past several decades and remains dismal for those with recurrent or metastatic disease. HDAC2, ALK, JAK1, and CDK4 were identified as potential targets using RNA sequencing performed on EWS patient tumors with the bioinformatic analysis of gene expression. Methods/Results: The pan-HDAC inhibitor Panobinostat was cytotoxic to all the Ewing sarcoma cell lines tested. Mechanistically, Panobinostat decreases the expression of proteins involved in the cell cycle, including Cyclin D1 and phospho-Rb, and DNA damage repair, including CHK1. Further, Panobinostat induces a G1 cell cycle arrest. The combination of Panobinostat with Doxorubicin or Etoposide, both of which are used as standard of care in upfront treatment, leads to a synergistic effect in EWS cells. The combination of Panobinostat and Doxorubicin induces an accumulation of DNA damage, a decrease in the expression of DNA damage repair proteins CHK1 and CHK2, and an increase in caspase 3 cleavage. The addition of Panobinostat to standard-of-care chemotherapy combinations significantly reduces cell viability compared to that of chemotherapy alone. Conclusions: Overall, our data indicate that HDAC2 is overexpressed in many EWS tumor samples and HDAC inhibition is effective in targeting EWS cells, alone and in combination with standard-of-care chemotherapy agents. This work suggests that the addition of an HDAC inhibitor to upfront treatment may improve response. [ABSTRACT FROM AUTHOR]

Purpose: Childhood central nervous system (CNS) tumors tend to have a longer time interval until diagnosis than other pediatric malignancies. The aim is to describe the time to diagnosis among Brazilian pediatric patients treated at a tertiary center and explore associated factors. Methods: Cross-sectional study; application of questionnaires to parents of children with CNS tumors during outpatient visit or inpatient care. Results: One hundred parents participated between August and November 2023. The median age of the children at diagnosis was 7.2 years old. Low-grade glioma (LGG) was the most common tumor type (37%), followed by medulloblastoma (24%). The most frequent symptoms were morning and/or persistent vomiting and headache. The mean prediagnostic symptomatic interval (PSI) was 150 days. The mean parental interval was shorter than the medical (58.1 days vs 92.8 days). LGGs and tumors located in the central area had longer intervals to diagnosis than other tumors (296 vs 54 days) (p = 0.005) and (206 vs 155 days) (p = 0.007), respectively. Despite 81% of the patients undergoing pediatric routine follow-up, 87% of them had been diagnosed at an emergency department. Children attended by the same physician had a shorter mean interval (18.2 vs 88.3 days) than those assisted by different professionals (p = 0.015). The mean time for referral to our specialized center was 23 days. Conclusions: This study is a crucial step in recognizing barriers to early diagnosis of CNS tumors in a middle-income country as low awareness of signs/symptoms by parents and health professionals, aiming to provide opportunities for intervention strategies to reduce the time to diagnosis. [ABSTRACT FROM AUTHOR]

OBJECTIVES: To characterize the spectrum of legal options considered in cases of treatment refusal, nonadherence, and abandonment (TRNA); clinicians' thought processes regarding legal intervention; and perceived consequences of legal involvement. METHODS: We conducted in-depth, semistructured interviews with 30 pediatric oncologists between May and September of 2019 regarding experiences with TRNA. The interview guide covered types of conflicts encountered; factors and strategies considered in response; effects of TRNA cases, personally and professionally; the role of ethical frameworks and legal requirements; and resources needed to manage TRNA cases. Interviews were transcribed and coded iteratively using thematic analysis. RESULTS: Participants represented a range of institutional sizes, geographic locations, and years in practice. Twenty-five of 30 interviewees discussed legal consideration with regard to TRNA. Most participants first engaged the legal system through child protective service agencies. They considered patient age, treatment efficacy, quality of life (burden of treatment), and prognosis; family resources and social context; and preservation of therapeutic relationships and possible consequences of reporting. Experiences and outcomes of legal involvement varied. CONCLUSIONS: Clinicians struggle with the tension between obligations to report medical neglect and fears that reporting may result in more harm than benefit to the child in question. We urgently need more dialog between health care professionals and child protective services and legal professionals. Stakeholders from both groups would benefit from a greater understanding of the other's thought processes; clarity regarding the relevant facts; and mutual progress toward creative, evidence-based solutions to working out these complex challenges. [ABSTRACT FROM AUTHOR]

Introduction: Young adult cancer survivors (YACS; ages 18–39) report a significant psychological burden. Entertainment media narratives (e.g., books, movies, shows that are produced for mass consumption) might be an effective tool for reducing this distress, although little is known about present use among YACS. Method: YACS completed a survey about their use of entertainment media narratives to cope with cancer using an adapted version of the Brief COPE. Additionally, YACS reported their use of entertainment media narratives to start conversations about their experience with others, and they described features of entertainment media narratives that they found helpful in coping. Results: We recruited 108 YACS from three recruitment sites. Most participants were White (n = 65), female (n = 54), and recruited from Prolific (n = 56), an Internet-based, crowdsourced data collection platform. Participants were, on average, 30 years old and 45 months from the completion of their primary cancer treatment. YACS who used entertainment media to cope with cancer (n = 32; 29.6%) were significantly younger and significantly closer to the end of their primary treatment. Compared to nonusers, users of entertainment media narratives to cope were also more likely to identify as Black; identifying as Black was associated with a 2.05-factor increase in using narrative entertainment media to cope with cancer even when controlling for other demographic differences. Additionally, compared to their peers, Black YACS reported greater use of narratives to start cancer-related conversations. Emotional and inspirational storylines were the most helpful story features. Discussion: Some YACS, especially Black YACS, use stories to cope with their cancer experience. YACS patients could find stories useful in exploring their cancer-related emotions, although the exact benefits are still unknown. [ABSTRACT FROM AUTHOR]

Objective: To describe the experience of implementing social robotics as an adjuvant during the hospitalization process in pediatric oncology patients. Methods: Before and after cohort study, applying an intervention with the Lego Mindstorms EV3 kit in patients between 8 and 17 years old that are hospitalized with a cancer diagnosis. We excluded patients from the intensive care unit or when their treating physician recommended so. The intervention consisted of a three-phase workshop: an open architecture story, building a car robot using the Lego Mindstorm EV3 kit, and cooperative playing activities such as races and passing obstacles. Results: Thirteen patients received the intervention with robotic lego. The median age was 15 years (IQR = 3), and 84.6% of the population (n = 11) were male. We found significant improvement in the language (topic management p =.011 and communicative intention p =.034). Other characteristics improved, but not significantly (self-care activities index, catching). No adverse events occurred during the intervention. Conclusions: The results of this pilot study suggest that implementing social robotics during hospitalization in children with cancer is a therapeutic adjuvant and safe intervention that promotes better communication, self-care, and a physical activity improvement. For future studies, the impact of this intervention could be measured in hospitalized pediatric cancer patients. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study is to examine the protective and risk factors of substance use behaviors (tobacco, marijuana, e-cigarette, and alcohol) among young adult childhood cancer survivors. The study focused on clinical (receipt of cancer-related follow-up care, treatment intensity, late effects, depressive symptoms, self-rated health) and demographic (race/ethnicity, neighborhood socioeconomic status) factors and their associations with substance use. Methods: Participants were from the Project Forward cohort, a population-based study of young adult survivors of childhood cancers. Participants (N = 1166, Mage = 25.1 years) were recruited through the Los Angeles Cancer Surveillance Program (Cancer Registry covering Los Angeles County, California). Multivariate path analyses were performed with substance use as the outcome variables and clinical and demographic factors as independent variables. Covariates included age and sex. Finding: Substance use was positively associated with depressive symptoms, and inversely associated with cancer-related follow-up care, female sex, age, Hispanic ethnicity, treatment intensity, and self-rated health. Neighborhood SES was inversely associated with tobacco use, while being positively associated with binge drinking and e-cigarette use. The results highlight the interrelationship between the clinical and demographic variables and their associations with different substance use. Conclusion: Findings support the need for effective interventions targeting substance use behavior among CCS. This will help improve long-term outcomes and mitigate the risk for early morbidity. [ABSTRACT FROM AUTHOR]

Purpose: Identify subgroups of patients with distinct joint anxiety AND depression profiles and evaluate for differences in demographic and clinical characteristics, as well as stress, resilience, and coping. Design: Longitudinal study Participants: Patients (n = 1328) receiving chemotherapy. Methods: Measures of state anxiety and depression were done six times over two cycles of chemotherapy. All of the other measures were completed prior to second or third cycle of chemotherapy. Latent profile analysis was used to identify the distinct joint anxiety and depression profiles. Findings: Three classes were identified (i.e. Low Anxiety and Low Depression (57.5%); Moderate Anxiety and Moderate Depression (33.7%), High Anxiety and High Depression (8.8%)). For all of the stress measures, a dose response effect was seen among the profiles. Two worst profiles reported higher occurrence rates for a number of adverse childhood experiences. Implications for providers: Patients need referrals for stress reduction techniques and mental health and social services. [ABSTRACT FROM AUTHOR]

Today, a majority of children diagnosed with cancer are expected to grow up and live—hopefully until old age. Still, knowledge of the lived experience of childhood cancer survivors is sparse. In pursuit of knowledge expansion, by combining my intersecting roles as an academic, educational counselor, and childhood cancer survivor, I approach my personal illness narrative. By means of evocative autoethnography, I write intentionally vulnerably about my experiences and make them available for consideration. I explore my narrative through archives, artifacts, memories of the past, and conversations evoked in the present. I re-visit the cultural landscape of a southern Norwegian girl growing up in the 00s with cancer. Through this, my illness narrative presents as positioned, tangled, and interwoven with a developmental trajectory. Specific educational experiences seem to linger, and many are related to being absent from or re-entering school after the onset of illness. To grasp the intersecting and conflicting experiences of being very ill while also young, I suggest Erik Erikson's moratorium as a key concept. To complement Arthur Frank's illness narratives of restitution, chaos, and quest, I establish the moratorium narrative. As a fresh resource, the moratorium narrative underlines the need to make sensitive our academic community's gaze on illness trajectories unfolding in formative phases and illness narratives defined by growing up. By providing a point of recognition that prompts elaboration, this could also provide the young and very ill with a much-needed narrative space of opportunity, of which more narratives are invited and insisted upon. [ABSTRACT FROM AUTHOR]

Childhood cancers are life-threatening diseases that affect the whole family. During the treatment moral situations might arise. Research on how parents perceive moral challenges in childhood cancer care is sparse. The aim was to explore parents' main concern, and how they deal with their main concern, when facing moral challenges in childhood cancer care. Data collection included focus group interviews with parents. The data analysis was carried out according to a classical theory and revealed the core category "Sheltering in chaos". The strategies to handle the main concern included "To bring the child through a life-saving trajectory" by "Balancing control" and "Deliberating of suffering. The consequences included feelings of being "Torn between roles". "Familiarity" emerged as a facilitating factor to handle moral challenges. The results indicate that parents are torn between different roles, and that the care experience is improved through familiarity with the healthcare professionals. The following findings may inspire new ways of offering moral support to families in childhood cancer care. [ABSTRACT FROM AUTHOR]

Different parents grieve differently. However, research directed at understanding the important contextual or individual factors that influence the path each bereaved parent takes is lacking. In this qualitative analysis we seek to understand the array of bereaved parent experiences more completely. By deeply diving into one parent dyad using interpretive phenomenology analysis and situating that story within the conventional content analysis of 13 other bereaved parents of adolescents and young adults (AYAs) who died from advanced cancer, we illustrate the roles of religion/spirituality, maintaining a connection, and fulfilling parental roles as elements of grief processing. Clinicians and investigators should consider similar individualized approaches to understanding and supporting the grief experiences of bereaved parents before and after the death of a child. [ABSTRACT FROM AUTHOR]

Background: Neuroblastoma is the most common extracranial solid tumor in children and accounts for 15% of pediatric cancer related deaths. Targeting neuroblastoma with immunotherapies has proven challenging due to a paucity of immune cells in the tumor microenvironment and the release of immunosuppressive cytokines by neuroblastoma tumor cells. We hypothesized that combining an oncolytic Herpes Simplex Virus (oHSV) with natural killer (NK) cells might overcome these barriers and incite tumor cell death. Methods: We utilized MYCN amplified and non-amplified neuroblastoma cell lines, the IL-12 expressing oHSV, M002, and the human NK cell line, NK-92 MI. We assessed the cytotoxicity of NK cells against neuroblastoma with and without M002 infection, the effects of M002 on NK cell priming, and the impact of M002 and priming on the migratory capacity and CD107a expression of NK cells. To test clinical applicability, we then investigated the effects of M002 and NK cells on neuroblastoma in vivo. Results: NK cells were more attracted to neuroblastoma cells that were infected with M002. There was an increase in neuroblastoma cell death with the combination treatment of M002 and NK cells both in vitro and in vivo. Priming the NK cells enhanced their cytotoxicity, migratory capacity and CD107a expression. Conclusions: To the best of our knowledge, these investigations are the first to demonstrate the effects of an oncolytic virus combined with self-maintaining NK cells in neuroblastoma and the priming effect of neuroblastoma on NK cells. The current studies provide a deeper understanding of the relation between NK cells and neuroblastoma and these data suggest that oHSV increases NK cell cytotoxicity towards neuroblastoma. [ABSTRACT FROM AUTHOR]

PURPOSE Pediatric lung tumors are primarily discussed in the surgical literature. However, limited research has been reported on their imaging findings, and only a few tumor types have been documented. Therefore, the aim of this article is to describe the imaging features of primary lung tumors in children. METHODS The archives of the pediatric radiology unit were reviewed for primary lung tumors documented between 2007 and 2023. In total, 24 patients (9 girls and 15 boys; aged 5 months to 16 years) were included in the study. Their demographic characteristics, clinical presentation, and histopathologic results were obtained. All imaging studies were reviewed by two radiologists for various findings (e.g., lymphadenopathy, atelectasis, pleural effusion, calcification, multiplicity, pneumothorax, axial and lobar location, laterality, tumor margin, mediastinal shift, contrast enhancement pattern, signal intensity on T1- and T2-weighted images, and diffusion pattern), and a final decision was made by consensus. The mean tumor size was compared between the benign and malignant groups using a t-test. RESULTS There were 15 (62.5%) benign tumors, as follows: inflammatory myofibroblastic tumor (IMT; n = 10, 41%), hemangioma (n = 2, 8%), pneumocytoma (n = 2, 8%), and mature cystic teratoma (n = 1, 4%). Moreover, there were 9 (37.5%) malignant tumors, as follows: pleuropulmonary blastoma (PPB; n = 6, 25%), adenocarcinoma (n = 2, 8%), and lymphoepithelioma-like carcinoma (LELC) (n = 1, 4%). The most frequently reported symptoms were cough, fever, dyspnea, chest pain, and recurrent infection; six patients reported no clinical symptoms. Fifteen tumors (62%) were located in the right lung. The mean tumor diameter at the time of diagnosis was 6.4 ± 3 cm (benign group: 6.7 ± 3.4 cm; malignant group: 6 ± 2.3 cm, P > 0.050). Calcification was present in 80% of the patients with IMT. At the time of diagnosis, two (8.3%) patients were found to have metastasis: one was diagnosed with adenocarcinoma and the other with LELC. Tumors were located peripherally in 18 (75%) patients. CONCLUSION The symptoms associated with lung masses are non-specific. There is no correlation between tumor size and malignancy. The most common tumors observed in this study were IMT and PPB, respectively. IMT is highly associated with calcification. CLINICAL SIGNIFICANCE Primary lung tumors are rarely seen in children, and they have different histopathological types. Calcification might be an important radiological clue for the diagnosis of IMT, which is the most common lung tumor in children. [ABSTRACT FROM AUTHOR]

CREB gene family (ATF1, CREB1, CREM) fusions with either EWSR1 or FUS gene partners drive the pathogenesis of a wide range of neoplasms, including various soft tissue tumors, intracranial myxoid mesenchymal tumors (IMMTs), hyalinizing clear cell carcinoma (HCCC), and rare mesotheliomas. Recently, a SMARCA2::CREM fusion was reported in one case each of IMMT and HCCC. In this study, we expand the clinicopathologic and molecular spectrum of these neoplasms by describing three additional cases with SMARCA2::CREM and one with a novel SMARCA4::CREM fusion, highlighting the recurrent potential of additional CREB gene fusion partners beyond FET family members. To evaluate if these fusions define a new pathologic entity, we performed a comprehensive genomic and methylation analysis and compared the results to other related tumors. Tumors occurred in children and young adults (median age 20 years) and spanned a broad anatomic distribution, including soft tissue, intracranial, head and neck, and prostatic urethra. Microscopically, the tumors shared an undifferentiated round to epithelioid cell phenotype and a hyalinized fibrous stroma. Immunohistochemically, a polyphenotypic profile was observed, with variable expression of SOX10, desmin, and/or epithelial markers. No targetable genomic alterations were found using panel‐based DNA sequencing. By DNA methylation and transcriptomic analyses, tumors grouped closely to FET::CREB entities, but not with SMARCA4/SMARCB1‐deficient tumors. High expression of CREM by immunohistochemistry was also documented in these tumors. Patients experienced local recurrence (n = 2), locoregional lymph node metastases (n = 2), and an isolated visceral metastasis (n = 1). Overall, our study suggests that SMARCA2/4::CREM fusions define a distinct group of neoplasms with round cell to epithelioid histology, a variable immunoprofile, and a definite risk of malignancy. Larger studies are needed to further explore the pathogenetic relationship with the FET::CREB family of tumors. © 2024 The Pathological Society of Great Britain and Ireland. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Osteochondroma is the most common bone tumor and is a surface bone lesion that includes cortical and medullary bone with a hyaline cartilage cap. Benign osteochondroma is a common tumor in children that may be asymptomatic but can cause pain and limit joint movement when arising in a joint. This report describes the presentation, diagnosis, and management of an intra-articular osteochondroma of the right elbow joint in an 8-year-old boy. Case Report: An 8-year-old boy experienced persistent right elbow pain and limited motion, which were unresponsive to conservative measures. Examination revealed a firm swelling in the right cubital fossa. Radiographic and advanced imaging confirmed an osteochondroma originating from the capitellum and trochlea. Surgical exploration via a lateral approach and capsulotomy excised a lobulated intra-articular mass (5×2×1.5 cm). Histopathology showed a hyaline cartilage cap with typical chondrocytes and endochondral ossification, and normal fatty marrow and hematopoietic elements in the stroma. The procedure restored normal elbow function. This case is the first documented instance of an elbow joint intra-articular osteochondroma. Conclusions: This report has highlighted the importance of surgical removal and histopathology in the diagnosis of this common bone lesion to exclude the differential diagnoses of intra-articular masses that include a foreign body, enchondroma, chondroblastoma, periosteal chondroma, chondromyxoid fibroma, or malignant chondrosarcoma. [ABSTRACT FROM AUTHOR]

Medulloblastoma is the most common malignant brain tumor in children. It has WNT-driven, SHH-driven/TP53 mutant, SHH-driven/TP53 wildtype, and non-WNT/non-SHH subgroups. MAGMAS (Mitochondrial Associated Granulocyte Macrophage colony-stimulating factor Signaling molecules) encodes a mitochondrial import inner membrane translocase subunit and is responsible for the translocation of matrix proteins across the inner membrane. We previously reported that a small molecule MAGMAS inhibitor, BT9, decreases cell proliferation, migration, and oxidative phosphorylation in adult glioblastoma cell lines. The aim of our study was to investigate whether the chemotherapeutic effect of BT9 can be extended to pediatric medulloblastoma. Methods: DAOY (SHH driven/tp53 mutant) and D425 (non-SHH group 3) were treated with BT9. For in vitro analysis, cell proliferation, death, migration, invasion, and metabolic activity were assessed using MTT assay, TUNEL staining, scratch wound assay, Matrigel invasion chambers, and seahorse assay, respectively. A D425 orthotopic xenograft mouse model was used to evaluate BT9 efficacy in vivo. Results: BT9 treatment resulted in a significant decrease in cell proliferation (DAOY, 24 hours IC50: 3.6 μM, 48 hours IC50: 2.3 μM, 72 hours IC50: 2.1 μM; D425 24 hours IC50: 3.4 μM, 48 hours IC50: 2.2 μM, 72 hours IC50: 2.1 μM) and a significant increase in cell death (DAOY, 24 hours p = 0.0004, 48 hours p<0.0001; D425, 24 hours p = 0.0001, 48 hours p = 0.02). In DAOY cells, 3 μM BT9 delayed migration and significantly reduced DAOY and D425 cell invasion (p < 0.0001). It also modified mitochondrial respiratory function in both medulloblastoma cell lines. Compared to control, however, BT9 administration did not improve survival in a D425 orthotopic xenograft mouse model. Conclusions: Our in vitro data showed BT9 antitumor efficacy in DAOY and D425 cell lines, suggesting that BT9 may represent a promising targeted therapeutic in pediatric medulloblastoma. These data, however, need to be further validated in animal models. [ABSTRACT FROM AUTHOR]

Background: Neuroblastoma (NB), a prevalent extracranial solid tumor in children, stems from the neural crest. Omics technologies are extensively employed in NB, and We analyzed published articles on NB omics to understand the research trends and hot topics in NB omics. Method: We collected all articles related to NB omics published from 2005 to 2023 from the Web of Science Core Collection database. Subsequently, we conducted analyses using VOSviewer, CiteSpace, Bibliometrix, and the Bibliometric online analysis platform (https://bibliometric.com/). Results: We included a total of 514 articles in our analysis. The increasing number of publications in this field since 2020 indicates growing attention to NB omics, gradually entering a mature development stage. These articles span 50 countries and 1,000 institutions, involving 3,669 authors and 292 journals. The United States has the highest publication output and collaboration with other countries, with Germany being the most frequent collaborator. Capital Medical University and the German Cancer Research Center are the institutions with the highest publication count. The Journal of Proteome Research and the Journal of Biological Chemistry are the most prolific journal and most co-cited journal, respectively. Wang, W, and Maris, JM are the scholars with the highest publication count and co-citations in this field. "Neuroblastoma" and "Expression" are the most frequent keywords, while "classification," "Metabolism," "Cancer," and "Diagnosis" are recent key terms. The article titled "Neuroblastoma" by John M. Maris is the most cited reference in this analysis. Conclusion: The continuous growth in NB omics research underscores its increasing significance in the scientific community. Omics technologies have facilitated the identification of potential biomarkers, advancements in personalized medicine, and the development of novel therapeutic strategies. Despite these advancements, the field faces significant challenges, including tumor heterogeneity, data standardization issues, and the translation of research findings into clinical practice. [ABSTRACT FROM AUTHOR]

Background: Robot-assisted surgery (RAS) is being performed with increasing frequency in pediatric oncology. We report our experience with RAS for renal tumors in children and compare the outcomes between RAS and laparoscopic surgery (LAS). Methods: A total of 23 patients with renal tumor who underwent minimally invasive surgery (MIS) between January 2020 and December 2023 were included in the study. The inclusion criteria enrolled in this study was unilateral tumors with maximum tumor diameter less than 10 cm. Patients who had enlarged lymph node, venous thrombosis, preoperative tumor rupture, bilateral renal tumor, or extrarenal extension on imaging were deemed contraindications and excluded. Patient demographics, operative details, postoperative outcomes and follow-up were recorded. Results: Among these patients, 17 underwent RAS and 6 underwent LAS. In the RAS group, the median age was 64 months (range, 9–156) with a median weight of 19.48 kg (range, 8.4–46.5); the maximum tumor diameter at operation was 55.65 mm (range, 22–88); the operation time was 188.8 min (range, 120–210), the intraoperative blood loss was 20 ml (range, 5–50), and the length of postoperative hospital stay was 4 days (range 1–9). There was no significant difference in patients' age, weight, location, tumor size, histological pattern and operation time between the two groups (P > 0.05). The RAS group had a significantly less intraoperative blood loss (P = 0.026) and less length of postoperative stay (P = 0.01) than the LAS group. Conclusion: Our initial experience suggested that RAS in pediatric renal tumor was feasible and safe, and it reduced surgical trauma and accelerate postoperative recovery for the patients. Due to the limitations of sample size and study quality, the clinical importance of these findings still needs to be further verified. [ABSTRACT FROM AUTHOR]

Introduction: Spinal astroblastoma is a rare highly malignant tumor that mainly affects children. We review the few cases described in the literature and highlight the challenges of managing this neoplasm by illustrating a case recently treated at our institutions. To our knowledge, this is the first published case of EWSR1-BEND2 fused spinal astroblastoma with long-term follow-up. Case Presentation: An 8-year-old girl was transferred from her home country to Switzerland for treatment of a recurrent intramedullary tumor of the cervical spine extending from C2-C7. The tumor was primarily diagnosed as an ependymoma of the spinal cord. Prior to her transfer to our department, the patient had undergone subtotal resection of the lesion, radiation therapy, multiple chemotherapy regimens, and biopsy of the recurrent tumor. Clinically, the patient presented with tetraparesis and had recently experienced worsening upper extremity weakness with complete loss of hand function. We performed a near total resection of the recurrent tumor. Ultra-fast Nanopore seq® based DNA methylome profiling allowed confirmation of the molecular diagnosis of a high-grade neuroepithelial tumor (HGNET-MN1) consistent with astroblastoma in less than 2 h, with subsequent molecular workup revealing a EWSR1-BEND2 fusion. After surgery, the patient gradually regained function in her hands. She was sent to a specialized pediatric rehabilitation center, and while the tumor was being followed radiologically with no adjuvant treatment planned, the patient presented with a relapse of the tumor in only 3 months. Given the acute worsening of radiating pain and sudden respiratory failure, a cervical decompression was performed. MRI of the cervical spine showed infiltration of the lower aspects of the brainstem. The patient was offered palliative comfort care. Conclusion: Spinal astroblastoma is a rare and highly aggressive tumor affecting children and young adults with a high recurrence rate and thus far not well-defined prognosis. The molecular signature of astroblastoma needs to be further characterized to establish a treatment-relevant classification and to allow a better prognostication. Currently, gross-total resection combined with radiotherapy remains the mainstay of treatment for spinal astroblastoma. [ABSTRACT FROM AUTHOR]

Simple Summary: We discuss the role of risk-stratification and personalized treatment for various pediatric cancer patients, with the goal being to improve tumor control and decrease late effects of radiation in long-term survivors. We discuss settings in which radiation can be safely omitted or de-escalated, such as Hodgkin lymphoma, Wilms tumor with lung metastases and WNT pathway Medulloblastoma, and settings that warrant treatment escalation such as larger tumors with rhabdomyosarcoma or Ewing sarcoma, poor responders to chemotherapy and oligometastatic disease settings. We also summarize currently enrolling COG and other cooperative group trials. While the cure rate of cancer in children has markedly improved in the last few decades, late effects continue to be a problem in survivors. Radiotherapy, which is a major component of treatment in many cancers, is one of the major agents responsible for late toxicity. In the past decade, radiotherapy has been omitted in patients achieving excellent response to chemotherapy, such as in Hodgkin lymphoma and some Wilms tumors with lung metastases. Likewise, response to chemotherapy has been used to determine whether lower doses of radiation can be delivered in intracranial germinoma and pediatric nasopharyngeal carcinoma. Molecular subtyping in medulloblastoma is currently being employed, and in WNT-pathway M0 tumors, the reduction in radiotherapy dose to the craniospinal axis and tumor bed is currently being investigated. Finally, dose escalation was recently evaluated in patients with rhabdomyosarcoma > 5 cm who do not achieve a complete response to initial 9 weeks of chemotherapy as well as for unresectable Ewing sarcoma patients to improve local control. [ABSTRACT FROM AUTHOR]

Simple Summary: Appendiceal neuroendocrine neoplasms are very rare in pediatric populations; thus, there are no common guidelines or consensus for the management in children and adolescents, and adult protocols are usually adopted. In the present study, we report on 17 patients that underwent appendectomy for appendicitis with incidental diagnosis of a neuroendocrine tumor revealed upon histologic examination. Patients' characteristics, tumor histology, follow-up and outcome are described and compared to data from the literature, with similar results being reported in various studies. Analysis of previous scientific publications on children with appendiceal neuroendocrine neoplasms was performed and recommendations for treatment and follow-up were reviewed. This tumor displays benign behavior and an excellent outcome in children and adolescents; thus, many authors agree that aggressive surgery after the patient's first appendectomy is not necessary and follow-ups can be reduced. Background/Objectives: Appendiceal neuroendocrine neoplasms (ANENs) are usually found incidentally during histology examination after appendectomy for appendicitis. Due to their rarity in pediatric populations, there is no consensus on treatment or follow-up. The analysis of patients with ANENs of our and other studies will increase the understanding of this tumor. Methods: Pediatric patients with ANENs were uniformly managed at our center between 1998 and 2023. Patients' presenting symptoms, surgery, tumor histology, post-surgical work-up, follow-up and outcome were analyzed. Results: Our report describes 17 patients with a diagnosis of ANEN after appendectomy. The median age was 14 years (range of 4–17 years). Tumors were located at the tip of the appendix in 58.8% of cases and only one had a diameter >1 cm. All were well-differentiated tumors with free resection margins. The submucosa was invaded in five cases, muscularis propria in eight and subserosa in four. Post-appendectomy work-up included tumor marker measurement, abdominal ultrasound and computed tomography or magnetic resonance imaging, chest X-ray and octreotide scintigraphy. No residual tumors or metastases were detected. Additional surgery was not necessary. Follow-up was carried out for a median duration of 6 years (range of 1–10 years). Only one patient was lost to follow-up and all other patients are alive without tumor recurrence. Conclusions: The tumor characteristics of our patients confirmed data from the literature. With the lack of a sufficient number of large prospective trials, it is important to add more information to confirm the benign nature and excellent outcome of this tumor, even without additional surgery. Consensus guidelines are needed for ANENs in pediatric populations. [ABSTRACT FROM AUTHOR]

Osteosarcoma (OS) is the most severe bone tumor in children. A chemotherapy regimen includes a combination of high-dose Methotrexate (MTX), doxorubicin, and cisplatin. These drugs cause acute and chronic side effects, such as infections, thrombocytopenia, neutropenia, DNA damage, and inflammation. Therefore, to identify new therapeutic strategies, effective and with a safety profile, is necessary. The Hedgehog (Hh) signaling pathway involved in tumorigenesis is active in OS. Hh components Patched receptor 1 (PTCH1), Smoothened (SMO), and glioma-associated oncogene homolog transcription factors (GLI1 and GLI2) are overexpressed in OS cell lines and patient samples. Curcumin (CUR)—with antioxidant and anti-cancer properties—downregulates Hh components in cancer, inhibiting progression. This study investigates CUR effects on the MG-63 OS cell line, alone and combined with MTX, to propose a novel therapeutic approach. Our study suggests CUR as a novel therapeutic agent in OS, particularly when combined with MTX. Targeting the Hh signaling pathway, CUR and MTX showed significant pro-apoptotic effects, increasing the BAX/Bcl-2 ratio and total apoptotic cell percentage. They reduced the expression of Hh pathway components (PTCH1, SMO, GLI1, and GLI2), inhibiting OS cell proliferation, survival, and invasion. CUR and MTX combined determined a β-Catenin decrease and a trend toward reducing NF-kB and matrix metalloproteinases (MMP-2 and MMP-9). Our findings suggest CUR as a support to OS treatment, improving outcomes and reducing the adverse effects of current therapies. [ABSTRACT FROM AUTHOR]

Objective: Testicular tumor (TT) is a uncommon disease posing serious health problem. There are differences in some aspects between adult and pediatric TT. The study was to compare their differences of clinical and histological characteristics through the analysis of the long-term experiences in TT patients from two institutions. Materials and methods: The clinical data of hospitalized patients was collected and analyzed retrospectively from January 2014 to January 2024 at a pediatric and an adult institution, respectively. The data included composition, gender, age, initial presentation, tumor size, tumor markers, pathological diagnosis. Results: A total of 195 hospitalized patients were included. There were 135 children and 60 adult with TT, respectively. Of these children, patients were aged from 1 month to 14 years, with a mean age of 2.32 years. More cases (37.04%) were diagnosed at age younger than 1 years. 69 cases were left-sided, 65 cases were right-side and only 1 case was bilateral. Pediatric TTs mainly included 82 prepubertal teratomas, 37 had prepubertal yolk sac tumors and 3 mixed malignant germ tumors. Testicular surgeries included testicular-sparing surgery (TSS) (n = 73), radical orchiectomy (n = 60), and testicular biopsy (n = 2). There were 24 patients receiving postoperative chemotherapy. Adult TTs mainly contained 17 seminomas, 10 prepubertal teratomas,7 postpubertal teratomas, 6 stromal tumors and 3 embryonal carcinomas. The average age was 34.08 years. There were 29 right-sided, 27 left-sided and 4 bilateral tumors. TSS (n = 26), radical orchiectomy (n = 33), and testicular biopsy (n = 1) were performed in these TT patients. Only 6 patients received postoperative chemotherapy. The most common symptom was a painless scrotal mass at initial diagnosis in both groups. In addition, we found that significant differences were explored between histological type and age, tumor size (P < 0.05). Yolk sac tumor and seminoma were the most common malignant TT in pediatric and adult population, respectively. After two year follow-up, two children with yolk sac tumor and 4 adults with seminoma died of their diseases. Conclusions: The majority of pediatric cases were benign compared to adult. The most common type was prepubertal teratoma and yolk sac tumor. Pediatric TTs often occurred under the age of 1 year. Seminomas and prepubertal teratomas were commonly found in adult TTs, especially for young adult. We found that pediatric tumor type was associated with age and tumor size. TSS should be considered for benign TTs based on frozen biopsy findings in children. [ABSTRACT FROM AUTHOR]

Background: The giant cell tumor of the tendon pod is a benign neoplasia that can be present in any bone or tendon pod. Its etiology is unknown. Nevertheless, it is related to a chronic inflammatory process. It usually occurs in women between the third and fifth decade as a palpable and painless mass and slow growth, although it can be deformed and lead to limb loss. Case presentation: In this report, we present the case of an 11-year-old male patient, which began on June 21, 2020, with a blunt trauma. An ultrasound was performed, reporting compatible data with synovial sarcoma. Magnetic resonance was requested, with mass evidence in soft tissues in central and planting portions. A surgical procedure with a split and biopsy of the mass was carried out. The specimen was sent to the Pathology Department, leading to a diagnosis of a giant cell tumor of the tendon pod. Conclusions: The giant cell tumor of the tendon pod is rare. However, in some cases, it is crucial to consider it as a differential diagnosis. The surgical management of this entity has proven to reduce recurrence rates. [ABSTRACT FROM AUTHOR]

Background: The number of children diagnosed with cancer is expected to increase by 2050. The caring aspect of these children is usually left to members of the family, more so the mothers. Caregivers are usually faced with various challenges, such as financial issues, social stigma, and emotional and psychological issues. Caregivers use various coping strategies to manage the challenges they experience in the caring process, which can be positive or negative coping strategies. Objective: To explore the challenges and coping strategies of caregivers of children with cancer receiving care at a national referral hospital in Kenya. Methods: This study employed a descriptive phenomenological approach to explore the challenges and coping strategies of caregivers of children with cancer receiving treatment at a national referral hospital in Kenya. Purposive and snowball sampling techniques were used to select 44 participants for five focus group discussions (FGDs) conducted between October and December 2023. The data were analysed via inductive thematic analysis. Results: Four themes arose from the challenges experienced, which included psychological, financial, treatment-related, and social/family-related issues. These eleven subthemes included stress, depression, lack of transport, lack of food, loss of employment, treatment delays, and marital issues, among others. Two themes related to coping strategies were positive adaptation and denial, which were highlighted as negative coping strategies. Positive coping included praying, crying, talking to people, and seeking help. Conclusion: Caregivers of children with cancer undergo various challenges and apply various coping strategies to try and adapt to their new normal. Reinforcing positive coping strategies and helping caregivers utilize other coping strategies, such as the use of support groups, could help them better care for their children. [ABSTRACT FROM AUTHOR]

Incidental brain tumor findings in children involve the unexpected discovery of brain lesions during imaging for unrelated reasons. These findings differ significantly from those in adults, requiring a focus on pediatric-specific approaches in neurosurgery, neuroimaging, and neuro-oncology. Understanding the prevalence, progression, and management of these incidentalomas is crucial for informed decision-making, balancing patient welfare with the risks and benefits of intervention. Incidental brain tumors are observed in about 0.04–5.7% of cases, with most suspected low-grade lesions in children showing a benign course, though up to 3% may undergo malignant transformation. Treatment decisions are influenced by factors such as patient age, tumor characteristics, and family anxiety, with conservative management through surveillance often preferred. However, upfront surgery may be considered in cases with low surgical risk. Initial follow-up typically involves a comprehensive MRI after three months, with subsequent scans spaced out if the lesion remains stable. Changes in imaging or symptoms during follow-up could indicate malignant transformation, prompting consideration of surgery or biopsy. Several challenges and controversies persist, including the role of upfront biopsy for molecular profiling, the use of advanced imaging techniques like PET-CT and magnetic resonance spectroscopy, and the implications of the child's age at diagnosis. These issues highlight the need for further research to guide management and improve outcomes in pediatric patients with incidental brain tumor findings. [ABSTRACT FROM AUTHOR]

Background: Low-grade glioma is the most common brain tumor in children with different modes of treatment and a high overall survival. Low-grade glioma is considered a chronic disease, since residual tumor is present in many children. The tumor and its treatment lead to acquired brain injury with diverse consequences for later life based on factors like the diverse tumor locations, treatment(s) applied, neurofibromatosis type 1, and age at diagnosis. Methods: An overview of affected domains is provided based upon cohort studies from literature and partially based on clinical experience with a practical approach regarding each domain of functioning in order to provide insight in the requirements for long-term care assistance after childhood low-grade glioma. Results: The diverse domains that can potentially be affected are described as follows: motor function, speech, eating and swallowing, sensory functions, seizures, neuropathy, organ function after systemic treatment, late effects due to cranial radiation (vascular changes and secondary tumors, endocrine and hypothalamic function, sleep and energy, neuro-cognition and education, psychosocial effects, and quality of life. Conclusion: Insight in affected domains guides advices for medical follow-up, diagnostics, supportive instructions, and assistive measures per domain of functioning and provide insight in the requirements for long-term care assistance after childhood low-grade glioma. [ABSTRACT FROM AUTHOR]

Purpose: Pediatric low-grade gliomas (pLGG) are the most common brain tumors in children and achieving complete resection (CR) in pLGG is the most important prognostic factor. There are multiple intraoperative tools to optimize the extent of resection (EOR). This article investigates and discusses the role of intraoperative ultrasound (iUS) and intraoperative magnetic resonance imaging (iMRI) in the surgical treatment of pLGG. Methods: The tumor registries at Tuebingen, Rome and Pretoria were searched for pLGG with the use of iUS and data on EOR. The tumor registries at Liverpool and Tuebingen were searched for pLGG with the use of iMRI where preoperative CR was the surgical intent. Different iUS and iMRI machines were used in the 4 centers. Results: We included 111 operations which used iUS and 182 operations using iMRI. Both modalities facilitated intended CR in hemispheric supra- and infratentorial location in almost all cases. In more deep-seated tumor location like supratentorial midline tumors, iMRI has advantages over iUS to visualize residual tumor. Functional limitations limiting CR arising from eloquent involved or neighboring brain tissue apply to both modalities in the same way. In the long-term follow-up, both iUS and iMRI show that achieving a complete resection on intraoperative imaging significantly lowers recurrence of disease (chi-square test, p < 0.01). Conclusion: iUS and iMRI have specific pros and cons, but both have been proven to improve achieving CR in pLGG. Due to advances in image quality, cost- and time-efficiency, and efforts to improve the user interface, iUS has emerged as the most accessible surgical adjunct to date to aid and guide tumor resection. Since the EOR has the most important effect on long-term outcome and disease control of pLGG in most locations, we strongly recommend taking all possible efforts to use iUS in any surgery, independent of intended resection extent and iMRI if locally available. [ABSTRACT FROM AUTHOR]

Pediatric low-grade gliomas (pLGGs) are the most common brain tumor types affecting children. Although gross-total resection remains the treatment of choice, many tumors are not amenable to complete removal, because they either involve midline structures, such as the optic chiasm or hypothalamus, and are not conducive to aggressive resection, or have diffuse biological features and blend with the surrounding brain. Historically, radiation therapy was used as the second-line option for disease control, but with the recognition that this often led to adverse long-term sequelae, particularly in young children, conventional chemotherapy assumed a greater role in initial therapy for unresectable tumors. A variety of agents demonstrated activity, but long-term disease control was suboptimal, with more than 50% of tumors exhibiting disease progression within 5 years. More recently, it has been recognized that a high percentage of these tumors in children exhibit constitutive activation of the mitogen-activated protein kinase (MAPK) pathway because of BRAF translocations or mutations, NFI mutations, or a host of other anomalies that converged on MAPK. This led to phase 1, 2, and 3 trials that explored the activity of blocking this signaling pathway, and the efficacy of this approach compared to conventional chemotherapy. Despite initial promise of these strategies, not all children tolerate this therapy, and many tumors resume growth once MAPK inhibition is stopped, raising concern that long-term and potentially life-long treatment will be required to maintain tumor control, even among responders. This observation has led to interest in other treatments, such as immunotherapy, that may delay or avoid the need for additional treatments. This chapter will summarize the place of immunotherapy in the current armamentarium for these tumors and discuss prior results and future options to improve disease control, with a focus on our prior efforts and experience in this field. [ABSTRACT FROM AUTHOR]

Purpose: Low-grade glioma is the most common brain tumor among children and adolescents. When these tumors arise in the temporal lobe, patients frequently present with seizures that are poorly controlled with antiepileptic drugs. Here we summarize the clinical features, pathophysiology, preoperative evaluation, surgical treatment, and outcomes of pediatric patients with low-grade gliomas in the temporal lobe. Methods: We reviewed the literature on pediatric low-grade gliomas in the temporal lobe, focusing on cohort studies and systematic reviews that described surgical treatment strategies and reported both oncologic and epilepsy outcomes. Results: The differential diagnoses of pediatric low-grade gliomas in the temporal lobe include ganglioglioma, dysembryoplastic neuroepithelial tumor, desmoplastic infantile ganglioglioma, papillary glioneuronal tumor, pilocytic astrocytoma, pleomorphic xanthoastrocytoma, angiocentric glioma, and polymorphous low-grade neuroepithelial tumor of the young. There is no consensus on the optimal surgical approach for these tumors: lesionectomy alone, or extended lesionectomy with anterior temporal lobectomy, with or without removal of mesial temporal structures. Gross total resection and shorter preoperative duration of epilepsy are strongly associated with favorable seizure outcomes, defined as Engel Class I or Class II, approaching 90% in most series. The risk of surgical complications ranges from 4 to 17%, outweighing the lifetime risks of medically refractory epilepsy. Conclusion: Pediatric patients with temporal low-grade glioma and tumor-related epilepsy are best managed by a multidisciplinary epilepsy surgery team. Early and appropriate surgery leads to prolonged survival and a greater likelihood of seizure freedom, improving their overall quality of life. [ABSTRACT FROM AUTHOR]

Pediatric-type low-grade glioma (PLGG) encompasses a heterogeneous group of WHO grade 1 or 2 tumors and is the most common central nervous system tumor found in children. PLGG extends beyond pediatrics, into adolescents and young adults (AYA, ages 15–40). PLGG represents 25% of all gliomas diagnosed in AYA with differences in tumor location and molecular alterations compared to children, resulting in improved outcome for AYAs. Long-term outcome is excellent, though patients may suffer significant morbidity depending on tumor location. There are differences in treatment practices with radiation used to treat PLGG in AYAs more often than in children. Most PLGG in AYA harbor an alteration in the RAS/MAPK pathway, with limited insight into response to targeted therapy in this age group. This review discusses the epidemiology, current therapeutic approaches, and challenges in the management of PLGG in AYA. [ABSTRACT FROM AUTHOR]

Neurofibromatosis type 1 (NF1) is an autosomal dominant cancer predisposition syndrome characterized by the development of both central and peripheral nervous system tumors. Low-grade glioma (LGG) is the most prevalent central nervous system tumor occurring in children with NF1, arising most frequently within the optic pathway, followed by the brainstem. Historically, treatment of NF1-LGG has been limited to conventional cytotoxic chemotherapy and surgery. Despite treatment with chemotherapy, a subset of children with NF1-LGG fail initial therapy, have a continued decline in function, or recur. The recent development of several preclinical models has allowed for the identification of novel, molecularly targeted therapies. At present, exploration of these novel precision-based therapies is ongoing in the preclinical setting and through larger, collaborative clinical trials. Herein, we review the approach to surveillance and management of NF1-LGG in children and discuss upcoming novel therapies and treatment protocols. [ABSTRACT FROM AUTHOR]

This study aimed to determine the predictive power of religious coping of parents of children with cancer on caregiver burden, depression, anxiety, and stress in Turkey. It was designed as a descriptive and cross-sectional study, utilizing correlational analysis and regression models to explore associations between variables. Data were collected from 164 parents in the pediatric hematology-oncology clinics of a university hospital between November 2023 and March 2024. There was a negative correlation between caregiver burden score and negative and positive religious coping scores. Caregiver burden scores were positively correlated with depression, anxiety, and stress scores. Results indicated that caregiver burden, education level, employment status, family structure, family income, and age at diagnosis significantly predicted positive religious coping. For negative religious coping, caregiver burden, education level, family structure, and family income were significant predictors. This suggests that religious coping may help reduce caregiver burden, underscoring the importance of promoting constructive coping strategies to support caregivers' well-being. [ABSTRACT FROM AUTHOR]

Simple Summary: Three-dimensional (3D) models enable simplified and controlled in vitro replication of tumor microenvironment features, making them easier to study. In the current work, we developed 3D hydrogels based on gelatin plus silk fibroin with tunable mechanical properties, incorporating vitronectin (a glycoprotein related to tumor aggressiveness) into the scaffolds. Digital analysis was applied to monitor Ewing sarcoma cell line growth and morphology in response to the 3D model composition and different culture times, opening the possibility of its use to study other proteins of interest and tumor types. Our findings highlight the heterogeneity of cell line responses to their microenvironment, underscoring the enormous potential of these hydrogels as mechanodrug-testing platforms and for studying cell protein secretion as an initial step in liquid biopsy biomarker research. Ewing sarcoma (ES) is an aggressive bone and soft-tissue pediatric cancer. High vitronectin (VN) expression has been associated with poor prognosis in other cancers, and we aimed to determine the utility of this extracellular matrix glycoprotein as a biomarker of aggressiveness in ES. Silk fibroin plus gelatin–tyramine hydrogels (HGs) were fabricated with and without cross-linked VN and cultivated with A673 and PDX73 ES cell lines for two and three weeks. VN secretion to culture media was assessed using ELISA. Morphometric analysis was applied for phenotypic characterization. VN release to culture media was higher in 3D models than in monolayer cultures, and intracellular, intercellular, and pericluster presence was also observed. A673-HGs showed lower density of clusters but a proportion of larger clusters than PDX73-HGs, which presented low cluster circularity. The cluster density of A673-HGs without added VN was higher than with added VN and slightly lower in the case of PDX73-HGs. Furthermore, a culture time of three weeks provided no benefits in cluster growth compared to two weeks, especially in A673-HGs. These advances in 3D modeling and digital quantification pave the way for future studies in ES and other cancers to deepen understanding about intra- and intercellular heterogeneity and anti-adhesion VN therapies. [ABSTRACT FROM AUTHOR]

Simple Summary: This research was inspired by a desire to understand how adverse childhood experiences (ACEs) impact depression among cancer survivors. The study's findings revealed that cancer survivors with three or more ACEs had a depression rate of 40.8%, compared to 18.7% among those with one to two ACEs and 10.9% among those with no ACEs. Survivors with three or more ACEs were significantly more likely to report experiencing depression. Among the three ACE subtypes—sexual abuse, physical abuse, and having family members with mental illnesses—cancer survivors with family members suffering from mental illnesses had the highest odds of depression, followed by those who experienced physical abuse. These findings provide a crucial basis for health providers to screen cancer survivors for ACEs and depression, guiding proactive screening and treatment of depression in this vulnerable population. Objectives: Adverse childhood experiences (ACEs) are linked to a heightened risk of depression. We explored the relationship between ACEs and both depression and mental distress among cancer survivors. Methods: This was a cross-sectional analysis using the 2022 Behavioral Risk Factor Surveillance System database of cancer survivors aged ≥18 (n = 14,132). The primary outcome was self-reported history of depression, and the secondary outcome was mental distress. The exposure variable was the number of ACEs, classified as 0, 1–2, and ≥3. Weighted multivariable logistic regression models assessed the association between the number of ACEs and depression and mental distress while adjusting for covariates. Results: Approximately 22% of respondents reported experiencing ≥3 ACEs. The prevalence of depression was 21.8%, and mental distress was 15.4%. Compared with cancer survivors who had experienced 0 ACEs, those who had experienced ≥3 (aOR = 3.94; 95% CI, 3.04–5.10) or 1–2 (aOR = 1.85; 95% CI, 1.47–2.32) ACEs had a higher likelihood of reporting depression. Compared with cancer survivors who had experienced 0 ACEs, those who had experienced ≥3 (aOR = 0.67; 95% CI, 0.48–0.93) had a lower likelihood of reporting mental distress. Conclusions: This study highlights the impact of ACEs on depression in adulthood among cancer survivors. [ABSTRACT FROM AUTHOR]

Background: The primary objective was to measure adherence to clinical practice guideline (CPG) recommendations for fertility preservation (FP) in pediatric cancer patients treated in National Cancer Institute Community Oncology Research Program (NCORP) sites. Secondary objectives were to describe factors such as site size associated with CPG-inconsistent care delivery and cryopreservation completion. Methods: This retrospective, multicenter study included patients 15 to 21 years old with a first cancer diagnosis from January 2014 through December 2015 who were previously enrolled to a Children's Oncology Group (COG) study and received care at a participating NCORP site. Patients were randomly selected from a list generated by the COG for chart review by participating sites. Primary outcome was care delivery that was inconsistent with a strong CPG recommendation on FP, namely discussion and offering of FP options before cancer treatment initiation, as adjudicated centrally by a panel. Results: A total of 129 patients from 25 sites were included. Among these, 48% (62/129) received CPG-inconsistent care. Most CPG-inconsistent care was due to lack of FP discussion documentation (93.5%, 58/62). Small site size, treatment at a pediatric (vs mixed adult/pediatric) site, and female sex were associated with higher odds of CPG-inconsistent care delivery. Conclusions: Newly diagnosed pediatric cancer patients often received CPG-inconsistent care for FP, with disproportionate gaps noted for females, and those treated at smaller or pediatric NCORP sites. The primary reason for CPG-inconsistent care is lack of FP discussion from clinicians. Opportunities to improve FP CPG implementation are highlighted. [ABSTRACT FROM AUTHOR]

Purpose: The purpose of the study was to answer the following questions. What was functional results of pediatric patients receiving a short stem allograft-prosthesis composite of the proximal femur? What was complication rate and revision-free implant survival? Was it possible to preserve the bone stock of the proximal femur in pediatric patients? Methods: We reviewed 10 pediatric patients treated with proximal femur resection for a primary bone tumor and reconstruction with short stem allograft-prosthesis composite, with at least 24 months follow-up. The median age was 9 years (4–13) at surgery. The mean resection length was 15 cm (6–29). In six cases, fixation was performed with a short plate positioned under the great trochanter while in four cases a long plate extended over the great trochanter was employed. Results: Nine complications that required surgical revision were assessed in six patients (one wound dehiscence, two nonunions, two fractures, one acetabular wear, three hypometria), while allograft-prosthesis composite removal was required in three patients. The revision-free survival was 57% (95% confidence interval 33%–100%) at 5 and 10 years. The graft removal-free survival was 75% (95% confidence interval 50%–100%) at 5 and 10 years. The mean Musculo-Skeletal Tumor Society Score was 28 (20–30). Conclusions: Allograft-prosthesis composites with short stem and compression plate represents an effective reconstructive option after proximal femur resection for primary bone tumors in growing patients, preserving bone stock. The use of a compression plate extended over the greater trochanter seemed to reduce failure rate. [ABSTRACT FROM AUTHOR]