bluuebunny/crossref_metadata_2025_split huggingface dataset:
QOL-32. COGNITIVE AND PSYCHOLOGICAL LATE EFFECTS IN CHILDREN AND ADOLESCENTS WITH PINEAL REGION TUMORS: A 35-YEAR-LONG EXPERIENCE IN A TERTIARY REFERRAL HOSPITAL
Authors: Verrico Antonio , Pisati Angelica , Bagnasco Francesca , De Giuseppe Sara , Rossi Andrea , Piccolo Gianluca , Molteni Marta , Mercuri Claudia , Consales Alessandro , Barra Salvina , Giannelli Flavio , Satragno Camilla , Garrè Maria Luisa , Milanaccio Claudia , Profio Sonia Di 
Published: June 2024

Abstract

BACKGROUND
Pineal region tumors (PRT) account for 2.8% of all central nervous system tumors in children and adolescents up to 19 years of age. Modern therapies allow more than 2/3 of patients to overcome their disease, but late effects impair quality of life of survivors. This study aimed to define the prevalence of cognitive and psychological late effects in PRT-survivors.

METHODS
Demographic information, medical variables, cognitive and psychological age-appropriated assessments of childhood and adolescence PRT-survivors, followed-up at Gaslini Children’s Hospital between 1988 and 2023, were retrospectively collected. Neurocognitive assessment was performed using the Wechsler scale. Psychological assessment was obtained using the Child Behavior Checklist, Beck Depression Inventory, State-Trait Anxiety Inventory, Trauma Symptom Checklist for children and Impact of Event Scale–Revised.

RESULTS
Sixty patients were included (53 males, 88%); median age at diagnosis was 12.8 years (Interquartile range, IQR, 9.0–15.7 years). Main entities were: germinoma (17, 28%), non-germinomatous germ cell tumor (16, 27%), bifocal germinoma (13, 22%), pineoblastoma (6, 10%), teratoma (3, 5%). 20/60 patients (33%) had neuroaxis spread, 48/60 (80%) presented hydrocephalus, 41/60 (68%) underwent surgery, 16/60 (27%) needed ventricular-peritoneal shunt, 51/60 (86%) received chemotherapy and 56/60 (93%) required radiotherapy. Median follow-up was 8.9 years (IQR 4.1–15.2). Six patients died, 5/6 died for progressive disease. Intelligence quotient (IQ) score was available for 43/60 patients with a median value of 91 (IQR 85-107). Complete cognitive and neuropsychological assessment was available for 25/60 patients showing presence of: anxiety symptoms (16/25, 64%), depression symptoms (20/25, 80%) and post-traumatic symptoms (7/25, 28%).

CONCLUSIONS
In our cohort, PRT-survivors showed IQ scores within expected average and a high frequency of anxiety and depression symptoms. Deeper insights into the mechanisms underlying cognitive and psychological late effects are needed, focusing on the role of demographics, treatments and disease-specific variables as well as on tumor location.
QOL-43. COMPARISON OF SOCIAL DETERMINANTS OF HEALTH MEASURES ON IQ AND ACADEMIC OUTCOMES IN PEDIATRIC BRAIN TUMORS
Authors: Gioia Anthony R , Jacobson Lisa A , Acharya Sahaja , Peterson Rachel K 
Published: June 2024

Abstract

BACKGROUND
Treatment for pediatric brain tumors impacts IQ and academic achievement; fewer studies have examined the role of social determinants of health (SDoH). To date, studies have primarily focused on family-based SDoH such as insurance type or parental education as predictors of cognition. Limited studies have examined community factors, though our work highlighted the role of education quality on IQ and academics in pediatric brain tumors. To our knowledge, no study has examined the predictive value of family and community-based SDoH.

METHODS
Participants included 151 pediatric brain tumor patients (M age at diagnosis=8.82) seen for a clinical neuropsychological evaluation (M age at evaluation=13.71). IQ was obtained from age-appropriate intelligence measures (WISC&gt;6 years, DAS-II&lt;6 years); untimed reading and math calculation were examined via WIAT/KTEA/WJ subtests. Family-based SDoH included insurance type (public, private) and maternal education (&lt;high school degree, some college, &gt;college degree). Community-based SDoH included the Area Deprivation Index (ADI) and Education, Health-Environment, and Social-Economic indices from the Childhood Opportunity Index (COI). Multiple regressions examined variance in IQ, reading, and math accounted for by each of the SDoH measures, after controlling for treatment exposures using the Neurological Predictor Scale (NPS).

RESULTS
Insurance [β=-.302, t(151)=-4.00; p&lt;001] and Education [β=.235, t(151)=3.14; p=.002] accounted for 14.9% and 19.7% of the IQ variance, respectively. Education also accounted for 12.8% of the reading variance (β=.304, t(109)=3.40; p&lt;.001). Both the Education [β=.570, t(107)=3.41; p&lt;.001] and Social-Economic [β=-.381, t(107)=-2.28; p=.03] indices accounted for 7.7% and 11.3% of the math calculation variance, respectively.

CONCLUSIONS
Community, particularly those related to education and social & economic resources, and family factors (e.g., insurance status) were associated with IQ and academic performance. Findings indicate the importance of investigating both family and community factors that may contribute to outcomes in pediatric brain tumors.
QOL-44. EARLY SEVERE HEARING LOSS IN CANADIAN CHILDREN WITH CENTRAL NERVOUS SYSTEM TUMORS: A POPULATION-BASED COHORT STUDY
Authors: Coltin Hallie , Trudeau-Ferrin Ofélie , Perreault Sébastien , Lafay-Cousin Lucie , Renzi Samuele , Larouche Valérie , Erker Craig , Bouffet Eric , Ramaswamy Vijay 
Published: June 2024

Abstract

BACKGROUND
Severe treatment-related hearing loss (HL) is associated with devastating psychosocial/neurocognitive outcomes. Children with central nervous system (CNS) tumors are highly vulnerable to severe HL due to tumor location and treatment exposures. This is the first study to report national CNS tumor-specific rates and predictors of early severe HL.

METHODS
We conducted a retrospective, population-based cohort study of all children ≤15yrs at diagnosis with CNS tumors between 2001-2019 using the Cancer in Young People in Canada registry. The primary outcome was Common Terminology Criteria for Adverse Events grade 3/4 HL ≤5yrs following diagnosis. We determined prevalence and probabilities of early HL; predictors were determined using multivariable logistic regression models.

RESULTS
Among 3,201 children with CNS tumors, 5.1% experienced early HL. Children with medulloblastoma (N=570) and ATRT/other embryonal tumors (N=269) experienced disproportionate rates (16.1%, 15.2%, respectively); 85.4% (medulloblastoma) and 68.8% (ATRT/other embryonal) received cisplatin, 74.6% (medulloblastoma) and 52.1% (ATRT/other embryonal) were irradiated. In children with medulloblastoma, when controlling for follow-up, age &lt;=5yrs at diagnosis [odds ratio (OR) 2.4, 1.5-3.8], radiation (OR 3.5, 1.6-7.6), and cisplatin (OR 20.4, 1.3-329.7) predicted early HL. In children with medulloblastoma post cisplatin, age ≤5yrs at diagnosis doubled the probability of early HL in both irradiated (29.8%, vs 15.3% if &gt;5yrs at diagnosis) and non-irradiated patients (10.6% vs 4.8% if &gt;5yrs at diagnosis). In children with ATRT/other embryonal, cisplatin (OR 28.2, 1.7-472.1) was the only predictor of early HL.

CONCLUSIONS
In the largest population-based study of early severe HL in children with CNS tumors, we report striking rates in children with embryonal tumors. Younger irradiated children had disproportionate probabilities of early HL, suggesting a novel additive interaction between age at diagnosis and radiation. Standardized otoprotection and research focusing on therapy de-escalation in young children with embryonal tumors are urgently needed.
QOL-52. SOCIOECONOMIC METRICS AND THEIR UNIQUE ROLE IN PREDICTING COGNITIVE AND FUNCTIONAL MORBIDITY IN RACIALLY AND ETHNICALLY DIVERSE PATIENTS WITH PEDIATRIC BRAIN TUMOR (PBT)
Authors: Chiang Jenna A , Mendoza Leanne , Whitaker Ashley M 
Published: June 2024

Abstract

BACKGROUND
Caregivers from more advantaged backgrounds have better understanding of their child’s needs, access to resources, and medical adherence, contributing to higher survival and neuropsychological (NP) outcomes in PBT, above and beyond diagnostic/treatment-related factors. Children with PBT from disadvantaged neighborhoods have increased medical morbidity, though cognitive morbidity is unknown. This study examines area deprivation index (ADI) quintiles (neighborhood disadvantage) in relation to established proxies for socioeconomic status (SES) and NP outcomes following PBT diagnosis.

METHODS
Parental education and median household income (SES proxies), as well as ADI quintiles (1-5, 5=most disadvantaged) were estimated for 120 PBT patients (ages 5-21; x̄=13.6 years, SD=3.8 years; 50% female). NP outcomes included estimated IQ (calculated from Wechsler Vocabulary/Matrix Reasoning) and/or the Adaptive Behavior Assessment System-Third Edition (ABAS-3).

RESULTS
Over 75% identified as minorities (53% Latinx, 10% Asian American, 5% Black, 8% multi/other). Findings revealed significant racial/ethnic disparities in household income [F(4,115)=11.208, p&lt;.001] and ADI [F(4,108)=9.663, p&lt;.001]. Parental education was correlated with household income [rs(80)=.490, p&lt;.001] and ADI [rs(76)=-.477, p&lt;.001]. Interestingly, household income [F(1,74)=7.498, p=.008] and parental education [F(1,49)=10.084, p=.003] significantly predicted IQ (SS=44-132; x̄=99.78, SD=14.66), while ADI did not. Neither SES proxy nor ADI predicted adaptive functioning (SS=51-120; x̄=90.12, SD=13.927), despite the relationship between IQ and adaptive functioning, r(47)=.273, p=.032.

CONCLUSIONS
The predictive value of household income (which varies by race/ethnicity) and parental education on cognition following PBT diagnosis continues to inform neuropsychological practice. While ADI is correlated with both established SES proxies, higher neighborhood disadvantage does not predict cognitive concerns in the same way, indicating potential unique housing factors (e.g., persons per room ratio) that may protect against cognitive morbidity (e.g., multigenerational living). Additional investigation is needed to understand why adaptive functioning is less susceptible to SES/ADI than cognition.
NURS-18. GERMINOMA PATIENTS – MORE PRONE TO PSYCHIATRIC DISORDERS, ALLERGIES, AND WEIGHT PROBLEMS? AN INSTITUTIONAL REVIEW
Authors: McKeown Tara , Chun Foo Jen , Li Amanda , Dutton Jordann , Rowe Rachel , Hawkins Cynthia , Janzen Laura , Bartels Ute 
Published: June 2024

Abstract

BACKGROUND
Germinoma are the most frequent type of intracranial germ cell tumour. Within nursing clinical practice an increased prevalence of psychiatric manifestations, allergies, and weight gain throughout treatment and into survivorship within the germinoma population has been observed. There is only limited literature about these problems available.

METHODS
A retrospective review of clinical presentation, psychiatric co-morbidity, therapeutic course, and follow-up of 27 Germinoma patients (6.2-17 years) diagnosed and treated at a tertiary care center from 2008-2021 was performed. In addition, the presence and occurrence of allergies and weight changes throughout their therapy was reviewed.

RESULTS
Nearly half of the Germinoma patients (n=11) (suprasellar, pineal, basal ganglia) were diagnosed with pre-existing psychiatric co-morbidity (anxiety, depression, mood disorder and eating disorder) as per DSM-5 criteria, more commonly verified after their brain tumour diagnosis and persistent beyond treatment. Three of 27 (11%) patients indicated allergies (medication, food) at diagnosis. Seventeen of the 27 (63%) patients presented with allergies throughout their therapy (medication, tape, cleaning products, blood products). Six (35%) of those patients developed anaphylactic reaction to Etoposide and/or blood products. Eight of the patients (suprasellar & pineal) were found to have a significant weight gain throughout their treatment and two (pineal & suprasellar) were found to have significant weight loss, the rest of the cohort remained stable. The impact of tumour location (suprasellar, pineal) on the specific problems is ongoing.

CONCLUSION
The findings of this retrospective review could guide clinical practice. The potential higher incidence of psychiatric disorders should prompt practitioners to upfront screening and throughout therapy to ensure adequate support. Allergy prevalence in pediatrics is variable but an allergy occurrence of 35% is elevated in comparison to the general population and therefore increased awareness of allergies within this population is important for the bedside nurse. Lastly, early involvement of dieticians to aid in weight management seems crucial.
OTHR-10. PILOT STUDY TO EVALUATE AUTOMATED LABORATORY DATA TRANSFER INTO MEDIDATA RAVE ACROSS THE NATIONAL CANCER INSTITUTE (NCI)-SUPPORTED PEDIATRIC BRAIN TUMOR CONSORTIUM (PBTC) AND PEDIATRIC EARLY PHASE CLINICAL TRIAL NETWORK (PEP-CTN) COOPERATIVE GROUPS
Authors: Miler Tamara P , Aplenc Richard , Minard Charles , Lensing Shelly , Wang Yu , Velamuru Prasanna , Foster Jennifer H , Silverman Milena , Sung Cheryl , Wong Karina , Beeles Thalia , Weigel Brenda , Fox Elizabeth , Onar-Thomas Arzu 
Published: June 2024

Abstract

BACKGROUND
Electronic data capture (EDC) has the potential to improve data accuracy and efficiency in oncology. The PBTC and PEP-CTN cooperative groups conducted a pilot project to 1) assess the feasibility of automated transfer of laboratory data from participating institutions’ electronic health records (EHR) to the Medidata Rave EDC housed within each consortium’s Uniform Resource Locator (URL); 2) evaluate discrepancies between retrospective automated laboratory data extraction from the EHR and manual data ascertainment during the original conduct of the study (MANUAL).

METHODS
Data from patients treated on 7 completed PBTC or PEP-CTN trials at 7 hospitals were included. Each consortium created a Rave instance to receive data. Sites elected to use local methods or the ExtractEHR R package to map and extract laboratory data for designated patients. Post-extraction data were uploaded to the Rave Batch Uploader (BU)-associated file transfer protocol (FTP) and BU uploaded data into Rave. After successful upload, discrepancies between EHR and MANUAL data were categorized. Two hospitals reviewed discrepancies at their sites to identify reasons for discrepancies.

RESULTS
Automated extraction and upload required significant PBTC/PEP-CTN operational effort and close work with hospital technical teams to map and extract data. All sites successfully extracted specified laboratory data and had ≥1 complete upload via BU to Rave; 2 sites had 2 uploads. Discrepancies were most commonly due to more data in EHR datasets (&gt;1 result on a given day, data on additional study window days) or mismatched values or units. Differences in percentages of discrepant results between hospitals were likely due to mapping differences for EHR extraction.

CONCLUSIONS
This study demonstrated that in the pediatric cooperative oncology group setting automated laboratory data ascertainment can be performed. Accomplishing this required upfront central operational and hospital-based effort to prepare for data upload and transfer. This effort varied by site.
OTHR-16. HYPERACTIVATION OF THE INTEGRATED STRESS RESPONSE VIA PROTEASOME INHIBITION KILLS DMG AND ATRT WHILE SPARING NORMAL IPSC BRAIN ORGANOIDS
Authors: Novak Orlandi V , Wu Alyssa , Eberhart Charles , Raabe Eric H 
Published: June 2024

Abstract

BACKGROUND
H3K27-altered diffuse midline glioma (DMG) and atypical teratoid/rhabdoid tumor (ATRT) have increased baseline activation of the integrated stress response (ISR), an evolutionarily conserved system that enables cells to tolerate stress. This activation is manifested by elevated levels of ATF4. Transient or low-level expression protects cells from stress, while persistent, high-level activation leads to cell death. Ixazomib is an orally bioavailable proteasome inhibitor that causes endoplasmic reticulum stress, which is a major upstream activator of the ISR. We hypothesized that the high baseline level of ATF4 in DMG and ATRT would make these tumors susceptible to ISR activators such as ixazomib.

METHODS
After determining the IC50, DMG and ATRT cell lines were treated with increasing concentrations of ixazomib. To evaluate if ixazomib selectively kills DMG and ATRT, we treated iPSC brain organoids in parallel with tumor cells. Combination treatment of ixazomib and idarubicin, a brain-penetrant anthracycline, was conducted to assess synergy.

RESULTS
Ixazomib significantly induced apoptosis and suppressed proliferation in multiple cell lines, as shown by western blots for cleaved PARP and phospho-Rb expression as well as cleaved caspase-3 and BrdU immunofluorescence (DMSO vs. 25nM p&lt;0.0001 by ANOVA). Additionally, western blots detected elevated ATF4 and CHOP expression in ixazomib treated cells, indicating activation of the ISR. CC3 immunofluorescence and western blot for cleaved PARP showed significantly increased apoptosis in DMG and ATRT cell lines treated with ixazomib (JHHDIPG1 and CHLA06 – DMSO vs. 25nM p&lt;0.0001 by ANOVA), while iPSC brain organoids were not affected. Synergy testing of ixazomib and idarubicin showed an overall zero-interaction-potency (ZIP) synergy score of 23.464 in CHLA06 and 19.084 in HSJD007 (scores of 10 or above indicate synergy).

CONCLUSIONS
By over-activating the ISR, ixazomib kills DMG and ATRT cells without impacting normal iPSC brain organoids. This effect may potentially be enhanced with the addition of idarubicin.
OTHR-18. IMPACT OF COVID-19 ON CLINICAL TRIAL ENROLLMENT IN THE DEPARTMENT OF PEDIATRIC HEMATOLOGY ONCOLOGY AT WASHINGTON UNIVERSITY SCHOOL OF MEDICINE/ST. LOUIS CHILDREN’S HOSPITAL FROM 2017 TO 2023
Authors: Roemerman Heather , Abdelbaki Mohamed , Barnwell Alison , Jones Sally , Hollingsworth Meili , Murray Lisa , Tackett Brittany 
Published: June 2024

Abstract

BACKGROUND
COVID-19 had a significant impact on clinical research within the healthcare system worldwide. However, there are only limited reports on its effects on clinical trials in pediatric cancer, specifically in brain tumors. We reviewed the impact of COVID-19 in the Department of Hematology and Oncology in St. Louis Children’s Hospital (SLCH). We analyzed the number of clinical trials open at our center from 2017 to 2023. We hypothesized that in 2020 there would be a significant drop in study participant enrollments at our site overall.

METHODS
We analyzed data from our online tracking system from 2017 to 2019, pre-pandemic, and 2020-2023, post pandemic. We will also perform a literature review of the impact COVID-19 had on clinical trial enrollment involving childhood cancers in the United States. We will compare these findings to our experience at SLCH.

RESULTS
In 2020, while non-therapeutic trial enrollments declined to about half of all enrollments compared to 2019, enrollments in therapeutic trials significantly increased to almost three-fold. Overall, when combining non-therapeutic and therapeutic enrollments from 2017-2020, there was a significant decrease in 2020. By 2023, total enrollments nearly recovered to pre-pandemic levels.

CONCLUSIONS
COVID-19 negatively impacted non-therapeutic studies at SLCH as many trials permanently closed; however, the negative effect on therapeutic studies was minimal.
OTHR-23. PATIENT DESIRE TO SHARE DATA AND PARTICIPATE IN RESEARCH IN THE DIPG/DMG COMMUNITY
Authors: Lyons Nicole , Ward Lisa 
Published: June 2024

Abstract
Direct and reciprocal communication between patient communities and their research and care teams is essential to building trust and ensuring that all essential information is shared. Patient participation in data collection of any sort is often challenging, as patients may be hesitant or unmotivated to share their information. The DIPG/DMG patient community faces the opposite challenge. Because of the frustration of the lack of treatment options and the established trust that exists between patient families and research professionals, DIPG/DMG patient families are both willing and eager to share any information they can. Patient families have seen how their knowledge has helped other families navigate impossible situations and care teams understand how to better support their patients. The information families eagerly provide is crucial to advancing treatment and research. Patient families have repeatedly expressed willingness to participate in quality of life assessments, repeated surveys during and after treatment, and provide their medical and biological information in the hopes that it can aid research. There is a need for a centralized manner for all of this data to be shared by the patient and accessed by care teams and researchers. Patient-led initiatives are addressing this by creating a network of resources. This network helps to guide other patient families through the data-sharing process, address concerns, and find ways to provide what is needed to push research and treatments ahead. There are many benefits to having a centralized data ecosystem for researchers and care teams, such as standardizing data collection and ease of analysis. In the future, this centralized hub could inform personalized care, making options such as n-of-1 trials and matching patients to optimal clinical trials possible in a manner that is effective and efficient.
LMIC-16. PRE-IRRADIATION CHEMOTHERAPY AND DELAYED RADIATION THERAPY IN INFANTS AND YOUNG CHILDREN WITH WITH CENTRAL NERVOUS SYSTEM EMBRYONAL TUMOURS
Authors: Prasad Maya , Gollamudi Venkata Ramamohan , Dasgupta Archya , Chatterji Abhishek , Sahay Ayushi , Singh Vikas , Shetty Prakash , Moiyadi Aliasgar , Sahu Arpita , Chaudhuri Amit , Bhattacharya Kajari , Epari Sridhar , Gupta Tejpal , Chinnaswamy Girish 
Published: June 2024

Abstract

BACKGROUND
Established strategies for infants and young children(IYC) with embryonal tumours of the central nervous system(CNSET) - autologous stem-cell rescue(ASCT) and intraventricular chemotherapy(IVCT) – are not universally available. We report the outcomes of IYC with CNSET treated using the strategy of surgery, pre-irradiation chemotherapy and delayed radiation.

METHODS
Children &lt;3years diagnosed with CNSET- medulloblastoma(MB), atypical teratoid/rhabdoid tumour(ATRT), embryonal tumour with multilayered rosettes(ETMR), CNS primitive neuroectodermal tumours(PNET)- and pineoblastoma(PB),underwent standard evaluation and staging, maximal safe excision, pre-irradiation chemotherapy(cyclophosphamide, etoposide and carboplatin;CEJ) upto 36months of age and either ris-adapted craniospinal with boost or focal irradiation. We performed a retrospective intention-to-treat outcome analysis.

RESULTS
Of 117children &lt;3years with CNSET treated January2011- December2022, 72 were treated with this strategy.The median age was 25months (range6-35), 49males,histology medulloblastoma-42(58%), ATRT-9(12.5%), PNET-8(11.1%), ETMR-8(11.1%) and PB-5(6.8%); 23(32%) metastatic.The median chemotherapy cycles were 6(range 1-15). 36 children went on to receive RT; CSI in 33 (20standard-dose,13reduced-dose) and focal in 3. At last follow-up, 27children are in remission, 43 have relapsed/progressed (9 received salvage treatment) and 2 died due to chemotherapy toxicity. The 3-year and 5-year event-free-survival(EFS) are 35% and 3-year and 5-year overall-survival(OS) are 41.8% and 40.2%. The 3-year EFS/OS for MB are 51.1%/ 62.8%,PNET 25%/25%,ATRT 11.1%/11.1%,ETMR 12.5%/12.5% and PB 0%. Only metastasis was prognostic in medulloblastoma (HR 2;95% CI 0.86-4.6 for EFS and HR 1.7;95% CI 0.67-4.5 for OS); age, molecular group and histology were not prognostic. Long-term follow-up (n=17) documented &gt;grade2 late effects in all (endocrine-16, neurocognitive-8, vision-3, hearing -8 and neurological-10).

CONCLUSION
The use of moderate-intensity pre-irradiation chemotherapy is feasible and effective in infants and young children with CNSETs. Despite inferior outcomes compared to ASCT or IVCT,this strategy could be used in resource-limited settings.