bluuebunny/crossref_metadata_2025_split huggingface dataset:
QOL-20. A REVIEW OF THE LITERATURE ON THE REHABILITATION OF PATIENTS WITH POSTERIOR FOSSA SYNDROME
Authors: Levin Jared , Rosenberg Nathan , Tsao Elaine 
Published: June 2024

Abstract

BACKGROUND
Posterior fossa syndrome (also recently described as pediatric postoperative cerebellar mutism syndrome) is a well described phenomenon, in which a patient develops a spectrum of speech and motor/coordination deficits generally in the acute stage after posterior fossa tumor resection. While most patients recover well, some patients evidence significant deficits years after diagnosis, and there is some evidence that minor long-term deficits may remain. While its frequency of occurrence is relatively low, and efforts have been made to identify strategies to decrease the rate of Posterior Fossa Syndrome, the fundamental interventions for functional optimization and rehabilitation of patients are still being discussed and researched.

METHODS
Along with reviewing the most up-to-date literature on the rehabilitation management of patients with posterior fossa syndrome, we will discuss current a survey based study designed by a group of representatives from the American Academy of Physical Medicine and Rehabilitation (AAPM&R) Pediatric Cancer Rehabilitation subcommittee, to better understand the standards of how the syndrome is managed by physiatrists in the US. This survey distributed via email, posting on an AAPM&R online community, as well as social media sites.

RESULTS
While literature is limited, there is evidence for the practice of therapy services, bracing, and in admission for acute inpatient rehabilitation to guide treatment for patients with posterior fossa syndrome. Results from the above survey study will be discussed, which preliminarily shows a consistent reliance on therapy services and a broader heterogeneity to frequency and treatment preferences among physicians in the US.

CONCLUSION
Posterior Fossa Syndrome can be a challenging and debilitating disorder for pediatric brain tumor patients. Further research into treatment options and outcomes is necessary to guide future management.
QOL-21. FUNCTION-BASED OUTCOME MEASURES AND HOW THEY CAN BE APPLIED IN PATIENTS WITH PEDIATRIC BRAIN TUMOR FOR TRACKING RECOVERY AND RESEARCH
Authors: Tsao Elaine , Tsetlina Vera , Levin Jared 
Published: June 2024

Abstract

BACKGROUND
Currently an estimated 15,000 children are diagnosed with cancer annually in the United States. Among pediatric patients, central nerve system tumors make up the highest percentage of solid tumor malignancies. Tumors of the peripheral nervous system also occur, most commonly in syndromes such as neurofibromatosis. Tumor growth and tumor treatments can result in developmental and functional impacts on patients. As survival rates continue to improve through the years with advancement of pediatric cancer treatment, an increasing focus is being placed on improving function and quality of life both during and after treatment. This presentation aims to introduce a variety of validated outcome measures that assess a patient’s strength and function, to discuss how they can be applied to patient monitoring, and to propose future studies in the pediatric neuro-oncology population.

METHODS
This presentation will review the currently validated and commonly used outcome measures used by physicians and therapists for assessing a patient’s strength, endurance, and function. Emphasis will be placed on how these tools can be used in assessing and monitoring patients with conditions such as chemotherapy induced peripheral neuropathy, posterior fossa syndrome, and cachexia.

RESULTS AND CONCLUSIONS
This presentation aims to educate attendees on the current tools and outcome measures available to track a patient’s function and progress during and after treatment of neuro-oncologic conditions. It aims to highlight how these tools can be used in clinic for patient monitoring, and for future possible prospective research.
QOL-24. TREATMENT- AND TIME-RELATED IMPACT ON LONG-TERM NEUROCOGNITIVE OUTCOMES IN ADULT SURVIVORS OF A PEDIATRIC BRAIN TUMOR
Authors: Bullens Kristien , Blommaert Jeroen , Sleurs Charlotte , Laic Rebeca Alejandra Gavrila , Lemiere Jurgen , Jacobs Sandra 
Published: June 2024

Abstract
Treatment for pediatric brain tumors has been associated with the risk of developing long-term neurocognitive impairment. However, it is not well understood which neurocognitive profiles exist within this population. We investigated neurocognitive functioning (NF) in survivors in early adulthood and aimed to assess the impact of radiotherapy dose (RTD) and time since treatment (TxT) on NF. Forty-six survivors of pediatric brain tumors (mean age 23.99±4.71, mean age at diagnosis 9.29±4.54) performed an extensive neurocognitive test battery resulting in intelligence (n=5), memory (n=2), and language (n=3) scores. NF in long-term survivors was compared to sex-, age-, and education-matched controls, and between the different treatment strategies (surgery-only n=19, chemotherapy n=8, radiotherapy n=11, or chemo-radiotherapy n=8). Furthermore, relation between max-RTD (range 40.28 – 61.61 Gy) as well as TxT and NF was assessed. Three ANCOVA models were tested, and false discovery rate (FDR) was applied. For all scales and domain scores, survivors scored lower compared to controls. Scores were significant lower on full scale intelligence (pfdr&lt; 0.001), verbal comprehension (pfdr&lt; 0.001), working memory (pfdr= 0.003), perceptual reasoning (pfdr&lt; 0.001), processing speed (pfdr&lt; 0.001), spoken word comprehension (pfdr= 0.001), and semantic word generation (pfdr= 0.036) compared to controls. NF was not significantly different between the different treatments, nor did the association with max-RTD reach significance. TxT was associated with verbal comprehension and spoken word comprehension; albeit significance was not retained after FDR correction. In this study, the overall survivor cohort scored lower on multiple cognitive outcomes. More detailed analysis of various risk factors might further elucidate the different neurocognitive profiles for this population. Furthermore, more detailed analysis of RTD distributions and affected brain structures may provide greater insight on the impact of radiotherapy on NF.
QOL-28. NEUROCOGNITIVE DEFICITS, EMOTIONAL DISTURBANCES, PSYCHOSOMATIC COMPLAINS – ADDITIONAL SYMPTOMS OR COMORBID DIAGNOSIS IN CNS GERMINOMA? OVERVIEW OF GROUP AND CASE STUDIES
Authors: Korzeniewska Justyna 
Published: June 2024

Abstract

BACKGROUND
CNS Germinoma cases are associated with good medical prognosis, low risk for neurological deficits and high cognitive functioning. Deeper psychological and neuropsychological examination and longitudinal observation shows also neurocognitive problems and emotional disturbances. The purpose of the study was to assess patients with CNS Germinoma (during and after anticancer treatment), expressed specific psychological and physical symptoms and to find the reason for this.

METHODS
Since 1998 all patients treated at our center are under psychological control and care. From these group 78 patients with CNS Germinoma with additional significant symptoms, seriously affected their quality of life and everyday activity, were selected for psychological and neuropsychological assessment. Age at psychological intervention ranged from 6 5/12 to 34 8/12 yrs. The patients have no pre-diagnosis history of neurological, developmental or psychiatric disorder. They were examined using a battery of standardized psychological and neuropsychological methods.

RESULTS
In our sample FIQ (full-scale intelligence quotient) score ranged from 59 to 139. The most patients presented average level of intelligence. However, cognitive functioning was often reduced due to emotional disturbances and psychosomatic complains. 2 patients showed memory disorders, one of them TGA (transient global amnesia) at the time of diagnosis. All patients struggled with emotional disturbances at different grades, especially internalizing type of problems (immaturity, depression, anxiety, instability, inhibition, negative attitude) or were considered to be at risk for psychological adjustment difficulties and tendency to somatization. Analyzed group of patients showed also social competences deficits. They may exhibit deficit in age-appropriate social competence and peers relations.

CONCLUSIONS
Although CNS Germinoma patients shows similar behavioral and emotional phenotype, there are significant differences between them in terms of cognitive functions. The range of results is wide, from high levels to neurocognitive disorders. Therefore, risk factors, diagnostic criteria and effective neuropsychological intervention, will also be presented and discussed.
QOL-30. NEUROPSYCHOLOGICAL FUNCTIONING AFTER WHOLE VENTRICULAR OR CRANIOSPINAL PROTON RADIATION FOR PEDIATRIC INTRACRANIAL GERM CELL TUMORS
Authors: Paltin Iris , O’Brien Meghan K 
Published: June 2024

Abstract

BACKGROUND
In Western countries, intracranial germ cell tumors (ICGT) represent 3-5% of central nervous system tumors, with peak incidence in the second decade of life. Compared to those receiving photon radiation therapy, pediatric patients receiving proton radiation therapy demonstrate stronger neurocognitive outcomes, particularly in verbal and visual reasoning. Ongoing treatment advances aim to reduce morbidity, such as neurocognitive late effects. This study characterizes memory functioning for patients with intracranial germinoma treated with either whole ventricular (WV) or craniospinal (CSI) proton radiation therapy.

METHODS
29 patients received proton radiation therapy (17 WV, 12 CSI) for an ICGT (20 germinoma, 8 NGGCT, 1 mixed GCT; 3 disseminated at diagnosis). Age at diagnosis was 5-21 years (M=14.14; SD=3.91) with age at neuropsychological evaluation 8-25 years (M=17.66, SD=4.11). Evaluations occurred 0.71-7.85 years after diagnosis (M=3.52; SD=2.02). Patients (82.8% male, 65.5% white, 44.8% Very High Childhood Opportunity Index [COI]) had multiple additional interventions (surgeries, chemotherapy) prior to neuropsychological evaluation.

RESULTS
Patients in the WV group did not differ from patients in the CSI group on sex, COI, race, or ethnicity. Mean performance was stronger in the WV group than the CSI group for Full Scale IQ (FSIQ; t(27)=2.50, p=.019, Cohen’s d =0.94), Verbal Reasoning (VCI; t(24)=2.52, p=.019, d=1.0), Visual Memory (WRAML-2 Picture Memory Recognition; t(14)=2.51, p=.025, d=1.26), and Verbal Memory [(WRAML-2 Story Memory Delay, t(15.32)=2.82, p=.013, d=1.25); (WRAML-2 Story Memory Recognition, t(20)=2.34, p=.03, d=1.0)], all with large effect sizes.

CONCLUSIONS
Within this small ICGT sample with various medical treatments and comorbidities, demographic factors did not differ between groups, and WV proton radiation therapy appears to have less negative impact on aspects of reasoning and memory abilities compared to CSI proton radiation therapy.
QOL-42. ANALYSIS OF LATE EVENTS AFTER RADIATION THERAPY FOR PRIMARY PEDIATRIC BRAIN TUMORS
Authors: Trkova Katerina , Vicha Ales , Krskova Lenka , Koblizek Miroslav , Ondrova Barbora , Kopecka Marie , Landova Lucie , III Vladimir Benes , Zapotocky Michal , Sumerauer David 
Published: June 2024

Abstract

BACKGROUND
Late events after radiation therapy for primary brain tumors consist of secondary malignant neoplasms (SMN) and tumor recurrences. The incidence and biology of late events are not very well described. Therefore, we aimed to identify late events in our institutional cohort and describe their biology.

METHODS
A retrospective chart review of all irradiated pediatric brain tumor patients treated between 2000 and 2015 at our institution was performed to obtain demographics and clinical data of late events. Late events were defined as intracranial intraaxial SMN or tumor recurrence occurring later than 3 years after radiation therapy. A whole-genome methylation array was employed to identify the methylation class of the late event.

RESULTS
In the observed period, 267 patients received some form of cranial radiotherapy for primary brain tumor; high-grade glioma (HGG, n=66), medulloblastoma (n=74), ependymoma (n=50), germ-cell tumors (GCT, n=22), low-grade glioma (LGG, n=24), and other diagnoses (n=31). Out of the 174 patients who were alive and without an event after 3 years from the radiation therapy, 30 developed late events (16.8%), and 27 of them were histologically verified. Verified late events consisted of 8 SMNs (29.6%) and 19 primary tumor recurrences. SMNs were mainly radiation-induced gliomas (RIG, n=7) and one atypical teratoid/rhabdoid tumor (ATRT) after craniopharyngioma. All RIG tumors clustered with the “pediatricHGG_RTK1” methylation class. Tumor recurrences consisted of medulloblastomas (Group 4 and Wnt), ependymomas (PFA and ST_ZFTA), GCTs, gliomas (one NF1-associated HGG, one H3-altered thalamic glioma, one diffuse astrocytoma with BRAF-V600E mutation), and craniopharyngiomas. The overall survival rate 5 years after a late event was 39.7%, with a worse survival rate for SMN patients (14.3%) compared to those with recurrences (44.9%) (p=0.05).

CONCLUSIONS
Late events affected 16.8% of long-term survivors. Strikingly, SMNs represented almost 30% of late events and were associated with a very poor prognosis.
QOL-49. BOOKS4BRAINS: PILOTING A COMMUNITY CIRCLE BOOK CLUB TO IMPROVE SELF EXPRESSION
Authors: Knight Kelsey M , Appel Susan , Coven Scott L 
Published: June 2024

Abstract

BACKGROUND
Children with brain or spinal cord tumors often have limited interactions with their primary care providers who focus on developmentally appropriate recommendations, including the assessment of literacy. Additionally, we know that children with brain or spinal cord tumors have neurocognitive disruptions secondary to their diagnosis. Due to their disease process, these children are at high-risk for learning difficulties. We want to assist in building their self-confidence and acceptance. The objective was to provide a monthly virtual experience that utilized books, creative writing, and illustration to build a community centered on trust, shared experiences, and discussions of social emotional tools.

METHODS
We aimed to enroll 40 total participants diagnosed with a brain or spinal cord tumor who were followed by the Pediatric Neuro-Oncology Program at Riley Hospital for Children. These participants were divided amongst two groups: 1st-3rd grade and 6th-8th grade. After final enrollment, we had a total of 30 participants that received the following supplies: a copy of each months book, an audio recording of each book, and creative writing and craft supplies for each session.

RESULTS
We held three monthly hour-long virtual sessions for each group on Saturday mornings. Ten total children were available to meet consistently over the two groups. Positive feedback we received included, “we loved it,” “everyone felt included in book time,” and “[it] was so helpful that the books were read out loud and it was amazing to have the read along aspect with it for her visual impairment.” We even heard from one caregiver that “[they] noticed that she has some more tools in her toolbox when she is facing a difficult day.”

CONCLUSIONS
A pilot community book club is feasible through community partnerships. We plan to build on this experience by focusing on improving consistent attendance in future sessions.
QOL-51. FEASIBILITY AND EASE OF USE OF A REMOTE ONLINE COGNITIVE BATTERY AMONG PEDIATRIC BRAIN TUMOR SURVIVORS
Authors: Lee Kristin M , Yu Joan , Stancel Heather , Okcu M Fatih , Chintagumpala Murali , Kahalley Lisa , Raghubar Kimberly P 
Published: June 2024

Abstract

BACKGROUND
Pediatric brain tumor survivors are at risk for cognitive deficits, including inattention, poor memory, and slow processing speed. Due to technology advances, computerized neuropsychological batteries were developed that can be completed via internet in the home. The primary aim of this study was to evaluate the rate of successful completion and ease of use of the Penn Computerized Neuropsychological Battery (CNB) among pediatric brain tumor survivors.

METHODS
Forty-eight pediatric brain tumor survivors completed the online remote administration version of the Penn CNB (10 subtests) assessing complex cognition, memory, executive control, processing speed, and motor speed. Participants were provided with an electronic link to the online cognitive battery as well as paper and electronic copies of an instruction guide and post-completion surveys to return via mail/email.

RESULTS
Patients (56.3% female) were diagnosed and treated for a brain tumor (54.2% received radiotherapy). All participants were at least 8 years of age (M=13.71, SD=3.82) and at least one-year post-diagnosis (M=4.76, SD=2.58 years). Eighty-eight percent of participants beginning the CNB completed all subtests. Sixty percent of participants completed and returned the post-assessment survey. Participants who returned post-assessment surveys were similar to those who did not on demographic and medical factors (all p-values &gt;.05). Mean ratings suggest that survivors were “very interested” in the tasks and they were “somewhat easy” to complete. Younger children were more likely to have trouble understanding task instructions (r=.56, p &lt;.01) and ask for help answering specific questions (r=.46, p &lt;.05).

CONCLUSIONS
Remote administration of computerized batteries are feasible among pediatric brain tumor survivors and may assist with advancing both research and patient care. From a research standpoint, remote batteries may allow for participation and engagement in research with minimal survivor burden. Clinically, such batteries may offer alternatives for screening patients who will benefit from comprehensive services.
SURG-01. ULTRA-FAST DEEP-LEARNED CNS TUMOR CLASSIFICATION USING STURGEON; THE IMPACT ON STRATEGY FOR THE PEDIATRIC NEUROSURGEON
Authors: Hoving Eelco , Rooda Oscar Eelkman , Sie Mariska , Vermeulen Carlo , Pages-Gallego Marc , Kester Lennart , Kranendonk Mariette , Wesseling Pieter , van der Lugt Jasper , Tops Bastiaan , de Ridder Jeroen 
Published: June 2024

Abstract

INTRODUCTION
Central nervous system tumors still represent one of the most challenging fields to create curation in pediatric oncology. The primary treatment always starts with a neurosurgical intervention to obtain tumor tissue for diagnosis. In most cases this intervention is a maximal safe resection of the tumor. The oncological prognostic value of extent of resection is determined by the precise molecular diagnosis and per-operative ultra-fast molecular classification of central nervous tissue provides the neurosurgeon relevant information for choosing the right strategy. This per-operative molecular diagnosis determines the balance between the need of radicality versus the prevention of neurological morbidity in the individual patient. We shall report on our neurosurgical experiences with the per-operative use of Sturgeon in our national practice of pediatric neuro-oncology in the Netherlands.

METHODS
The ultra-fast AI-based nanopore sequencing method, Sturgeon, has been implemented as per-operative diagnostic in most neurosurgical tumor resections in our center (1). An overview of our nanopore sequencing pipe- and timeline will be presented and its impact on neurosurgical strategies will be shared.

RESULTS
Reliable and accurate diagnoses can be obtained within 40 minutes after starting sequencing in 45 out of 50 retrospectively sequenced samples (while abstaining from diagnosis of the other 5 samples). The implementation in real time surgery results in a turnaround time between taking out sample material to diagnosis in less than 90 minutes. The most important result is that there is a high sensitivity and virtually no false positives since it is refraining from diagnosis when there is too much uncertainty. Several illustrative cases with impact on the neurosurgical strategy have been collected.

CONCLUSION
Ultra-fast deep-learning CNS tumor classification using Sturgeon is feasible. Results of per-operative implementation illustrate the impact on neurosurgical strategy. 1. Ultra-fast deep-learned CNS tumour classification during surgery. Vermeulen C et al. Nature. 2023 Oct;622(7984):842-849.
SURG-02. INTRAOPERATIVE APPLICATION OF A POLYMER-BASED HYDROGEL AGAINST MEDULLOBLASTOMA GROUP 3 AND ATYPICAL TERATOID/RHABDOID TUMOURS
Authors: Moloney Cara , ElSherbeny Amr , McCrorie Phoebe , Porter Harry , Bastiancich Chiara , Bayraktutan Hulya , Oz Umut Can , Grundy Richard G , Nilsson Karin Forsberg , Brem Henry , Alexander Cameron , Tyler Betty , Smith Stuart J , Rahman Ruman 
Published: June 2024

Abstract

BACKGROUND
Local drug delivery systems (LDDSs) applied during neurosurgery offer a means of overcoming poor blood brain barrier (BBB) permeability and have been extensively investigated for the treatment of adult gliomas. To the best of our knowledge, they have not been investigated for the treatment of paediatric cerebellar tumours, including medulloblastoma group 3 (MB3) and atypical teratoid/rhabdoid tumours (ATRT), both of which are associated with poor prognoses.

METHODS
We prepared and characterised an injectable and biodegradable poly(ethyleneglycol)-poly(caprolactone)-poly(ethyleneglycol) (PECE) hydrogel, which has been investigated broadly throughout the literature with an array of therapeutic agents against numerous cancer types. Here, we loaded the PECE hydrogel with chemotherapeutics previously identified as being effective against primary MB3 and ATRT in vitro, but which do not cross the BBB in vivo, namely CHIR99021, ribavirin and PG545. Biocompatibility and cytotoxicity of drug loaded hydrogels was assessed in vitro. LDDSs safety and efficacy was evaluated using patient derived MB3 and ATRT intracranial xenograft resection models.

RESULTS
The hydrogel was both biocompatible and effected cytotoxicity following drug release. In vivo efficacy experiments against MB3 indicated a comparable median survival in treatment arms receiving radiotherapy or CHIR99021 and PG545 loaded LDDS. However, combined radiotherapy and LDDS conferred a significant survival enhancement including long-term survivors. Against ATRT, the median survival of arms receiving radiotherapy was comparable to that of the CHIR99021 and ribavirin loaded LDDS, with long-term survivors observed only in the latter arm.

CONCLUSIONS
The application of LDDSs against cerebellar brain tumours offers a promising novel therapeutic alternative. For MB3, the combination of radiotherapy and a LDDS significantly enhances survival compared to radiotherapy alone. For ATRT, the comparable survival of the LDDS and radiotherapy alone is encouraging and indicates the possibility of circumventing radiation-induced adverse effects for young children impacted by this disease.