Pediatric Neuro-oncology Patients Research Articles

NURS-20. A PATIENT AND FAMILY EDUCATION PROJECT: COMPREHENSIVE RESOURCE FOR MEK INHIBITOR-INDUCED PARONYCHIA

Abstract BACKGROUND Targeted therapies for pediatric tumors of the central nervous system have recently brought promising results as well as new challenges in the clinical management of these patients. FDA approval of MEK inhibitors has prompted a significant increase in use of these agents in pediatric neuro-oncology patients. MEK inhibitors are commonly used for treatment of pediatric low and high-grade gliomas, as well being a proven treatment for plexiform neurofibromas. Cutaneous reactions to targeted therapy occur frequently, with paronychia arising in more than half the patients. METHODS Despite providing a robust MEK inhibitor skin care in-service at the start of treatment, the multidisciplinary neuro-oncology team identified a need for specific paronychia education for patients undergoing therapy with MEK inhibitors. The education content was developed following a thorough literature review of MEK inhibitors and associated side effects. Evidence-based information was selected to create a clear, concise, one-page educational resource. RESULTS The educational flyer addressed five key educational objectives including defining and reviewing paronychia as an expected side effect of MEK inhibitor therapy, discussing strategies for prevention and management of paronychia, and identifying when to contact a healthcare provider for paronychia support. Content included written and pictorial definitions to aid in recognition of paronychia, bulleted list and visual guide for preventive nail maintenance, recommendations and instructions for over-the-counter management strategies, review of therapies that may be prescribed, and specific guidelines on when to seek prompt medical attention. This flyer served as a valuable educational tool, equipping individuals undergoing MEK inhibitor therapy with knowledge about paronychia, prevention, and effective management. CONCLUSION MEK inhibitor-induced paronychia is an undesirable side effect that can impact adherence to treatment. By empowering patients and families with this educational resource, healthcare providers may enhance patient engagement, promote adherence to treatment, and optimize quality of life.

Electrographic Seizures and EEG Background Features in Pediatric Neuro-oncology Patients Admitted to the Pediatric Intensive Care Unit (P5-8.004)

Electrographic Seizures and EEG Background Features in Pediatric Neuro-oncology Patients Admitted to the Pediatric Intensive Care Unit (P5-8.004)

Reliability of signal intensity in the basal ganglia on non-contrast T1-weighted MR scans after repetitive application of a gadolinium-based contrast agent in pediatric neuro-oncology patients

PurposeTo evaluate the reliability of signal intensity (SI) changes in the basal ganglia as a supposed indicator of gadolinium deposition in the brain after repetitive application of gadolinium-based contrast agents (GBCAs) in a pediatric neuro-oncological collective. MethodsOne hundred and eight neuropediatric patients (54 male, 54 female, 0–17 years old), with repetitive GBCA-enhanced cranial MRIs between 2003 and 2017, were retrospectively analyzed. Two radiologists measured SI in the nucleus dentatus (ND), globus pallidus (GP), thalamus (T), and the pons (P). The NDP and GPT ratio were calculated. An intraclass correlation coefficient, and multiple linear regressions with subsequent stepwise backward variable selection were performed to evaluate the influence of gender, patient’s age at the first MRI, time interval between the first and last MRI, linear or macrocyclic GBCAs, residual pathology, treatments, and magnet field strengths. ResultsThe inter-reader agreement was good for GPT and NDP in the whole collective (ICC = 0.837 and ICC = 0.793) and for children >2 years of age (ICC = 0.874 and ICC = 0.790), but poor to moderate for children ≤2 years of age (ICC = 0.397 and ICC = 0.748). The intra-reader agreement was good (ICC = 0.910 and ICC = 0.882). An SI increase was only observed for both readers in GPT (p = 0.003, or p < 0.001). None of the considered cofactors showed a consistent effect on SI changes for either readers or regions. ConclusionMeasurements of SI changes in the basal ganglia are not a reliable parameter with which to evaluate or estimate gadolinium deposition in the brain or to identify suspicious influential factors after repeated GBCA applications.

OUTC-11. RACE AND AGE ARE ASSOCIATED WITH ICD-10 DIAGNOSES IN THE POST-OPERATIVE PERIOD FOR PEDIATRIC PATIENTS WITH PRIMARY BRAIN TUMORS UNDERGOING NEUROSURGERY

Abstract BACKGROUND Disparities in pediatric cancer care and outcomes are known to exist. This work evaluates the relationship of race, ethnicity, and social determinants of health (SDoH) on the post-operative neurosurgical course of pediatric neuro-oncology patients in a single-institution. METHODS Retrospective data were collected on patients undergoing initial neurosurgery for a primary central nervous system (CNS) tumor between May 2011 and April 2021. Newly attributed ICD-10 codes in the 30-day post-operative period were collected. Descriptive statistics were used to evaluate demographics, geographic region of residence, and insurance type. Logistic regression was performed to estimate the association between covariates and 30-day readmission as well as ICD-10 diagnoses that can complicate the postoperative course including cardiac/respiratory, bleeding, infection, wound, hardware implantation, and neurologic conditions. Linear regression was used to measure association of covariates and length of stay (LOS). RESULTS A total of 836 patients, median age 10.0 years, were included (n=368 females, 44%). Patients were predominantly white (69%) and non-Hispanic/Latino (88%). White (OR [95% CI] 0.55 [0.33,0.90], p=0.02), Native American/Indian (0.25 [0.07,0.89], p=0.03), and multiracial (0.24 [0.08,0.68], p=0.01) patients were significantly less likely to have new ICD-10 codes compared to Black patients. White patients trended toward shorter LOS (p=0.06) compared to Black patients. Younger patients were significantly more likely to have newly reported ICD-10 codes (0.95 [0.92,0.97], p&amp;lt;0.001), increased LOS (r=-0.59 [-0.87,-0.32], p&amp;lt;0.001), and 30-day readmission (0.81 [0.72,0.89], p&amp;lt;0.001). Sex, ethnicity, insurance type, and region had no significant association with outcomes. Income level analyses are ongoing. CONCLUSION Non-black patients were less likely to have new ICD-10 diagnoses reported after surgery for primary CNS tumors. Older age was a protective factor against new post-operative diagnoses, increased LOS, and mortality. More research is necessary to understand the relationship between race, ethnicity, SDoH and post-operative risk including possible mechanisms of interaction across variables.

Intensity at the end of life in pediatric neuro-oncology patients: The case for pediatric palliative care.

10021 Background: Although pediatric cancer is rare, it is the leading cause of death by disease after infancy for children and adolescents in the United States. Children exist in the context of a family; hence, excellent care for a child must also include attention to the needs of the family. Palliative care aims to enhance quality-of-life for these children living with life threatening illnesses and their families. Among pediatric cancers, brain tumors have high disease-related mortality rates; therefore, early introduction of palliative care to these patients is critical. This study aims to evaluate the association of palliative care during the terminal admissions of pediatric neuro-oncology patients and intensity of care provided at the end of life. Methods: Pediatric patients with brain tumors were identified using the Pediatric Health Information System (PHIS). Inclusion criteria were age &lt;18, diagnosis of brain tumor as defined by ICD-10 codes, discharged as deceased, and hospital admission between 1/1/2017 and 12/31/2021. Intensity of care measures included: pediatric intensive care unit (PICU) admission, mechanical ventilation, total parenteral nutrition (TPN), and extracorporeal membrane oxygenation (ECMO). Results: 567 patients met the inclusion criteria. 359/567 (63%) patients had an inpatient palliative care consultation during their terminal hospital stay. Patients receiving a palliative care consultation had a lower percentage of PICU admission (64.9 vs 83.2, p=&lt;0.001), mechanical ventilation use (51.0 vs 76.0, p=&lt;0.001), and TPN use (18.1 vs 25.5, p=0.027). There were no significant differences in length of stay (22.07 vs 22.25 days, p=0.181) and ECMO use (0.28% vs 1.92%, p=0.450). Conclusions: Pediatric patients receiving palliative care consultations during their terminal admission received less intense care while having comparable lengths of stay. Palliative care should be offered to all families, even with aggressive goals, to help incorporate quality of life and comfort in the treatment plan and to ensure bereavement support is available if needed. Similar to data in the adult palliative care literature, this data demonstrates reduced resource utilization among pediatric patients receiving palliative care. Palliative care has been shown to be a supportive service that has the potential to improve the quality of life of patients. The first step toward equitable healthcare is to ensure that all children with a potentially terminal cancer diagnosis have access to full supportive services, including palliative care.

Physician, patient, and caregiver support for a formal certification in pediatric neuro-oncology: A survey-based report from the SNO pediatrics working group.

Although CNS tumors are the most common pediatric cancer in the United States, most physicians caring for these patients are not formally certified in the subspecialty. To determine support for developing a formal certification process in pediatric neuro-oncology, the Society for Neuro-Oncology's Pediatrics Special Interest Track Training and Credentialing working group performed a cross-sectional survey-based study of physicians and patients/caregivers of children with a CNS tumor history. Surveys were built in Survey Monkey and were available for 3 months. The physician survey had 34 questions and was open to doctors currently caring for pediatric neuro-oncology patients. The patient/caregiver survey had 13 questions. Both surveys were completed anonymously. The physician survey was completed by 193 participants, the majority of whom self-identified as oncologists. Only 5.6% of survey participants had ever been board-certified in neuro-oncology; the majority of participating physicians were either unaware that this certification existed or thought they were not eligible due to training in pediatrics rather than neurology or internal medicine. Almost half of the self-identified pediatric neuro-oncologists had not completed any specific clinical neuro-oncology training. Over 75% of physicians were supportive of the implementation of a formal certification process in pediatric neuro-oncology. A total of 30 participants completed the patient/caregiver survey. Although the majority of survey participants were highly satisfied with their oncologist, 70% would have been more comfortable if their oncologist had been specifically certified in pediatric neuro-oncology. There is support from physicians, patients, and caregivers to establish a formal certification process in pediatric neuro-oncology.

"I Feel Happy Again": Methylphenidate Supports Health-Related Quality of Life in Survivors of Pediatric Brain Tumor.

Background: The deleterious impact upon the cognitive development of survivors of pediatric brain tumors (PBT) is well documented. Impairment in cognitive function is associated with reduced health-related quality of life (HRQoL), such that survivors of PBT report difficulties in multiple distinct domains and an overall reduced quality of life. Studies of the use of methylphenidate in survivors of PBT to alleviate impairment in cognitive functions have shown some success. The current study aimed to explore the impact upon HRQoL in survivors of PBT of a trial of psychostimulant medication. Method: Data were collected from 12 pediatric neuro-oncology patients aged 7–17 years receiving methylphenidate treatment. HRQoL was measured using the PEDS QL quality of life self-report measure and a semi-structured questionnaire-based interview. Results: Analyses of data demonstrates benefit to five domains associated with HRQoL: social, emotional, academic, physical, and cognition. Conclusion: Survivors of PBT reported favorable views as to the subjective benefit of methylphenidate on post-treatment impairment of HRQoL. This medication may offer the potential for restoration of a sense of ‘normality’ of function following cancer treatment in this clinical population.

TBIO-12. Screening for Cancer Predisposition Syndromes in Pediatric Neuro-Oncology Patients: a Single Institution Experience

BACKGROUND: Traditional screening for cancer predisposition syndromes centers on family history, phenotypic features, and tumor histology. With expanded accessibility of next generation sequencing, identification of de novo germline mutations is increasing and the predictive value of family history has become less clear. While identification of germline mutations often affects care of pediatric neuro-oncology patients, germline sequencing is not currently standard of care. We hypothesize that routine screening for germline mutations in pediatric neuro-oncology patients reveals unsuspected cancer predisposition syndromes and impacts care. METHODS: A retrospective analysis was performed on pediatric neuro-oncology patients at a single institution who had targeted next generation sequencing of approximately 500-cancer associated genes (UCSF500) on tumor and paired germline DNA. We determined the proportion of patients with germline mutations and assessed impact on future screening and current tumor treatment. We interrogated clinical notes, family history, and interviewed treating physicians to determine if predispositions were previously suspected. RESULTS: Between June 2015-December 2019, 187 patients had paired testing; of these 29 (16%) harbored germlines mutations that were pathogenic or likely pathogenic. Germline mutations were found in patients with high-grade glioma (n=12), low-grade glioma (n=7), medulloblastoma (n=4), ATRT (n=2), and choroid plexus papilloma (n=1). Known cancer predisposition syndromes were identified or confirmed in 18 patients. Of these, the most common alterations were in TP53 (n=6), CHEK2 (2), NF1 (n=2), SMARCB1 (n=2), and PTEN (n=2). Patients were referred to genetic counseling in 26 cases and malignancy screenings were implemented in 25 cases. Germline findings affected malignancy treatment in 10 cases, most often through use of targeted therapeutics or avoidance of radiation. CONCLUSIONS: In our series, we found that 16% of pediatric neuro-oncology patients harbored germline mutations, the majority of which were associated with cancer predisposition syndromes. These results support standardizing screening for pathogenic germline mutations in pediatric neuro-oncology patients.

PATH-11. Detection of genetic and epigenetic alterations in Liquid Biopsies from pediatric brain tumor patients

Abstract BACKGROUND: Liquid biopsies (LBs) hold great promise as a non-invasive method for tumor detection and disease monitoring. Their application in pediatric neuro-oncology patients has been challenging due to the blood-brain barrier limiting the amount of circulating tumor DNA (ctDNA) and low volumes of cerebrospinal fluid (CSF) and serum samples. Here, we assessed the feasibility of LBs in a pediatric brain tumor cohort (n=12 patients) by adapting pre-analytical protocols to low cell-free DNA (cfDNA) input and bioinformatic pipelines for genome-wide analyses. METHODS: Genomic DNA isolated from tumor tissues, including medulloblastoma (n=6), ependymoma (n=4), low-grade glioma (n=1), and choroid plexus papilloma (n=1), and cfDNA isolated from matched CSF and serum samples were subjected to low-coverage whole genome sequencing (lcWGS) and the EPIC DNA methylation array. For methylation analyses, samples were prepared using both traditional bisulfite- and recently developed enzymatic-based methylation conversion. Molecular tumor (sub)type predictions were performed using the Heidelberg Brain Tumor Classifier. RESULTS: lcWGS revealed tumor-specific copy number variations (CNVs) in both CSF and serum samples. The detection rate of tumor-specific CNVs (median 92% vs. 23%) and the ctDNA fraction (median 31% vs. 1.5%) were higher in CSF vs. serum samples. Bisulfite-converted as well as enzymatically converted tumor DNA and cfDNA samples could be readily analyzed using the Heidelberg Classifier. In comparison to bisulfite-based conversion, enzymatic-based conversion improved the quality of methylation analyses for minimal input cfDNA samples. CONCLUSIONS: Our results highlight the feasibility of liquid biopsies from both CSF and serum in brain tumor patients. In contrast to CNV-based tumor detection from serum samples, ctDNA analysis from CSF allowed a more comprehensive genetic and epigenetic profiling which is expected to assist in less-invasive tumor classification, identification of drug targets, or the investigation of tumor evolution. Altogether, this study provides the rationale for further implementation of LBs in pediatric neuro-oncology.

OTHR-08. Pediatric Neurologic Assessment in Neuro-oncology (pNANO) Scale: A tool to assess neurologic function for Response Assessment in Neuro-oncology (RAPNO)

Background: The Neurologic Assessment in Neuro-Oncology (NANO) scale, a standardized metric to objectively measure neurologic function in adult brain tumor patients, complements radiographic assessment in evaluating outcomes of neuro-oncology patients in clinical trials and clinical practice. Currently, there is no standardized measure for neurologic function in pediatric neuro-oncology patients despite their distinct clinical presentations and tumor locations. Therefore, we developed a dedicated pediatric NANO (pNANO) scale. Methods: An international group of pediatric neurologists and adult and pediatric neuro-oncologists convened bi-weekly over 5 months to draft the pNANO scale as an objective and quantifiable measure of neurologic function in children that can be administered during routine examination by pediatric-trained providers of any sub-specialty and be utilized together with other indicators to assess response in clinical trials. Results: Ten relevant domains of neurologic function were identified based on common pediatric brain tumor locations: gait, strength, cerebellar function, visual fields, visual acuity, facial strength, level of consciousness, extraocular movements, dysarthria and dysphagia. For each domain, developmental age appropriate levels of function were defined and categorized. Each domain, based on direct observation and testing during any routine neurological exam, can be used for longitudinal monitoring. Conclusions: The pNANO scale has been developed and aims to provide an objective metric of neurologic function for pediatric brain tumor patients. This scale will be tested for reliability, feasibility and inter-observer variability. Consistent evaluation of neurologic function using pNANO along with radiographic assessment will enable more comprehensive and standardized response assessment in pediatric neuro-oncology patients enrolled in clinical trials.

QOL-18. Neuropsychological screening in a pediatric neuro-oncology multidisciplinary clinic.

Abstract BACKGROUND: The purpose of this project was to examine the usefulness of neuropsychological screening in a multidisciplinary clinic for neuro-oncology populations and determine whether a brief evaluation can adequately screen children for cognitive dysfunction or psychiatric comorbidities. METHODS: Thirty-one children or young adults with a history of CNS tumor had neuropsychological screening as part of their multidisciplinary clinic visit. This screening evaluation consisted of clinical interview, neuropsychological testing, and the completion of emotional/behavioral checklists. Our neuropsychological test battery included the Kaufman Brief Intelligence Test-Second Edition (KBIT-2) and the NIH Toolbox Fluid Cognition Composite, which consists of 6 subtests - flanker task, working memory, processing speed, oral reading, card sort, and memory. RESULTS: Over a 15-month period 31 children, adolescents, and young adults with CNS tumors were seen in our neuropsychology screening clinic. Females made up 39% of the sample. The average age was 12.7 years (range=5–24 years). The average time to complete neuropsychological testing was 60.3 minutes. Thirty-six percent did not have any new neuropsychological diagnoses, 42% had one new diagnosis, 19% had two new diagnoses, and 3% had three new diagnoses. The most common new diagnoses were mild or major neurocognitive disorder (mild=29%, major=6%), anxiety (32%), ADHD (10%), and mood disorder (13%). Neurocognitive test scores were mostly in the average range (Verbal-IQ=97.7; Nonverbal-IQ=95.3; Total IQ=96.2; Flanker T-Score=46.1; Working Memory T-Score=44.0; Card Sort T-Score=43.7; Picture Memory T-Score=48.5). Processing speed and fluid cognition composite scores for the group were in the low-average range (Processing Speed T-Score=42.7; Fluid Cognition Composite T-Score=41.4). Behavioral health recommendations were needed for 71% of this sample. Educational recommendations were needed for 58%. CONCLUSIONS: Brief neuropsychological screening completed as part of multidisciplinary clinics for pediatric neuro-oncology patients was efficient and useful in identifying neuropsychological diagnoses in children and assisted with informing necessary behavioral health and academic interventions.

SWK-01. Partnership between caregivers, patients and healthcare professionals: promoting collaboration in a complex care pathway in pediatric neuro-oncology

Children with a brain tumor and their families encounter, from the moment of diagnosis, a complex, multidisciplinary and multisite journey. Oscar's Angels (OA) is an association of trained patient advocate volunteers within a hospital structure who provide daily support to pediatric neuro-oncology patients and their families in complex care pathways. The association promotes collaboration in healthcare by facilitating a strong link between the daily reality of patients and their families and that of the hospital. OA volunteers are active and fully accepted members of the hospital multi-disciplinary team and collaborate on a very high level in the care of pediatric patients. Such a collaborative setting is possible because the work of OA is structured around three principles: Innovative, practical and theoretical initial and ongoing training and quality assessment of volunteers Patient and family support from Oscar’s Angels volunteers throughout the care pathway Government certification, institutional representation and active collaboration in educating healthcare professionals about the importance of patient advocacy and patients’ needs Operating in France since 2001 and in Italy since 2017 (with more than 600 and 200 families respectively supported last year), the OA volunteering program is specifically tailored to the needs and demands of hospitals and families. It can therefore be successfully reproduced in many settings. OA’s services come at no cost to hospitals. This is particularly important for implementing the program in developing countries where healthcare resources are extremely limited. But it is also relevant in developed countries as well where healthcare budgets are under strain. In 2020 an informal internal OA survey highlighted that the patient/family/volunteer/healthcare professional interface provides added value for all of these stakeholders. A formal survey will be conducted in 2022 to corroborate these results.

QOL-07. The importance of an onco-fertility program for pediatric neuro-oncology patients

INTRODUCTION: In the Netherlands pediatric oncology care is centralized in one hospital since 2018. 600 new patients a year are seen with 100-120 new neuro-oncology (NO) patients. Of the NO patients 20-25% classify as high risk for infertility (HR) such as patients with medulloblastoma, ependymoma, ATRT and germinoma . An onco-fertility program was started navigated by a nurse-practitioner. The program runs with intense collaboration between the different specialties. All new patients are identified according to the international guidelines on fertility care. The fertility-risk is based on the CED score (cyclofosfamide equivalent dose) and radiotherapy dose. Since 2018 awareness was created by teaching sessions among colleagues, nursing staff, and parent association organizations. METHODS: All HR children are informed by the nurse-practitioner and can be referred for counseling to gynaecology for OTC (ovarian tissue cryopreservation) or urology for sperm cryopreservation or testicular biopsy. Monthly the onco-fertility working-group members discuss cases and research in the field. RESULTS: In 2019, 19% NO cases and in 2020 18% of cases were HR for infertility. In both years 36% of these cases had fertility preservation performed. In 2021, 22% cases HR were identified and in 55% preservation was performed. Reasons for not preserving fertility were diverse, varying from poor prognosis, or too ill to be included. CONCLUSION: Awareness of the fertility risk in NO patients who are HR is necessary. These patients need to be informed, and stratified for counseling and offered fertility preservation before start of their treatment. An active onco-fertility program helps to offer the best option for future fertility for these patients

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)

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.

NCOG-37. THE UTILITY OF NEUROPSYCHOLOGICAL SCREENING IN MULTIDISCIPLINARY CLINICS FOR PEDIATRIC NEURO-ONCOLOGY POPULATIONS

Abstract BACKGROUND The purpose of this project was to examine the utility of neuropsychological screening in multidisciplinary clinics for pediatric neuro-oncology populations and determine whether a brief evaluation can adequately screen children for cognitive dysfunction or psychiatric comorbidities. METHODS Children underwent neuropsychological screening as part of their multidisciplinary clinic visit. This screening evaluation consisted of clinical interview, neuropsychological testing, and the completion of emotional/behavioral checklists. Our neuropsychological test battery included the Kaufman Brief Intelligence Test-Second Edition (KBIT-2) and the NIH Toolbox Fluid Cognition Composite (consisting of 6 subtests). RESULTS Over a six-month period 40 children and adolescents with CNS tumors and/or NF1 were seen in our neuropsychology screening clinic. Females made up 37.5% of the sample. The average age was 11.1 years (range=5-25 years). The average time to complete neuropsychological testing was 56.8 minutes. On average, children in this sample met criteria for 1.8 neuropsychological diagnoses with an average of 1.2 new diagnoses that had not been given prior to their neuropsychological screening. The most common new diagnoses were major or mild neurocognitive disorder (mild=27.5%, major=20%), anxiety (27.5%), ADHD (15%), mood disorder (12.5%), and intellectual disability (10%). On average, these children performed lower than expectation on neurocognitive tests, including intellectual functioning (KBIT-2 Verbal IQ=93.1; KBIT-2 Nonverbal IQ=88.7; KBIT-2 Total IQ=90.1), attention (NIH Flanker T-Score=44.1), working memory (NIH Working Memory T-Score=42.9), processing speed (NIH Processing Speed T-Score=39.9), cognitive flexibility (NIH Card Sort T-Score=43.4), memory (NIH Picture Memory T-Score=44.5), and fluid cognition (NIH Fluid Cognition Composite T-Score=38.7). Behavioral health recommendations were needed for 72.5% of this sample. Educational recommendations were needed for 62.5%. CONCLUSIONS Brief neuropsychological screening completed as part of multidisciplinary clinics for pediatric neuro-oncology patients was efficient and useful in identifying neuropsychological diagnoses in children.

Genetic syndromes predisposing to pediatric brain tumors.

The application of high-throughput sequencing approaches including paired tumor/normal sampling with therapeutic intent has demonstrated that 8%-19% of pediatric CNS tumor patients harbor a germline alteration in a classical tumor predisposition gene (NF1, P53). In addition, large-scale germline sequencing studies in unselected cohorts of pediatric neuro-oncology patients have demonstrated novel candidate tumor predisposition genes (ELP1 alterations in sonic hedgehog medulloblastoma). Therefore, the possibility of an underlying tumor predisposition syndrome (TPS) should be considered in all pediatric patients diagnosed with a CNS tumor which carries critical implications including accurate prognostication, selection of optimal therapy, screening, risk reduction, and family planning. The Pediatric Cancer Working Group of the American Association for Cancer Research (AACR) recently published consensus screening recommendations for children with the most common TPS. In this review, we provide an overview of the most relevant as well as recently identified TPS associated with the most frequently encountered pediatric CNS tumors with an emphasis on pathogenesis, genetic testing, clinical features, and treatment implications.

QOL-57. SOUTHERN CALIFORNIA KAISER PERMANENTE PEDIATRIC NEURO-ONCOLOGY PROGRAM DEVELOPMENT

KEY MESSAGEStandardization of care for subspecialty patients require centralization and support across multi-disciplinary groups within the Kaiser Permanente medical group, which is a large health maintenance organization (HMO) in the United States.BACKGROUNDPrior to the development of a Pediatric Neuro-Oncology program, Southern California Kaiser Permanente pediatric neuro-oncology patients were routinely referred to respective regional academic centers for consultation. The process was not standard across the region, resulting in additional costs and differences in treatment recommendations, potentially affecting outcomes.METHODSA Pediatric Neuro-Oncology program was established, July 2017, based at the Kaiser Permanente Los Angeles Medical Center (LAMC), consisting of pediatric neuro-oncology, pediatric neurosurgery, pediatric neuro-radiology, pediatric radiation oncology, and pediatric neuro-oncology case management.RESULTSA Pediatric Neuro-Oncology tumor board was established to meet on a bi-monthly basis. Pediatric neuro-oncology patients across the Southern California now have their magnetic resonance imaging (MRI) reviewed by the same pediatric neuro radiologists. Neuropathology is standardized and sent to Children’s Hospital Los Angeles and reviewed at the molecular neuropathology tumor board attended by the pediatric neuro-oncologist. Cases discussions regarding the patients include the regional pediatric neurosurgeons, the pediatric radiation oncologists, and the pediatric neuro-oncologist, and treatment plans are recommended and recorded by the case manager.CONCLUSIONSCentralization of care has allowed for more consistent and standard care across the Southern California Region, but requires support from multi-disciplinary groups.

LINC-01. COMPLIANCE TO FOLLOW UP IN PEDIATRIC PATIENTS WHO HAVE RECEIVED CRANIOSPINAL IRRADIATION

OBJECTIVEAttendance to follow-up after completion of cancer treatment is understudied area. Pediatric cancer patients have sequelae of illness or treatment. Many have no symptom immediately after completion of treatment. Long term follow-up is important to access disease control, early diagnosis of recurrence, second cancer and treatment-related morbidities. Purpose of this study was to evaluate the compliance to follow-up in pediatric patients treated with craniospinal irradiation (CSI).METHODSThis was retrospective review of follow-up in pediatric neuro-oncology patients who received (CSI) from January 2017 to June 2018 in the Radiotherapy Department of Yangon General Hospital, Myanmar. RESULT: Twenty-three patients received CSI; majority (43%) were medulloblastoma. Median age was 7.5 years (3–17 years). Only seven patients (30.4%) were attended to follow-up more than 6 months after completion of treatment. More than two-thirds of patients (n=16,69.6%) were lost to follow-up. Patients in active follow-up were diagnosed and treated at earlier age below 10years (n=5,21.7%). Demographically, 5 patients (22%) were living in the region around tertiary hospital. Sixteen patients (69.6%) from rural area had limited transportation and difficulty for accommodation in which they were treated. In socioeconomic points, 18 parents (78.2%) had poor education and financial status, lack of understanding about disease, treatment, long-term effects and follow-up.CONCLUSIONAlthough this was limited data in CSI patients only, loss to follow-up after 6 months was high. We need to evaluate in all pediatric cancer patients and collaborate to provide financial support, childcare centres for lodging, transportation and health education to promote compliance to follow-up.

NURS-07. STAFF EDUCATION THROUGH NURSING AND PHARMACY COLLABORATION

Even within the focused field of pediatric oncology, there are healthcare providers who lack education regarding the specialized population of children with brain tumors. In order to improve staff knowledge of pediatric neuro-oncology, nursing and pharmacy developed a collaborative Lunch and Learn program to provide additional education. An eight week brain tumor curriculum was developed, and informal sessions grouped by diagnosis were held over lunch between the neuro-oncology nursing team (nurse practitioners and nurse coordinator) and a clinical pharmacy resident. A nurse practitioner provided academic literature and the pharmacy resident did further research and developed an outline for discussion. During these sessions, nursing was able to contribute academic knowledge and clinical experience, while pharmacy presented an overview of each tumor and provided education about medications. After each session, the pharmacy resident presented the information from the Lunch and Learn to all staff oncology pharmacists, which then increased their working knowledge of neuro-oncology as a whole, helping them feel better able to manage this population within their scope of practice. Because this innovative collaboration was so successful in heightening knowledge and awareness of the care and management of pediatric neuro-oncology patients for all those involved, the team now has future plans to utilize a similar model to provide neuro-oncology education to clinic and inpatient RNs.

Combined Denoising and Suppression of Transient Artifacts in Arterial Spin Labeling MRI Using Deep Learning.

Arterial spin labeling (ASL) is a useful tool for measuring cerebral blood flow (CBF). However, due to the low signal-to-noise ratio (SNR) of the technique, multiple repetitions are required, which results in prolonged scan times and increased susceptibility to artifacts. To develop a deep-learning-based algorithm for simultaneous denoising and suppression of transient artifacts in ASL images. Retrospective. 131 pediatric neuro-oncology patients for model training and 11 healthy adult subjects for model evaluation. 3T / pseudo-continuous and pulsed ASL with 3D gradient-and-spin-echo readout. A denoising autoencoder (DAE) model was designed with stacked encoding/decoding convolutional layers. Reference standard images were generated by averaging 10 pairwise ASL subtraction images. The model was trained to produce perfusion images of a similar quality using a single subtraction image. Performance was compared against Gaussian and non-local means (NLM) filters. Evaluation metrics included SNR, peak SNR (PSNR), and structural similarity index (SSIM) of the CBF images, compared to the reference standard. One-way analysis of variance (ANOVA) tests for group comparisons. The DAE model was the only model to produce a significant increase in SNR compared to the raw images (P < 0.05), providing an average SNR gain of 62%. The DAE model was also effective at suppressing transient artifacts, and was the only model to show a significant improvement in accuracy in the generated CBF images, as assessed using PSNR values (P < 0.05). In addition, using data from multiple inflow time acquisitions, the DAE images produced the best fit to the Buxton kinetic model, offering a 75% reduction in the fitting error compared to the raw images. Deep-learning-based algorithms provide superior accuracy when denoising ASL images, due to their ability to simultaneously increase SNR and suppress artifactual signals in raw ASL images. 3 TECHNICAL EFFICACY STAGE: 1.