bluuebunny/crossref_metadata_2025_split huggingface dataset:
QOL-24. Evaluating the diagnostic validity & predictive value of the Cerebellar Cognitive Affective Syndrome (CCAS) in pediatric posterior fossa tumour patients
Authors: Hoffmann-Lamplmair Doris , Leiss Ulrike , Peyrl Andreas , Slavc Irene , Czech Thomas , Gram Alexandra , Pletschko Thomas 
Published: June 2022

Abstract
OBJECTIVE: The present study aimed to examine the extent of deficits associated with the Cerebellar Cognitive Affective Syndrome (CCAS) in patients treated for paediatric posterior fossa tumours (PFT). Furthermore, we wanted to assess its diagnostic validity and predictive value for late sequelae. METHOD: Neuropsychological test data from 56 PFT patients treated at our pediatric neuro-onocology unit (mean age at treatment: seven years; mean age at first neuropsychological examination: nine years) were examined for severity of deficits in the CCAS core domains of executive functions, verbal functions, visuospatial abilities and emotions/behaviour. Patients with CCAS (n=20) were compared to two control groups without the syndrome (PFT patients treated either surgically (n=18) or surgically followed by radio/chemotherapy (n=18)) in terms of long-term neuropsychological and educational outcomes (mean age: 14 years). We also examined risk factors for CCAS development. RESULTS: The results showed mild to severe impairment in at least one CCAS domain in 55 out of 56 patients. Complete CCAS was diagnosed in 35.7% of cases. Seven years after tumour diagnosis, information processing, logical thinking, verbal functions, visuospatial skills and executive functions were more impaired in CCAS patients than in controls. They also needed more special educational support. Information processing speed was equally poor in CCAS patients and in controls treated with chemotherapy/radiotherapy. A significant association was found between risk factors and deficits in information processing speed, but not with the occurrence of CCAS. CONCLUSION: Deficits in the central CCAS features are common in PFT patients, but in varying degrees, suggesting that it is a continuous syndrome rather than a dichotomous one. However, it is problematic that domains such as information processing speed are not included in the diagnosis. External criteria regarding everyday life should be considered in future studies.
QOL-27. Sociocultural variables have a major impact on participation in patients treated for pediatric posterior fossa tumors
Authors: Hoffmann-Lamplmair Doris , Gram Alexandra , Krottendorfer Kerstin , Peyrl Andreas , Leiss Ulrike , Czech Thomas , Slavc Irene , Pletschko Thomas 
Published: June 2022

Abstract
OBJECTIVE: This study investigates the extent of participation barriers in patients with pediatric posterior fossa tumors (PFTs) at long-term follow-up. While most studies focus on pathobiological variables that promote the occurrence of adverse late effects, the present analysis aims to investigate the importance of environmental variables. METHOD: Ten years after tumor diagnosis, 42 patients (mean age: 17 years) treated at our pediatric neuro-oncology unit were assessed for school and social difficulties using a questionnaire developed within the framework of this study. Participation barriers were assumed if patients and/or their parents reported difficulties at school or with peers. The children and adolescents were classified into groups with adequate and limited participation. Potential pathobiological (treatment type, hydrocephalus, tumor recurrence, gender, age at diagnosis, seizures, cerebellar mutism) and environmental risk factors (parental and maternal education, siblings, primary language, discrepancies between personal and environmental values, regular physical activity, private housing) were identified and examined to see if the participation groups differed in the amount of risk factors. RESULTS: Almost a decade after treatment, two thirds of the patients had school and/or social difficulties. Not only pathobiological risk factors (type of treatment, hydrocephalus, tumor recurrence, cerebellar mutism and seizures), but also environmental factors (low maternal education level, siblings, a main language other than German, discrepancies between social and personal values, and irregular physical activity) were associated with limited participation. These variables discriminated significantly between patients with and without participation barriers. CONCLUSION: Participation barriers in patients treated for pediatric PFT are frequent. In addition to pathobiological variables, environmental risk factors also play an important role in limiting school and social participation. This underlines the fact that a sole consideration of pathobiology is not sufficient when examining risk factors for the occurrence of late effects. The adoption of biopsychosocial perspectives in future studies is inevitable.
NURS-02. Incorporating Nurses and Advanced Practice Providers into Clinical Trial Consortiums; results of a multi-institutional survey from the Pacific Pediatric Neuro-Oncology Consortium (PNOC)
Authors: Raber Shannon , Hoffman Carly , Kline Cassie , Prados Michael , Mueller Sabine , Meyer Ashley 
Published: June 2022

Abstract
BACKGROUND: Incorporation of nurses and advanced practice providers (APPs) creates an opportunity to enhance the work of clinical trial consortia in providing care to children with central nervous system tumors enrolled on clinical trials. Within PNOC, nurses and APPs led an effort to engage nurse scientists in collaborative work. A formalized APP and nursing committee confirmed multi-institutional interest for this effort via a widely distributed, electronic survey. OBJECTIVES: The primary aim was to assess the need for a nursing and APP committee by surveying nurses, APPs, physicians, and CRCs across academic institutions within US and international sites. A secondary aim was to explore educational needs of nurses and APPs and establish resources to augment clinical trial protocol initiation and compliance. METHODS: A de-identified, electronic Qualtrics survey was distributed to all PNOC member institutions in Fall 2021. Participants were divided into 3 groups: physicians, nurses or APPs, and CRCs. RESULTS: A total of 68 participants from 20 different institutions completed surveys. All groups confirmed support for a formalized nursing and APP committee within PNOC. Nurses requested education on targeted therapies, specific study protocols, general neuro-oncology information, and late effects. Barriers for nurse participation included lack of dedicated time (50%) and travel funding for conferences (39%). The surveys elicited physicians overwhelmingly identified key needs for APP or nursing involvement in standardizing protocolized care across institutions (100%), education for patients and staff (95%), and protocol development (89%). CONCLUSIONS: Based on multi-institutional survey results, a nursing and APP working group has been established within PNOC. In response to the needs-based survey, the working group provides a quarterly nursing newsletter, offers education sessions and mentorship opportunities, and participates in multi-disciplinary, disease-specific working groups. Future efforts will review the impact of this committee on protocol development and compliance and overall clinical care of pediatric neuro-oncology patients.
OTHR-05. Visual impairment in children with a newly diagnosed brain tumor: a Dutch prospective nationwide cohort study
Authors: Nuijts Myrthe , Stegeman Inge , Seeters Tom , Bennebroek Carlien , Naus Nicole , Porro Giorgio , van Egmond-Ebbeling Michelle , Imhof Saskia , van Meeteren Antoinette Schouten- 
Published: June 2022

Abstract
PURPOSE: Visual impairment is a serious adverse effect in children with a brain tumor. To date, visual impairment is often underestimated and unrecognized, while early detection of visual impairment is important to potentially preserve the visual function. Our aim was to assess the prevalence and type of abnormal ophthalmological findings in an unselected cohort of children with a newly diagnosed brain tumor and to identify potential oncological and clinical risk factors. PATIENTS AND METHODS: In this prospective nationwide cohort study, we included children, aged 0-18 years, with a newly diagnosed brain tumor in the Netherlands between May 2019 and August 2021. A comprehensive ophthalmological examination (including orthoptic evaluation, visual acuity, visual fields and funduscopy) was performed at diagnosis. Multivariable logistic regression analyses were performed to estimate the odds ratio (OR) and 95% confidence interval (CI) of the risk factors. RESULTS: In total, 170 children were included (43.5% female; median age [interquartile range], 8.3 [3.9 – 13.0] years; 48.2% infratentorial tumors, 31.2% midline tumors and 20.6% cerebral hemispheres tumors). Overall, 101 children (59.4%) presented with visual symptoms at diagnosis. The most common findings on ophthalmological examination were papilledema (52.4%), gaze deficits (33.5%), visual field defects (28.1%), nystagmus (24.8%), strabismus (19.9%) and decreased visual acuity (8.6%). The risk of papilledema was associated with older age in years (OR 1.2, 95% CI 1.1 – 1.3) and hydrocephalus (OR 14.8, 95% CI 5.4 – 40.0). Visual field defects were detected more frequently in children with a tumor located in the cerebral hemisphere (OR 4.5, 95% CI 1.3 – 15.3) or midline (OR 7.0, 95% CI 2.2 – 21.9). CONCLUSIONS: More than 50% of the children had abnormal ophthalmological findings at brain tumor diagnosis. These findings emphasize the necessity of standardized ophthalmological surveillance and the importance of awareness of health care providers of ophthalmological abnormalities in this patient group.
OTHR-06. Knowledge gap among health care providers in childhood central nervous system tumors: Result from an international multicenter survey
Authors: Rajagopal Revathi , Moreira Daniel C , Faughnan Lane , Wang Huiqi , Naqvi Sameen , Devidas Meenakshi , Qaddoumi Ibrahim 
Published: June 2022

Abstract
BACKGROUND: Central nervous system (CNS) tumors in children are associated with a longer delay in diagnosis. One of the contributing factors is the lack of awareness regarding childhood CNS tumors presentation among health care providers. To evaluate the knowledge gap among health care providers, we conducted a cross-sectional survey that was distributed globally. METHODS: The survey was disseminated to health care practitioners via electronic mail in November 2018 and it was closed in March 2020. The participants were asked to complete a pre-test survey, requested to view a CNS tumor education seminar, and subsequently complete a post-test survey. The survey had nine questions focusing on CNS tumor symptoms, pre-diagnosis symptom interval (PSI), and imaging indication. The knowledge gap was evaluated with pre-test and post-test scores. RESULTS: 889 pre-test and 392 post-test responses were received. The majority of the respondents were from Asia, with a percentage of 73.1% and 87.5% in pre-test and post-test respectively. For the pre-test, the median score for accurate answers was 40.0% (range:13.1-92.9%). Interestingly, a high rate of correctness was achieved in the post-test with a median score of 77.1% (14.9-98.2%). In the pre-test, only 18.7% of the participants responded precisely that Cushing’s triad is a less common symptom and just 15.0% recognized that older children &gt;10 years old are at risk for late diagnosis. Surprisingly, 21.9% falsely reported that patients with malignant tumors experience the longest PSI, and 54.5% of the respondents wrongly selected medulloblastoma as the commonest CNS tumor. Overall, the pre-test scores among pediatricians and professionals with &gt;10 years of experience did not demonstrate improved knowledge when compared to other specialties. CONCLUSIONS: The survey analysis showed a significant knowledge gap regarding childhood CNS tumors among health care providers. Therefore, raising professional awareness is very important and can be achieved through targeted educational strategies.
OTHR-09. The prevalence and complex management of MEK inhibitor induced cutaneous side effects
Authors: Meyer Ashley , Ogle Andrea , Shatara Margaret , Cantor Evan , Cluster Andrew , McHugh Michele , Beck Mary , Reiners Stephanie , Coughlin Carrie , Abdelbaki Mohamed , Brossier Nicole 
Published: June 2022

Abstract
BACKGROUND: Cutaneous side effects commonly occur with MEK inhibitor (MEKi) therapy and can be challenging to manage. METHODS: A retrospective chart review was performed on sixteen pediatric patients treated with MEKi therapy for at least three months. These patients were diagnosed with either a brain tumor, plexiform neurofibroma, Langerhans Cell Histiocytosis, or Parkes Weber Syndrome (PWS). OBJECTIVES: To describe cutaneous side effects from MEKi therapy, compare the side effect profiles of patients treated for different diagnoses, and compare the side effect profiles between different MEKi agents. RESULTS: The most prevalent cutaneous toxicities for all eligible patients were acneiform rash (81%, most common), paronychia (50%, second most common), hair changes and xerosis. Mean number of cutaneous skin toxicities were 3.2 (trametinib group: 3.4, selumetinib group: 2.8). Only one patient, treated with trametinib for PWS, did not experience a cutaneous side effect. The average number of interventions (new medication or over-the-counter product) recommended to prevent or treat cutaneous-related toxicities was 6.8 per person, the majority of those were for acne and paronychia. Over 75% of the interventions were topical. Of those who experienced an acneiform rash, the average number of recommended interventions were 3.1. Sixty two percent of these patients developed acne within the first cycle; average time to development was 2.6 months into therapy. Most exhibited improvement overtime following intervention. Those patients who experienced paronychia were treated with a mean of 3.1 interventions – 37.5% developed paronychia within the first cycle of therapy; average time to development was 3.9 months into therapy. CONCLUSIONS: Cutaneous side effects are common, occur early in therapy, and require multiple interventions. The number and complexity of these interventions may further complicate the overall management of cutaneous side effects given that the responsibility of the administration falls on the patient. This differs from traditional chemotherapy regimens.
OTHR-10. Pilocytic astrocytoma with respect to treatment
Authors: Hwee Tang Phua , Nisa Sameema 
Published: June 2022

Abstract
AIM: To describe the sizes of pilocytic astrocytoma with respect to treatment Methodology Pediatric pilocytic astrocytomas cases from 2001 to 2021 were retrospectively reviewed in this Institutional Review Board approved study. Imaging reports, location of tumour, maximum dimension of tumour at diagnosis, treatment given (operation/chemotherapy/ radiotherapy), degree of tumor excision were captured. RESULTS: Imaging was available in 33 with 23 centered in the posterior fossa (1 extending into thalamus), 4 in suprasellar region, 2 in cerebral hemisphere, 2 in thalamus, 1 in pineal thalamic region and 1 in cervicomedullary spine, Tumor dimension at presentation was 5.40 cm ± 2.34 cm. Tumor size at presentation did not show significant correlation with age. 30 patients underwent operation with tumours completely excised in 15 and partially excised in 14 and no postoperative information for 1. Three patients, where tumour involved the thalamus, did not have operation and were given radiotherapy, average size of tumour being 3.47 + 1.15 cm. compared to the 5.59 + 2.34 size of tumours that underwent operation (p=0.06). Completely excised tumours measured 6.29 ± 2.04 cm at presentation while incompletely excised ones measured 4.76 ± 2.53 cm, not significantly different (p=0.09). Unoperated tumours are statistically smaller than those completely excised (p=0.02). One of the completetly excised tumours was located in the parietal cerebral hemisphere with the rest of the 15 in the posterior fossa. Seven of the incompletely excised tumours were located in the posterior fossa with 4 in suprasellar region, 1 in thalamus, 1 in spine and 1 in cerebral hemisphere. 3 patients with uncompletely excised tumours (1 cerebral, 1 post fossa, 1 spine) had post-operative radiation while 2 suprasellar tumours were given post-operative chemotherapy. CONCLUSION: Completely excised tumours are mainly located in posterior fossa. Tumours not operated on are located in thalamus and significantly smaller than tumours which are completely excised.
OTHR-12. Incidentalomas; should benign, indeterminate MRI findings be discussed at the paediatric neuro-oncology MDT
Authors: Large Alexandra , Picton Sue 
Published: June 2022

Abstract
BACKGROUND: In 2020, 503 patients were discussed at the weekly neuro-oncology MDT meeting, at a UK tertiary paediatric hospital. This was a 50% increase from 2016. Anecdotally, an increased number of non-tumours were being discussed. OBJECTIVES: To determine whether Paediatric Neuro-oncology MDT is consistent with follow up of benign, incidental findings on MRI scan and whether these lesions become significant.METHODS: MDT discussion notes reviewed for all incidental and benign MRI head findings between 05/09/18 and 02/09/20.RESULTS: 59 MRI head lesions, that were deemed to be both incidental and benign were discussed at the MDT. 44% of referrals were from the local hospital; this may be due to availability of the MDT or complexity of the patient cohort. Findings discussed included pineal cysts (24), arachnoid cysts (3), epidermoid cysts (1), pituitary lesions (4) and other indeterminate lesions or signal change (27). The majority of children received serial MRI imaging after MDT. There were large inconsistencies in the follow-up interval recommended, even within similar pathologies. For example, pineal cysts follow up ranged from no repeat imaging to multiple repeat scans with 6 and 12 month intervals. ‘Complex’ cysts were more likely to receive follow up but there is little consensus between radiologists about criteria for a ‘complex cyst’. None of the lesions reviewed changed significantly over time. Perhaps most strikingly, only 2/27 of the ‘indeterminate lesions’ have been discharged from the MDT. CONCLUSIONS: As clinical reasoning is seldom recorded in reports or MDT discussion notes the reasons for different surveillance recommendations is difficult to ascertain retrospectively. However, as MRI scans become more readily available incidental and being findings will increase, potentially putting great pressure on an already stretched service. Serial MRI imaging also creates unnecessary anxiety for both the child and their family.
OTHR-16. Nutritional screening of U.S. pediatric central nervous system tumor survivors
Authors: Soja Jacqueline , Wilson Rebekah , Yunker Alexandra , Aziz-Bose Rahela , Gordon Erin , Dieli-Conwright Christina , Cooney Tabitha 
Published: June 2022

Abstract
INTRODUCTION: Adult survivors of pediatric central nervous system (CNS) tumors are at the highest risk for morbidity and late mortality among all childhood cancers – owed in part to chronic health condition burden, which intersects with environmental and lifestyle factors. Their weight epidemiology has not been previously characterized. METHODS: An institutional, cross-sectional study was carried out amongst young adults ages 18-39 years, previously treated for pediatric CNS tumors and seen in our specialty survivorship clinic from January 2016 to December 2021. Sociodemographic, clinical, and treatment information was extracted from medical records and summarized by descriptive statistics. Anthropometric information extracted from clinical visit records was used to calculate body mass index (BMI) and weight status category according to CDC guidelines. RESULTS: We identified 200 survivors, 47% male and 53% female. Median age was 26 years (range 18-39); patients were median 16.5 years (range 1.5-34.2) from initial diagnosis. Most common primary diagnoses were low grade-glioma (51.0%) and embryonal tumor (20.5%.) One hundred and five (52.5%) survivors had received prior cranial radiation; 81 (40.5%) had an endocrinologic disorder at time of visit. Amongst the entire cohort, 39% were healthy weight, 5% were underweight, 27% overweight, 21% obese, and 8% severely obese. CONCLUSION: The majority of young adult survivors seen in our clinic were not healthy weight by BMI definition. The distribution of BMI groups appears comparable to contemporary studies of the general young adult U.S. population. For those with decreased muscle mass, a previously recognized issue in pediatric CNS tumor survivors, BMI may underestimate body fat. By abstract presentation, univariable analyses will be performed using the Fisher’s exact test or Wilcoxon rank sum test to assess for undernutrition and overnutrition risk factors. Our refined understanding of the nutritional epidemiology in this population will allow for more strategic diet and exercise interventions.
OTHR-17. Guidance for providing families with autopsy research results
Authors: Meyer Ashley , Beck Mary , Cluster Andrew , Ogle Andrea , Rubin Joshua 
Published: June 2022

Abstract
BACKGROUND: The Legacy Program is a project where research autopsies are performed on the brain of Neuro-Oncology patients. There continues to be growing consensus that investigators should offer their study participants results once the research project has concluded. Several organizations like the Children’s Oncology Group have established Return of Results programs however, returning research results from autopsy samples is not a common practice. Families have voiced the importance of autopsy donation and want results from that donation. To them, this donation creates a legacy for their loved one and provides hope that their contribution to research will impact the life of future patients. As researchers, this project provides a novel platform where patients and their families can directly influence new research endeavors. OBJECTIVES: We organized a retreat to disseminate research findings to the families of patients who have donated tissue. In addition to the family members, the entire research team and clinical staff were invited. Our primary aims were to evaluate participant satisfaction and obtain feedback that would be utilized for future program planning. METHODS: At the end of the retreat a de-identified, open-ended questionnaire was handed out. Six were returned and evaluated. RESULTS: All participants were in favor of the format and content that was presented and requested an invitation to future retreats. Participant feedback included how to become more involved with the Legacy Program and the request for the creation of a social media group where families could continue to connect and provide support to each other CONCLUSIONS: Our study confirmed the importance of returning autopsy results. The retreat provided needed support to the participating families. During the retreat, researchers were able to hear direct feedback from their findings and the families voiced their goals for future research. This information was invaluable and helped shape our program.