bluuebunny/crossref_metadata_2025_split huggingface dataset:
QOL-12. CLINICAL SIGNIFICANCE OF RADIATION-INDUCED CEREBROVASULAR DISEASE IN CHILDHOOD BRAIN TUMOR SURVIVORS
Authors: Remes Tiina , Suo-Palosaari Maria , Ritari Niina , Puosi Riina , Koskenkorva Päivi , Sutela Anna , Toiviainen-Salo Sanna-Maria , Arikoski Pekka , Arola Mikko , Hovén Emma , Lähteenmäki Päivi , Lönnqvist Tuula , Riikonen Pekka , Sirkiä Kirsti , Rantala Heikki , Harila Marika , Ojaniemi Marja , Harila-Saari Arja 
Published: December 2020

Abstract

BACKGROUND
Childhood brain tumor survivors have a high risk of early cerebrovascular disease, but currently its clinical significance is unknown.

METHODS
In a nation-wide study, we investigated 68 childhood brain tumor survivors treated with radiotherapy by using magnetic resonance imaging (MRI) and neuropsychological examination after median follow-up time of 20.6 years (range 5.0 – 33.1 years) since radiotherapy. Associations between imaging markers of cerebrovascular disease, white matter hyperintensities and the results of neuropsychological examination were investigated.

RESULTS
Majority (65 %) of the survivors was diagnosed with cerebrovascular disease at median age of 27.1 years (range16.2 – 43.8 years). The presence of imaging markers of cerebrovascular disease or white matter hyperintensities was associated with poorer performance in verbal (VIQ) and performance (PIQ) intelligent quotient, working and semantic memory, executive functions, visuospatial ability, and immediate and general auditive memory (P &lt; 0.05). Survivors with microbleeds performed worse in PIQ, processing speed, executive functions, and visuospatial ability (P &lt;0.05). Lacunar infarcts were associated with difficulties in visuospatial ability (P &lt;0.05). Survivors with white matter hyperintensities in MRI had higher impairment of working and semantic memory, visuospatial ability, and general auditive memory (P &lt; 0.05). Cerebrovascular and small-vessel disease burden associated with poorer neurocognitive performance.

CONCLUSION
The imaging markers of cerebrovascular disease and white matter hyperintensities were related to poorer cognitive performance in radiation-treated long-term survivors of childhood brain tumor. Longitudinal studies are urgently needed to investigate how cerebrovascular disease and related cognitive impairment progress in the survivors.
QOL-18. A LONGITUDINAL STUDY OF NEUROCOGNITION IN CHILDREN TREATED FOR A BRAIN TUMOR
Authors: Lemiere Jurgen , Van den Wyngaert Linde , Vandereydt Josefien , Vandenabeele Karen , Vercruysse Trui , Sleurs Charlotte , Jacobs Sandra 
Published: December 2020

Abstract
It is well known that neurocognition in children treated for a brain tumor can be affected. However, studies on the trajectory of these neurocognitive problems are scarce. In the present study we investigated the evolution of neurocognition between timepoints of diagnosis, 2, 4 and 6 years later. A total of 53 children diagnosed with a brain tumor were recruited in this study, of which all completed a comprehensive neuropsychological test battery at three successive timepoints and 30 at 4 timepoints. The first assessment was conducted as soon as possible after diagnosis and before initiation of chemo- and/or radiotherapy. Mean age at diagnosis was 8.06 years. The most common diagnoses were pilocytic astrocytoma (n=28) and medulloblastoma (n=10). 24.5% and 18.9% of these patient groups received focal or craniospinal irradiation, respectively. A repeated measures analysis with cranial irradiation (no, focal, craniospinal) as between-subjects factor demonstrated a significant interaction effect between time and type of irradiation for overall intelligence (p=0.02) for children with three assessments. The same interaction effect was found for overall intelligence and processing speed for children with four assessments (p=.005 and p=.002, respectively). The group who received craniospinal irradiation demonstrated the most pronounced decline. Interestingly, no main time effect or interaction effect was found for general memory functioning. Our results demonstrate that not all neurocognitive functions in children treated for a brain tumor decline after treatment. Overall IQ and processing speed are the most vulnerable outcomes in our cohort, especially for the children treated with craniospinal irradiation.
QOL-27. SWALLOWING ASSESSMENT IN PEDIATRIC PATIENTS WITH BRAIN TUMOR
Authors: Machado Natalia Oliveira , Duarte Ana Paula , dos Santos Aline Azevedo , Mançano Bruna Minniti , Almeida Carlos 
Published: December 2020

Abstract

BACKGROUND
Neurosurgical intervention is the initial modality of treatment for the vast majority of pediatric brain tumors. However, studies on the swallowing process in pediatric patients with brain tumors are scarce, especially comparing changes that can be identified before and after surgery. In clinical practice, it is possible to observe that these patients may present modifications in the swallowing phases both before and after surgery. Therefore, we conducted a longitudinal study with a cohort of 20 patients ranging in age from 0 to 17 years, in order to characterize the swallowing disorders.

RESULTS
30% of the patients presented some change in orofacial motricity in the organs related to initiation, coordination, and maintenance of swallowing at the time of hospital admission, and 65% of the patients exhibited these changes after surgery. Due to worsening in swallowing after surgery, 40% of the patients required modification of the consistence of oral diet or required the use of an alternative route of feeding.

CONCLUSIONS
There is a high prevalence of swallowing disorders in pediatric patients with brain tumors, mainly regarding the proper functioning of organs related to initiation, coordination, and maintenance of swallowing even before the surgical intervention, and these changes increase after surgery - especially in patients with posterior fossa tumors. The role of the speech/language pathologist is of paramount importance, given their role in the assessment and adequacy of the feeding route, identifying patients at risk of pulmonary aspirations, minimizing swallowing complications, and also facilitating communication with patients and their families.
QOL-31. USE OF PATIENT-REPORTED OUTCOMES TO IDENTIFY YOUTH AT RISK FOR IMPAIRED OVERALL HEALTH
Authors: Ingerski Lisa , Lewis Rebecca Williamson , Mertens Ann , MacDonald Tobey 
Published: December 2020

Abstract
Pediatric brain tumor survivors often experience persistent and clinically significant late-effects following treatment. Critical to understanding morbidity is utilization of patient-reported outcomes (PROs). The current study evaluated PROs of individuals previously diagnosed with a pediatric brain tumor and identified risk factors for less optimal overall health. Participants included 127 youth 10.59±4.81 (M±SD) years old at survey completion and 4.45±3.82 years from diagnosis of a brain tumor (34.6% Pilocytic Astrocytoma, 9.4% Medulloblastoma, 9.4% Ependymoma, 7.9% Craniopharyngioma, 38.6% Other). Outcomes were assessed via Patient-Reported Outcomes Measurement Information System (PROMIS) parent-proxy measures. Overall health was assessed via PROMIS Global Health (i.e., a measure of general, physical, mental, and social health). Univariate and logistic regression analyses examined potential demographic, medical, and psychosocial factors (e.g., age, race, diagnosis, treatment) related to poor global health. Initial descriptive analyses suggested that most youth experienced anxiety symptoms (T-score M±SD=50.71±11.54), depressive symptoms (47.96±10.34), cognitive functioning (46.52±9.10), and fatigue (55.14±10.62) similar to their peers. However, 31.0% of youth experienced impaired global health (T-score&lt;40). After adjusting for other potential covariates, the final model suggested that youth with significant anxiety (OR=6.20, CI=1.56–24.65), youth with significant fatigue (OR=3.96, CI=1.26–12.41), and youth who did not undergo a gross total resection (OR=0.25, CI=0.07–0.96) were at risk for impaired global health. Identifying those at-risk for impaired health is essential to reducing survivor morbidity and optimizing overall quality of life following treatment. Current data suggest potentially modifiable factors that may improve long-term outcomes for survivors of pediatric brain tumors.
QOL-36. USE OF CANNABINOIDS IN THE PEDIATRIC CENTRAL NERVOUS SYSTEM TUMOR POPULATION
Authors: Dorris Kathleen , Channell Jessica , Mettetal Ashley , Hemenway Molly , Briones Natalie , Tran Alexander , Griesinger Andrea , Donson Andrew , Vibhakar Rajeev , Green Adam , Nellan Anandani , Levy Jean Mulcahy , Ambruso Daniel , Foreman Nicholas 
Published: December 2020

Abstract

BACKGROUND
Cannabinoids, including cannabidiol (CBD) and tetrahydrocannabinol (THC), are a class of compounds found in marijuana. Numerous studies in adults have examined cannabinoid use in management of cancer-related symptoms such as nausea, anorexia, and pain. Less is known about the use in the pediatric oncology population.

METHODS
A prospective observational study has been ongoing since 2016 at Children’s Hospital Colorado to evaluate cannabinoids’ impact using PedsQL™ modules on quality of life of pediatric patients with central nervous system (CNS) tumors who are 2–18 years old. Laboratory assessments of T-cell activity and pharmacokinetics of CBD, THC and associated metabolites are in process. Diaries with exploratory information on cannabinoid use patterns are being collected.

RESULTS
Thirty-three patients (14:19; male:female) have been enrolled with a median age of 6.4 years (range, 2.9–17.7 years). The most common tumor type in enrolled patients is embryonal tumors (13/33; 39%). Nine (27%) patients have low-grade glial/glioneuronal tumors, and eight (24%) had high-grade/diffuse midline gliomas. The remaining patients had ependymoma or craniopharyngioma. The median time on cannabinoids is 9 months. Most (n=20) patients have used oral products with CBD and THC. One patient continues on cannabinoid therapy in follow up. Preliminary immune function analyses identified impaired neutrophil superoxide anion production and chemotaxis in patients taking cannabinoids at early time points on therapy.

CONCLUSIONS
Families of children with various CNS tumors are pursuing cannabinoid therapy for both antitumor and supportive care purposes. Analysis of the impact of cannabinoids on patients’ quality of life is ongoing.
QOL-37. USE OF COMPUTERIZED NEUROPSYCHOLOGICAL MEASURES TO ASSESS COGNITIVE MORBIDITY IN CHILDREN UNDERGOING ACTIVE RADIATION THERAPY
Authors: Kais Lorri , Roesser Kellie , Kleman Michelle , Wilkening Greta , Liu Arthur , Hankinson Todd , Foreman Nicholas , Hutaff-Lee Christa 
Published: December 2020

Abstract
Cognitive late effects of brain tumors and related treatments are well-established; however, limited information regarding changes in cognition during radiation therapy (RT) is available. Recent advances in computerized neuropsychological assessments for monitoring of acute and late treatment effects have been developed, though the feasibility of using these tools in a population undergoing active RT has limited empirical evidence. This study investigated performance of pediatric patients with brain tumors actively undergoing RT on the NIH Toolbox (N = 10; M age = 11.29 ± 3.35 years; 86% Caucasian; 86% female). Given significant individual variability, one-sample proportion tests were calculated to assess whether the proportion of patients with performances &gt;1 standard deviation below the mean significantly differed from normative expectations. Of the 12 participants that were enrolled in the study, 10 completed the NIH Toolbox during active RT. Compared to normative expectations, a greater proportion of participants undergoing active RT exhibited deficits on measures of processing speed, working memory, and response inhibition (p=&lt;.01). Differences between participants and normative expectations were not seen on measures of visual memory and vocabulary (p=&gt;.05). Seventy-seven percent of recruited participants completed computerized assessment during active RT, suggesting reasonable feasibility within the small cohort recruited. Consistent with the literature regarding late effects of RT, performance on computerized measures of cognitive functioning mediated by processing speed and aspects of executive functioning were lower for patients undergoing active RT. Further investigation will focus on clarifying the trajectory of deficits across treatment course and comparing computerized measures to traditional neuropsychological measures.
QOL-40. THE IMPACT OF TASK COMPLEXITY ON INFORMATION PROCESSING SPEED AND NEURAL COMMUNICATION IN PAEDIATRIC BRAIN TUMOUR SURVIVORS
Authors: Cox Elizabeth , Atton Juanita , Tseng Julie , Bells Sonya , de Medeiros Cynthia , Laughlin Suzanne , Bouffet Eric , Mabbott Donald J 
Published: December 2020

Abstract
Paediatric brain tumour survivors (PBTS) experience slower information processing speed (IPS) that contributes to difficulty performing tasks of minimal (MC) and greater complexity (GC), and is related to aberrant neural communication. It is still unknown whether deficient IPS exists during increasing complexity. We aim to determine if PBTS experience deficient IPS and neural communication relative to typically developing children (TDC) during an increasingly complex visual-motor reaction time (RT) task. During magnetoencephalography recording, participants (n=58, 12.69 ±3.24 years) pressed a button with their left or right thumb after an arrow pointing in the corresponding direction appeared on a screen. During two MC conditions, the arrow pointed in a single direction. During a GC condition, the arrow alternated direction randomly. Mean RT &gt;3SD and signal artifacts were removed prior to analyses. The phase lag index (PLI) estimated neural communication between 90 cortical sources. Linear regression and Network Based Statistics assessed group differences in mean RT and the PLI. PBTS demonstrated increased RT relative to TDC during the GC condition (p=0.04, MPBTS=354.00s, MTDC=326.00s). Group differences in mean RT during MC conditions and the PLI during all conditions were not detected (p&gt;0.05). These results suggest PBTS experience slower IPS during GC. Reduced IPS is thought to contribute to difficulty recruiting cognitive resources needed to perform more complex tasks. Subtle deficits in neural communication may underlie slower IPS. The weighted PLI is superior to the PLI when estimating small differences in neural communication. We will now use the weighted PLI to assess task-related neural communication.
QOL-44. ASSESSMENT OF NEUROCOGNITIVE FUNCTION AND MRI PARAMETERS IN LONG-TERM SURVIVORS WITH POSTERIOR FOSSA TUMORS: A COMPARISON BETWEEN MEDULLOBLASTOMAS TREATED BY REDUCED-DOSE CRANIOSPINAL IRRADIATION AND OTHER TUMORS
Authors: Kagawa Naoki , Miyamura Takako , Hirayama Ryuichi , Yokota Chisato , Nakagawa Tomoyoshi , Kijima Noriyuki , Kinoshita Manabu , Hashii Yoshiko , Okada Keiko , Hara Jyunichi , Kishima Haruhiko 
Published: December 2020

Abstract

BACKGROUND
Children with medulloblastoma cannot avoid chemoradiotherapy including craniospinal radiation, although prognosis of medulloblastoma has improved and previous studies have reported a significant risk of intellectual disturbance by these treatments. We retrospectively analysed neurocognitive functions, clinical MRI parameters of patients with posterior fossa tumors, especially medulloblastomas.

MATERIALS AND METHODS
Twenty-two patients (12 medulloblastomas, 5 ependymomas, 5 astrocytomas) treated in our institution were enrolled in this study. Mean age was 7.8 years and 6.5 years, percentage of hydrocephalus at onset was 66.7% and 60%, respectively in medulloblastoma group and in other tumor group (ependymoma and astrocytoma). Postoperative chemoradiotherapy including reduced-dose craniospinal irradiation (18Gy) was done for medulloblastoma group and local radiation or operation only was done for other group. Version 3 or 4 of Wechsler Intelligent Scale for Children (WISC) was used by neurocognitive function analysis. Ventricular size, white matter volume and other parameters were also was estimated based on MRI. Follow-up duration was 6–17 years (mean: 10.5 years).

RESULTS
Evaluations of neurocognitive functions based on WISC pointed out lower performance IQ than verbal IQ in long term survivor of both group, especially working memory (P=0.05). Both hydrocephalus and cranial nerve complications was influenced lower scores of WISC, but age at onset did not influence WISC scores. Comparison between both group showed there was no significant difference about cognitive function and white matter volume.
QOL-54. HEIGHT, WEIGHT AND CARDIOVASCULAR EFFECTS OF STIMULANTS ON CHILDREN WITH BRAIN TUMOR
Authors: Khan Raja , Bano Maha , Mirman Igal , Wang Fang , Haitao Pan , Smith Jessica , Andrea Simmons , Greene William , Christensen Anthony , Sadighi Zsila 
Published: December 2020

Abstract

INTRODUCTION
Children with brain tumors may develop inattention, slow processing, and hypersomnia. Stimulant medications improve these problems but their effect on growth, heart rate, and blood pressure are inadequately explored.

METHODS
We retrospectively studied children with brain tumors treated at our institution that had data available for one-year pre and two year post stimulant treatment. Tumor location, gender, radiation treatment (RT), age at RT, drug type, and hormone therapy were variables of interest.

RESULTS
We identified 65 children (35 males) that fulfilled eligibility criteria. Focal RT was utilized in 58; 11 additionally received whole brain RT. Thirty were treated for hypersomnia and inattention, 8 for hypersomnia alone, and rest for inattention. Modafinil was the first drug in 18 (27.7%) and methylphenidate in the others. Forty-seven (72.3%), 40 (61.5%) and 49 (75.4%) were on thyroxine, cortisone and growth hormone respectively. There was no difference in pre and post stimulant BMI, heart rate, and blood pressure. There was also no difference between modafinil and methylphenidate groups. Rate of increase in height slowed on stimulants (p=0.0096). Thyroxine treatment correlated with increase in BMI after stimulants (p=0.0434). Younger age (p=0.0003) and higher BMI (p=0.0063) pre stimulants correlated with increased heart rate on stimulants, while higher age at RT (0.0159) correlated with elevated systolic BP on stimulants. No association of studied variables was found with height and diastolic BP.

CONCLUSION
Stimulants are well tolerated by children with brain tumors that are appropriately managed for endocrine deficiencies but may reduce the trajectory of height attainment.
RARE-01. MANAGEMENT AND OUTCOMES OF PAEDIATRIC CRANIOPHARYNGIOMA: A 15-YEAR EXPERIENCE IN SINGAPORE
Authors: Lim Mervyn Jun Rui , Liu Sherry Jiani , Ho Cindy Wei Li , Teo Kejia , Lwin Sein , Chou Ning , Yeo Tseng Tsai , Kimpo Miriam , Nga Vincent Diong Weng 
Published: December 2020

Abstract

BACKGROUND
Craniopharyngiomas are rare embryonic malformations of the sellar region with high survival rates but high morbidity due to long-term sequelae caused by the location of the tumour. We summarise our institution’s experience on the management and outcomes of paediatric craniopharyngiomas in Singapore.

METHODS
This was a retrospective review of all paediatric patients (18 years and below) with histologically diagnosed craniopharyngioma managed by the National University Hospital, Singapore from January 2002 to June 2017. Data on clinical presentation, imaging, treatments, and outcomes were extracted from the electronic medical records using a standardized data collection form. Data analysis was conducted using RStudio (Version 1.2.5033). Institutional ethics approval was obtained for the study.

RESULTS
We identified 12 cases of paediatric craniopharyngiomas. The majority of cases were male (8, 66.7%) and the median age at presentation was 6.0 (IQR 3.8 – 9.5). Initial surgical management was tumour excision (11, 91.7%) or insertion of a reservoir into the cyst cavity (1, 8.3%). All cases had diabetes insipidus, 10 (83.3%) had endocrine dysfunction, and 8 (66.7%) had visual impairment on long term follow up. 7 (58.3%) cases had recurrence, and 3 (25.0%) had demised. Cox-regression showed that females (HR=33.9, p=0.049), and Chinese race (HR=13.3, p=0.034) were at higher risk for recurrence, but age at diagnosis and residual tumor on post-operative MRI was not significant.

CONCLUSION
The management of craniopharyngioma is complex as it is complicated by high recurrence rates and significant long-term morbidity. Further research on treatment strategies focusing on maintaining quality of life is important.
RARE-07. THE LANDSCAPE OF GENOMIC ALTERATIONS IN ADAMANTINOMATOUS CRANIOPHARYNGIOMAS
Authors: Khadka Prasidda , Prince Eric , Lu Sophie , Santagata Sandro , Ligon Keith , Manley Peter , Beroukhim Rameen , Hankinson Todd , Bandopadhayay Pratiti 
Published: December 2020

Abstract

INTRODUCTION
Adamantinomatous craniopharyngiomas (ACPs) are characterized by activating mutations in the CTNNB1 gene. Here we perform a comprehensive genomic analysis of 23 ACPs to define the landscape of genomic alterations in this disease.

METHODS
We performed whole-genome sequencing of 24 ACPs and their matched normal tissues. We used Mutect 2.0 to detect mutations and indels in these samples and MutSig2CV to identify significant mutations. Copy numbers were called using the GATK4 pipeline and GISTIC 2.0 was applied to identify significant alterations. Finally, SvABA was applied to identify genome-wide structural variants and rearrangements.

RESULTS
18/24 (75%) of the sequenced ACPs harbored activating mutations in exon 3 of CTNNB1 gene with an average variant allele fraction (VAF) of 0.4±0.1. These mutations have previously been shown to activate the WNT signaling pathway in these tumors. No other significantly recurrent mutations were detected in our samples. The ACPs were quiet with regard to copy number alterations and no recurrent amplifications or deletions were detected. 528 structural variations and rearrangements were detected in total in all 24 samples with an average of 22 variants per sample. Gene-Set Enrichment Analysis (GSEA) of the RNAseq data revealed upregulation of WNT/B-catenin (FDR q-value &lt;0.25) in the CTNNB1 mutant samples compared to CTNNB1 WT samples.

CONCLUSION
Our study identified previously described activating CTNNB1 mutations in the majority of ACPs. In addition, we identified several rearrangements and structural variations in these tumors that could play an important role in the pathogenesis of the disease.