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Background: To enhance the quality of care available for children with central nervous system (CNS) tumors across the world, a systematic evaluation of capacity is needed to identify gaps and prioritize interventions. To that end, we created the pediatric neuro-oncology (PNO) resource assessment aid (PANORAMA) tool., Methods: The development of PANORAMA encompassed 3 phases: operationalization, consensus building, and piloting. PANORAMA aimed to capture the elements of the PNO care continuum through domains with weighted assessments reflecting their importance. Responses were ordinally scored to reflect the level of satisfaction. PANORAMA was revised based on feedback at various phases to improve its relevance, usability, and clarity., Results: The operationalization phase identified 14 domains by using 252 questions. The consensus phase involved 15 experts (6 pediatric oncologists, 3 radiation oncologists, 2 neurosurgeons, 2 radiologists, and 2 pathologists). The consensus phase validated the identified domains, questions, and scoring methodology. The PANORAMA domains included national context, hospital infrastructure, organization and service integration, human resources, financing, laboratory, neurosurgery, diagnostic imaging, pathology, chemotherapy, radiotherapy, supportive care, and patient outcomes. PANORAMA was piloted at 13 institutions in 12 countries, representing diverse patient care contexts. Face validity was assessed by examining the correlation between the estimated score by respondents and calculated PANORAMA scores for each domain ( r  = 0.67, P  < .0001)., Conclusions: PANORAMA was developed through a systematic, collaborative approach, ensuring its relevance to evaluate core elements of PNO service capacity. Distribution of PANORAMA will enable quantitative service evaluations across institutions, facilitating benchmarking and the prioritization of interventions., Competing Interests: The authors declare no competing interests., (© The Author(s) 2024. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Background: Pediatric-type diffuse low-grade gliomas (pLGG) harboring recurrent genetic alterations involving MYB or MYBL1 are closely related tumors. Detailed treatment and outcome data of large cohorts are still limited. This study aimed to comprehensively evaluate pLGG with these alterations to define optimal therapeutic strategies., Methods: We retrospectively reviewed details of pLGG with MYB or MYBL1 alterations from patients treated or referred for pathologic review at St. Jude Children's Research Hospital. Tumor specimens were centrally reviewed, and clinical data were collated., Results: Thirty-three patients (18 male; median age, 5 years) were identified. Two tumors had MYBL1 alterations; 31 had MYB alterations, MYB::QKI fusion being the most common (n = 10, 30%). Most tumors were in the cerebral hemispheres (n = 22, 67%). Two patients (6%) had metastasis at diagnosis. The median follow-up was 6.1 years. The 5-year event-free survival (EFS) rate was 81.3% ± 8.3%; the 5-year overall survival (OS) rate was 96.4% ± 4.1%. Patients receiving a near-total or gross-total resection had a 5-year EFS of 100%; those receiving a biopsy or subtotal resection had a 5-year EFS rate of 56.6% ± 15.2% (P < .01). No difference in EFS was observed based on location, histology, or molecular alterations. However, the tumors that progressed or metastasized may have distinct methylation profiles with evidence of activation of the MAPK and PI3K/AKT/mTOR pathways., Conclusions: pLGG with MYB/MYBL1 alterations have good outcomes. Our findings suggest that surgical resectability is a crucial determinant of EFS. Further characterization is required to identify optimal treatment strategies for progressive tumors., (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.)

Background: [ 11 C]-Methionine positron emission tomography (PET; [ 11 C]-MET-PET) is principally used for the evaluation of brain tumors in adults. Although amino acid PET tracers are more commonly used in the evaluation of pediatric brain tumors, data on [ 11 C]-MET-PET imaging of pediatric low-grade gliomas (pLGG) is scarce. This study aimed to investigate the roles of [ 11 C]-MET-PET in the evaluation of pLGGs., Methods: Eighteen patients with newly diagnosed pLGG and 26 previously treated pLGG patients underwent [ 11 C]-MET-PET met the inclusion and exclusion criteria. Tumor-to-brain uptake ratio (TBR) and metabolic tumor volumes were assessed for diagnostic performances (newly diagnosed, 15; previously treated 26), change with therapy (newly diagnosed, 9; previously treated 7), and variability among different histology ( n  = 12) and molecular markers ( n  = 7) of pLGGs., Results: The sensitivity of [ 11 C]-MET-PET for diagnosing pLGG, newly diagnosed, and previously treated combined was 93% for both TBR max and TBR peak , 76% for TBR mean , and 95% for qualitative evaluation. TBR max showed a statistically significant reduction after treatment, while other PET parameters showed a tendency to decrease. Median TBR max , TBR peak , and TBR mean values were slightly higher in the BRAFV600E mutated tumors compared to the BRAF fused tumors. Median TBR max , and TBR peak in diffuse astrocytomas were higher compared to pilocytic astrocytomas, but median TBR mean , was slightly higher in pilocytic astrocytomas. However, formal statistical analysis was not done due to the small sample size., Conclusions: Our study shows that [ 11 C]-MET-PET reliably characterizes new and previously treated pLGGs. Our study also shows that quantitative parameters tend to decrease with treatment, and differences may exist between various pLGG types., Competing Interests: Financial disclosure: No potential conflicts of interest relevant to this article exist. Disclaimer: The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health., (© The Author(s) 2024. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.)

Purpose: Central nervous system (CNS) tumors are the most common solid malignancies in children worldwide, including in Armenia. The current study aims to analyze epidemiological data, treatment, and outcomes of children and young adults (≤25 years) with CNS tumors in Armenia during the last 26 years., Methods: We collected data from pediatric and young adult patients treated in selected sites in Armenia from 1 st January 1995 to 31 st December 2020. Incidence by sex, age at diagnosis, time from first complaints to diagnosis, histopathology results, treatment strategies, complications, and overall survival (OS) rates were calculated., Results: The multicenter data analysis revealed 149 patients with diagnosed primary CNS tumors over 26 years. Among them, 84 (56.4%) were male. The median age at diagnosis was 7 years (range, 3 months to 25 years), and the median time from the first complaints to diagnosis was 2 months (range, 1 week to 70 months). Medulloblastomas and other embryonal tumors (47), low-grade gliomas (32), and high-grade gliomas (22) were the most commonly diagnosed malignancies. Ependymomas, craniopharyngiomas, germ cell tumors, and other malignancies were observed in 22 patients. For 26 patients, no histopathological or radiological diagnosis was available. Follow-up information was available for 98 (65.8%) patients. The 5-year OS rate for the whole study group was 67.7%., Conclusion: Consistent with international data, embryonal tumors, and gliomas were the most commonly diagnosed CNS malignancies in Armenia. Multimodal treatment was often not available in Armenia during the study period, especially for early cases., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Smoking remains a significant risk factor for numerous health issues, including lung cancer, chronic obstructive pulmonary disease, ischemic heart disease, stroke, and respiratory infections. This study investigates the burden of tobacco-related diseases in the Middle East and North Africa (MENA) region., Methods: Utilizing the GBD data, we examined the risk of smoking and second-hand smoke exposure and their related causes of death and disability in the 22 MENA countries. Smoking prevalence and disease burden data were analyzed with estimates reported as age-standardized rates., Results: Tobacco abuse accounted for 14.5% of all deaths and 23.2% of deaths tied to known risk factors, with an age-standardized death rate of 110.8 per 100,000. Cardiovascular diseases were the primary cause of smoking-related deaths and DALYs, representing 53.4% of all deaths and 50.3% of all DALYs. This was followed by neoplasms (24.6% of all deaths and 20.3% of all DALYs), chronic respiratory diseases(12.4% of all deaths and 11.9% of all DALYs), and respiratory infections and tuberculosis(4% of all deaths and 3.4% of all DALYs). Second-hand smoking caused 20.5% of tobacco-related deaths and 21.5% of tobacco-related DALYs, disproportionately affecting younger individuals. An increasing disease burden was observed in Lebanon, Turkey, Syria, Tunisia, UAE, and Libya, and declining rates were most evident in Oman and Qatar., Conclusion: Our study emphasizes the impact of smoking on cardiovascular disease, the primary cause of smoking-related mortality and morbidity in the MENA region. Our findings highlight the urgent need for effective tobacco control policies and interventions.

Background: High-grade gliomas (HGG) in young children pose a challenge due to favorable but unpredictable outcomes. While retrospective studies broadened our understanding of tumor biology, prospective data is lacking., Methods: A cohort of children with histologically diagnosed HGG from the SJYC07 trial was augmented with nonprotocol patients with HGG treated at St. Jude Children's Research Hospital from November 2007 to December 2020. DNA methylome profiling and whole genome, whole exome, and RNA sequencing were performed. These data were integrated with histopathology to yield an integrated diagnosis. Clinical characteristics and preoperative imaging were analyzed., Results: Fifty-six children (0.0-4.4 years) were identified. Integrated analysis split the cohort into four categories: infant-type hemispheric glioma (IHG), HGG, low-grade glioma (LGG), and other-central nervous system (CNS) tumors. IHG was the most prevalent (n = 22), occurred in the youngest patients (median age = 0.4 years), and commonly harbored receptor tyrosine kinase gene fusions (7 ALK, 2 ROS1, 3 NTRK1/2/3, 4 MET). The 5-year event-free (EFS) and overall survival (OS) for IHG was 53.13% (95%CI: 35.52-79.47) and 90.91% (95%CI: 79.66-100.00) vs. 0.0% and 16.67% (95%CI: 2.78-99.74%) for HGG (p = 0.0043, p = 0.00013). EFS and OS were not different between IHG and LGG (p = 0.95, p = 0.43). Imaging review showed IHGs are associated with circumscribed margins (p = 0.0047), hemispheric location (p = 0.0010), and intratumoral hemorrhage (p = 0.0149)., Conclusions: HGG in young children is heterogeneous and best defined by integrating histopathological and molecular features. Patients with IHG have relatively good outcomes, yet they endure significant deficits, making them good candidates for therapy de-escalation and trials of molecular targeted therapy., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)