bluuebunny/crossref_metadata_2025 huggingface dataset:
QOL-18. THE MODERATING ROLE OF SOCIAL VULNERABILITY IN THE RELATIONSHIP OF MATERNAL CAREGIVER CONDITION MANAGEMENT ABILITY AND TRANSITION READINESS OF THEIR YOUNG ADULT SURVIVORS OF PEDIATRIC BRAIN TUMORS
Authors: Anil Ashley , Hammer Shannon N , Hobbie Wendy L , Daly Brian P , Deatrick Janet A , Barakat Lamia P
Published: June 2024

BACKGROUND

The transition into adulthood is a particularly vulnerable time for young adult survivors of pediatric brain tumors (YAS) due to tumor/treatment-related late effects. Reduced supportive resource access and social determinants of health (SDH) can exacerbate challenges, impacting caregiver condition management ability (CMA) and YAS transition readiness. This study’s aim was to evaluate the relationship between YAS cognitive late effects, caregiver CMA, and YAS transition readiness, and the moderating role of SDH for YAS and caregivers with condition-focused family management.

METHODS

Fifty-three maternal caregivers (93% white, 98% non-Hispanic) of YAS (Mage=22) reporting low CMA completed the Transition Readiness Assessment Questionnaire (TRAQ; 5 subdomains) and rated YAS cognitive late effect severity. Medical experts scored 1) cognitive medical sequelae (0-6 scale), and 2) the Pediatric Neuro-Oncology Rating of Treatment Intensity. Social Vulnerability Index (SVI) measured cumulative SDH using 16 factors across US Census residential zip code data. Pearson correlations and moderated mediation analyses in Process Macro were used.

RESULTS

Caregiver ratings were associated with overall transition readiness (r=-.38, p=.005), cognitive medical sequelae (r=.53, p<.001), and treatment intensity (r=.28, p=.039). Expert ratings were not related to transition readiness (ps>.05); therefore, only caregiver ratings were used in moderated mediation models. For overall transition readiness, managing medications, appointment keeping, talking with providers, and managing daily activities, the direct effects of caregiver ratings (ps>.05) and indexes of moderated mediation via CMA (ps>.05) were not significant. For tracking health issues, the direct effect of caregiver ratings (p=.085) was not significant, but the index of moderated mediation was significant (95%CI=.026;.315).

CONCLUSIONS

Only caregiver cognitive late effect ratings were helpful in explaining YAS overall transition readiness. The role of social vulnerability in CMA and transition readiness warrants further exploration. Empirically based transition readiness YAS programs targeting access to resources for families with more social vulnerability are needed.
QOL-31. NEUROPSYCHOLOGICAL FUNCTIONING AND QUALITY OF LIFE IN INFANT AT/RT SURVIVORS: FOCUS ON FLUID INTELLIGENCE AND VISUAL PROCESSING
Authors: Traunwieser Thomas , Loos Elena , Ottensmeier Holger , Mynarek Martin , Bison Brigitte , Kandels Daniela , Rossius Anne , Lüttich Peggy , Baust Katja , Faulstich-Ritter Kristin , John Rainer , Kreisch Andrea , Landmann Judyta , Manteufel Eva , Nest Alexandra , Prüfe Jenny , Schubert Lisa , Stamm Walther , Timmermann Beate , Gerss Joachim , Rutkowski Stefan , Schlegel Paul-Gerhardt , Eyrich Matthias , Gnekow Astrid K , Frühwald Michael C
Published: June 2024

BACKGROUND

Understanding the long-term cognitive sequelae in infant brain tumor survivors remains incomplete, particularly regarding the impact of tumor type, multimodal treatment, and other patient-related factors. This retrospective analysis explores neuropsychological and quality of survival (QoS) outcomes in survivors of atypical teratoid/rhabdoid tumors (AT/RT) and extracranial malignant rhabdoid tumors of soft tissues (eMRT) and kidneys (RTK), all treated within the same framework. Neuropsychological data from children with AT/RT were compared to data from children with non-irradiated low-grade glioma (LGG).

METHODS

Patients (0 - 36 months at diagnosis) underwent various treatments, including radio-chemotherapy for AT/RT (n = 13) and eMRT/RTK (n = 7), chemotherapy only for LGG (n = 4) and eMRT/RTK (n = 1), or observation for LGG (n = 11). Neuropsychological evaluations were conducted at a median of 6.8 years (AT/RT), 6.6 years (eMRT/RTK), and 5.2 years (LGG) post-diagnosis.

RESULTS

Impairments were observed for all tumour types. Patients with AT/RT exhibited impairments in fluid intelligence (p =.041; d = 1.11) and visual processing (p =.001; d = 2.09) when compared to LGG-patients. Both groups demonstrated deficits in psychomotor speed and attention abilities (p <.001–.019; d = 0.79–1.90). Diagnosis significantly predicted cognitive outcomes, whereas gender and age-related variables did not. QoS outcomes for all rhabdoid patients indicated lower scores in psychosocial functioning (p =.023; d = 0.78) and quality of life (p =.006; d = 0.79) compared to healthy controls.

CONCLUSIONS

Infant rhabdoid tumor survivors experience cognitive and quality-of-life sequelae. Patients with AT/RT are especially vulnerable to impairments in fluid intelligence and visual processing, while infant LGG-patients without radiotherapy demonstrated comparable deficits in psychomotor and attention abilities. Close monitoring of neuropsychological and quality of life outcomes is crucial for early onset and multimodal treatment.
QOL-33. CANCER WORRY AND PSYCHOPATHOLOGICAL SYMPTOMS IN ADOLESCENTS AND YOUNG ADULTS SURVIVORS OF PEDIATRIC BRAIN TUMORS
Authors: Profio Sonia Di , De Giuseppe Sara , Verrico Antonio , Piccolo Gianluca , Molteni Marta , Venturino Cristina , Garrè Maria Luisa , Milanaccio Claudia
Published: June 2024

BACKGROUND

Adolescent and young adult (AYA) survivors of pediatric brain tumors may experience cancer-related worries, defined as concerns about relapse and late effects of treatment. Late psychological effects include psychological distress, depression, immaturity, and poor development of social skills. Furthermore, pediatric brain tumor survivors (PBTS) are more vulnerable to developing long-term neurocognitive and psychosocial consequences compared both to the general population and survivors of other types of childhood cancer. The purpose of the study was to identify the presence of cancer worry and psychopathological symptoms in AYA survivors of pediatric brain tumors.

METHODS

AYA survivors were assessed using the Cancer Worry Scale and Child Behavior Checklist questionnaires. Neurocognitive evaluation was performed using the age-appropriate Wechsler Scale.

RESULTS

Among 22 AYA survivors (M =14; F=8), the diagnosed tumor types were: germ cell tumor (10/22), low-grade glioma (4/22), embryonal tumor (3/22), high-grade glioma (2/22), craniopharyngioma (2/22), and mesenchymal tumor of the meninges (1/22). Survivors received the assessment at a median age of 20.2 years (range 13.6-28.7), with a median of 92 months after diagnosis. Cancer worry was reported in 50% of patients. The psychological evaluation showed clinical levels of internalizing problems in 17% and externalizing problems in 8%. Depressive and anxiety problems were reported in 25% of our cohort. Neurocognitive assessment demonstrated an average tIQ of 104.

CONCLUSIONS

As reported in the literature, cancer worry can influence the psychosocial adjustment process, alter self-image, and influence lifestyle decisions in PBTS. These concerns can affect their life prospects and their ability to successfully complete developmental tasks. Therefore, these issues must be early identified to prevent, through targeted psychosocial interventions, the onset of troubles in having romantic relationships, finishing school, entering the job market, and obtaining a good quality of life.
QOL-36. IMPACT OF RADIATION ON SYMPTOMS OF ANXIETY IN PEDIATRIC PATIENTS WITH BRAIN TUMORS
Authors: Lucas Bryony J , Stancel Heather H , Raghubar Kimberly P , Christopher Krystal K , Ibarrola Ana Sofia C , Grosshans David R , Paulino Arnold D , Okcu M Fatih , Chintagumpala Murali M , Brown Austin L , Scheurer Michael E , Kahalley Lisa S
Published: June 2024

BACKGROUND

Children and adolescents with brain tumors are at increased risk for anxiety compared to unaffected peers, but the impact of treatment on anxiety are unknown.

METHODS

We identified patients diagnosed with a brain tumor at Texas Children’s Hospital (2012-2019), excluding high-grade glioma. Anxiety was evaluated using the BASC-2, with T-scores ≥60 considered positive. Baseline assessment occurred within six months of start of treatment and were repeated annually. Chi squared tests of independence were performed.

RESULTS

Of 156 patients included, 92 received proton radiation therapy (RT) and 64 did not receive any RT. 52% were male, and patients were diagnosed at a median age of 10.4 years (range: 6-20) with a median follow-up of 4.2 years (range: 0-11.4). 42% of patients (n=66/156) screened positive for anxiety at least once during the follow-up period. Among those treated with RT, 15% screened positive for anxiety at baseline and the proportion significantly increased at 1-year (29%) and 2-years (34%) follow-up (p=0.016). For patients spared radiation, there was no statistically significant change over time (p=0.56), although the proportion positive for anxiety trended lower over time with 30% positive at baseline, 26% positive at 1-year and 20% positive at 2-years of follow-up. In the non-RT cohort, patients reported more anxiety symptoms at baseline than patients who did receive RT (p=0.029), but no significant differences were observed at 1-year (p=0.80) or 2-year follow-up (p=0.13).

CONCLUSIONS

Nearly half of pediatric brain tumor patients report anxiety during or in the years following treatment. We identified differences in the trajectory of anxiety symptoms based on RT, with declining levels of anxiety observed in the population not receiving RT and an increasing proportion of patients reporting anxiety in the years following RT. These findings may help guide the delivery of supportive services to populations most at-risk of anxiety.
QOL-47. FINANCIAL TOXICITY IN PEDIATRIC NEUROSURGICAL ONCOLOGY
Authors: Larsen Alexandra M Giantini , Brown Samantha , Reiner Anne , Hoffman Caitlin , Greenfield Jeffrey , Souweidane Mark , Newman W Christopher
Published: June 2024

BACKGROUND

Financial toxicity encompasses the negative impact of medical costs on patients. To our knowledge, financial toxicity in pediatric neurosurgical oncology remains unstudied.

METHODS

All pediatric neurosurgical cases from 2016 to 2020 performed at Memorial Sloan Kettering Cancer Center were included. Financial toxicity (FT) was defined as patients experiencing: bankruptcy, bills sent to collections, settlement, or timed payment plan, expression of financial concern to social work, or utilization of copay, financial, or social work philanthropy assistance programs. FT was assessed in the 2 years pre- and post- surgical intervention for all patients.

RESULTS

Two hundred and ninety-five surgical procedures across 203 patients were analyzed. The rate of FT in the two years prior to the first neurosurgical procedure was 17% (34/203). The most common toxicities were financial assistance program (47%), social work philanthropy (29%), and bills sent to collections (24%). The rate of FT for the subset of patients surviving at least 2 years after surgery was 34% (30/87) with the most common toxicities being financial assistance program (33%), collections (33%) and social work philanthropy (27%). The majority of patients who experienced FT had at least one commercial insurance policy (56% for the pre-surgical cohort, 70% for the post-surgical cohort). For all 203 patients, the 2-year cumulative incidence of FT following first neurosurgical procedure was 19% (CI:14%,25%) with those experiencing pre-operative toxicity having a significantly higher two-year cumulative incidence of FT at 62% (95%CI: [43%,76%], p<0.001) following first neurosurgical procedure. Patients without FT prior to first procedure had a two-year cumulative incidence of FT of 11% (95%CI: [7%,16%], p<0.001).

CONCLUSIONS

FT rates are substantial in the pediatric neurosurgical oncology population. Pre-operative FT influenced post-surgical FT. The most common toxicity was financial assistance program utilization. The majority of patients and families who experienced FT had at least one commercial insurance plan.
NURS-05. CATALYZING AND INFORMING THE DEVELOPMENT OF MORE COMPREHENSIVE AND ACCESSIBLE PROGRAMS FOR CAREGIVERS OF YOUNG ADULT SURVIVORS OF CHILDHOOD BRAIN TUMORS
Authors: Ramaswamy Nithya , Herchline Emily , Joseph Melanie , Hammer Shanon , Anil Ashley E , Hobbie Wendy , Minturn Jane E , Barakat Lamia P , Deatrick Janet A
Published: June 2024

BACKGROUND

The chronic and intensive care needs of young adult survivors of childhood brain tumors are most often addressed by their maternal caregivers with limited, specific psychosocial or other programs to reduce caregiving demands and enhance their family management. Using a social ecological perspective, the purpose of this study was to catalyze and inform the development of more comprehensive and accessible programs for caregivers of young adult survivors of childhood brain tumors.

METHODS

Semi-structured interviews were conducted with 21 maternal caregivers, 6 post baccalaureate students/professional coaches for a problem-solving intervention for maternal caregivers, and 16 neuro-oncology clinical experts using interview guides tailored to their roles and constructed based on the Consolidated Framework for Implementation Science. Qualitative. Interview data were analyzed using content analysis.

RESULTS

Barriers included: need for internet connected devices (microsystem level); lack of care matched to their identified needs (mesosystem); poor tracking of long-term survivors and lack of survivorship psychosocial services (macrosystem); and lack of supportive health care policies and funding mechanisms for individuals and organizations (exosystem). Facilitators included: web-based platform accessible to the individuals and population (microsystem); widespread acknowledgement regarding gaps in survivorship psychosocial care (mesosystem); partnerships with survivors of childhood brain tumors, caregivers, and organizations that treat them (macrosystem); and dissemination among childhood brain tumor treatment/survivorship programs, healthcare systems, and community organization and endorsement from them (exosystem).

CONCLUSIONS

Multilevel approaches that address barriers and facilitators for improving health equity for maternal caregivers of young adult survivors of childhood brain tumors incorporate the design and dissemination of accessible, web-based programs designed in partnership with survivors, caregivers, and organizations to build care matched to need, as well as organizational, political, and community advocacy.
NURS-09. FERTILITY PRESERVATION AND PALLIATIVE CARE: RECOGNIZING THE NURSING ROLE IN THE PROMPT IMPLEMENTATION OF SUPPORTIVE CARE INTERVENTIONS IN THE PEDIATRIC CENTRAL NERVOUS SYSTEM TUMOR PATIENT POPULATION
Authors: McHugh Michele , Waters Kaaren , Briamonte Courtney Baker , Higgins Micah , Hoffman Carly , Holtzclaw Susan , Meyer Ashley , Raber Shannon , Rangan Kasey , Schmus Cindy , Seidl Kenny , Strachan Marylynne , Yeh-Nayre Lanipua
Published: June 2024

BACKGROUND

Given the complexity of pediatric central nervous system (CNS) tumors, an interdisciplinary approach is necessary to incorporate supportive care interventions that complement oncologic treatment plans. However, early implementation of both palliative care and fertility preservation services presents challenges within the pediatric neuro-oncology population.

METHODS

An extensive literature review was conducted for articles published between 2013-2023 using PubMed, Google Scholar, and CINAHL databases. The articles focused on nurses’ roles in pediatric oncology, palliative care, fertility preservation, and barriers to supportive care. Furthermore, the review incorporated collective knowledge sharing among pediatric neuro-oncology nursing specialists. This joint initiative applied evidence-based practices to showcase the successful implementation of palliative care and fertility preservation services, broadening this project’s scope.

RESULTS

One prevalent theme that emerged highlighted the delay in supportive care referrals attributed to perceived obstacles by healthcare team members. The misconception that palliative care is solely for end-of-life situations or the assumption that families are not ready to acknowledge an incurable diagnosis causes delays in palliative care referrals. Fertility preservation in pediatric oncology encounters obstacles, including the urgency to commence treatment, determining optimal candidate criteria, and navigating discussions with younger patients. Nurses are uniquely positioned in their role to bridge gaps between oncologic treatment interventions and the incorporation of supportive care measures. Recognizing the supportive care needs of patients, nurses can educate families, advocate for early referrals, and coordinate integrative care. Advanced Practice Nurses (APNs) can offer clinical consultation, and order appropriate screening, testing, medications, and referrals.

CONCLUSION

Disseminating nursing education across pediatric oncology practices and institutions is essential for identifying perceived barriers and reforming the early supportive care intervention process. This collaborative and proactive approach is designed to optimize the overall care experience, with the goal of enhancing the delivery of supportive care for pediatric neuro-oncology patients.
NURS-12. NEURO-RADIOLOGY 101 FOR RNS AND APPS
Authors: Strachan Marylynne , Briamonte Courtney Baker , Higgins Micah , Holtzclaw Susan , McHugh Michele , Meyer Ashley , Raber Shannon , Rangan Kasey , Schmus Cindy , Seidl Kenny , Waters Kaaren , Yeh-Nayre Lanipua , Hoffman Carly
Published: June 2024

BACKGROUND

Magnetic Resonance Imaging (MRI) brain are the gold standard of diagnosing pediatric brain tumors. In pediatrics, MRI’s are often presented at new diagnosis or recurrence at tumor boards, which includes APP and RN’s in a multidisciplinary approach. By providing basic understanding of MRI imaging and sequencing, APP’s and RN’s will be able to participate and understand patient treatment discussions.

METHODS

During the presentation, we will review the 3 common planes used in MRIs: axial, coronal, and sagittal. We will then discuss the common MRI sequences, including T1, T2, FLAIR, DWI, and post-contrast imaging and discuss which common brain tumors and anatomy are best seen on said sequences.

RESULTS

NA.

CONCLUSIONS

By the end of this presentation, pediatric RNs and APPs will have the basic understanding of MRI images and feel more confident to participate in multidisciplinary tumor board discussions.
NURS-17. SHOULD EVERY CENTRE HAVE A TARGETED THERAPY CLINIC?
Authors: Ryles J , Arif T , Connor A , Halliday G , Hill R M , Leech S , Rennison R , Thornton T , Bailey S
Published: June 2024

There is increased use of targeted therapies for children with Central Nervous System (CNS) tumours both at presentation and at relapse. Eighteen months ago, a dedicated CNS targeted therapy clinic was set up at The Great North Children’s Hospital in Newcastle upon Tyne. Referrals are accepted from all major centres in the north of the UK. Within our monthly clinic, five targeted therapies have been used so far including Selumetinib and Tovorafenib with an average of eight children per clinic. General practitioners are asked to carry out all blood investigations forty-eight hours prior to clinic. This has been audited and initial analyses show we have successfully reduced the time children spend in hospital from four hours to one hour. Having blood results prior to clinic means treatment can be prescribed and dispensed promptly. Furthermore, obtaining bloods in the community has reduced the workload for our oncology day unit. The side effect profile of targeted therapies differ from those of standard chemotherapy and requires specific expertise from members of the multidisciplinary team. We are able to predict trends in symptoms and manage them successfully and efficiently. We have direct access to the ophthalmology and Neurofibromatosis team at each clinic. We offer skin care management prior to beginning treatment and will refer to the dermatology team for a same day review where needed. Finally, this clinic has enabled us to offer informal peer support for children and their families, which enhances their overall hospital experience. In summary, our Targeted Therapy Clinic, with the invaluable input of many members of the MDT, has improved the quality of care for children being treated on targeted agents. Given these benefits, we are keen to support other Primary Treatment Centres to develop similar clinics, especially with the increased use of targeted treatments across CNS tumours.
NURS-19. IMPLEMENTING A NURSING LED WORKING GROUP WITHIN THE PEDIATRIC NEURO-ONCOLOGY CONSORTIUM (PNOC), A TWO-YEAR EXPERIENCE AND EVALUATION
Authors: Raber Shannon , Hoffman Carly , Kline Cassie , Mueller Sabine , Prados Michael , Meyer Ashley
Published: June 2024

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 and young adults with central nervous system tumors enrolled on clinical trials. Within PNOC, a formalized APP and nursing working group (“APPNIC”) was established in 2021. Since inception, APPNIC has assigned members to each clinical trial committee, developed support documents to partner with protocols, highlighted supportive care measures, and provided nursing education across all PNOC sites.

METHODS

A de-identified, electronic Qualtrics survey was distributed to PNOC members in Fall 2023 to evaluate the 2-year impact of APPNIC. Physicians, nurses or APPs, and CRCs were eligible to participate.

RESULTS

Thirty-six surveys were completed in total from physicians or principal investigators (n=15; PIs), nurses/APPS (n=18), and clinical research coordinators (n=3; CRCs) representing multiple institutions. PI satisfaction with APPNIC was high with all reporting APPNIC to be “somewhat helpful” (40%) or “very helpful” (60%) and most PIs (53%) working directly with APPNIC members. Nurses confirmed they use the protocol roadmaps (87%) and education sheets (80%) generated by APPNIC. Barriers to APPNIC participation continue to indicate scarce resources allowing time for APPs and nurses to participate in hybrid meetings and limited travel funds to attend meetings and conferences. Identified education needs of APPs and nurses include identifying supportive care for novel treatments, understanding developmental therapy strategies, and receiving routine study updates.

CONCLUSIONS

With high overall satisfaction of PIs and utilization of study deliverables by consortium site members, we plan to continue APPNIC as currently structured. Ongoing efforts to integrate APPNIC members early in protocol development will increase PI and APPNIC member collaboration. Education for APPs and nursing will continue to be a priority, along with investigating mechanisms to increase funding to attend meetings and conferences.
NURS-20. A PATIENT AND FAMILY EDUCATION PROJECT: COMPREHENSIVE RESOURCE FOR MEK INHIBITOR-INDUCED PARONYCHIA
Authors: Apelquist Jacqueline O’Connell , Burnside Kasey L , Day Sarah , Trinidad Vless , Rangan Kasey , Waters Kaaren , Smith Lara G
Published: June 2024

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.
NURS-22. NAVIGATING DISPARITIES: COMPREHENSIVE CARE AND ACCESS TO CLINICAL TRIALS FOR ADOLESCENTS AND YOUNG ADULTS WITH CENTRAL NERVOUS SYSTEM TUMORS
Authors: Briamonte Courtney , Holtzclaw Susan , Higgins Micah , Hoffman Carly , McHugh Michele , Meyer Ashley , Raber Shannon , Rangan Kasey , Schmus Cindy , Seidl Kenny , Strachan Marylynne , Waters Kaaren , Yeh-Nayre Lanipua
Published: June 2024

BACKGROUND

Central nervous system (CNS) tumors are one of the most common causes of cancer-related death in the adolescent and young adult (AYA) age group. Despite being a prevalent cancer diagnosis, adolescents and young adults continue to be underrepresented in clinical trials. Literature review shows a notable lack of research focused on clinical care, reproductive health, and psychosocial aspects specific to this population. The longstanding issue of low AYA participation in clinical trials persists across various cancer types, with a particular scarcity of data regarding CNS tumors. Notably, the lack of participation in clinical trials has been associated with worse survival outcomes and increase in frequency and severity of treatment-related toxicity, emphasizing the critical impact of this issue on patients’ overall morbidity and mortality. The literature underscores the under-representation of AYAs with CNS tumors in neuro-oncology trials..

METHODS

This talk will review challenges faced by adolescents and young adults (AYAs) diagnosed with central nervous system (CNS) tumors.We will discuss the necessity for molecular profiling in the diagnostic workup of CNS patients to delineate tumor biology and guide treatment plans, including clinical trial access. To address the insufficient understanding of best practices for this group, there is a crucial call for expanding access to clinical trials. We will explore barriers to clinical trial access and provide case examples of successful and unsuccessful work with AYA patients with CNS tumors with ideas to improve access. Finally, we will discuss the holistic care of AYAs with CNS tumors through a multidisciplinary approach, incorporating expertise in neuro-oncology, oncofertility, palliative care, and psychosocial aspects within both pediatric and adult care settings

RESULTS

N/A

CONCLUSIONS

By the end of this presentation, pediatric RNs and APPs will have an understanding of the differences in caring for this unique population of patients including benefits of enrolling adolescents and young adults with CNS tumors in clinical trials.
OTHR-04. HEALTH SYSTEM DELAYS IN DIAGNOSIS AND TREATMENT OF CHILDREN WITH CENTRAL NERVOUS SYSTEM (CNS) TUMOURS IN SRI LANKA
Authors: Somathilaka Mahendra , Jayathilaka Ashika , Jayasekera Dhanushka , Azwer Mohomad I M , Jayaweera Nilrukshan , Garusinghe Sanjeewa , Jayawickrama Modhini , Constantine Roshana , Gupta Tejpal , Chinnaswamy Girish , Upadhyaya Santhosh , Salman Zeena , Qaddoumi Ibrahim , Gunasekera Sanjeeva
Published: June 2024

BACKGROUND

Children with central nervous system(CNS) tumours have to navigate multiple layers of care to reach the only centre providing oncology care (chemotherapy and radiotherapy) in Sri Lanka. This can lead to significant delays and affect outcomes.

METHODS

This is a retrospective study of patients less than 19 years registered at the National Cancer Institute Sri Lanka(NCISL) from 1st January 2022 to 31st December 2023. Data was extracted from clinic records and interviewing caregivers.

RESULTS

From a total of 828 new children with cancer 135 had CNS tunours and data was available for 130 for analysis. Commonest cancers seen were low grade gliomas and medulloblastoma. Fifty-one percent were males and the median age was 6 years (Range 1-18). Most common presenting symptoms were headache and vomiting. Median time from onset of symptoms to first medical contact was 4 days (InterQuartile Range(IQR) 1-9.5) and to first imaging it was 44 days (IQR 3-151) for all symptoms. In the presence of neurological symptoms, the time to imaging was 26 days (IQR 2-141). Ninety-six(74%) had upfront surgery at a median time 14 days from first imaging (IQR 7-34.5) with a median time of additional 25 days to reach an oncology centre (Range 7-94). Patients who did not have upfront surgery took 13.5 days (IQR 2-36.5) for the same. After arriving at the oncology centre, radiotherapy (80/130, 62%) was initiated in 24 days. When calculated from date of surgery radiotherapy initiation time was 61 days. (Range 5-88)

CONCLUSION

Although there seem to be no significant delay in presentation of children with CNS tumours to the health system, there were significant delays in imaging and initiating adjuvant radiotherapy. Further studies are warranted to determine the attributing factors and corrective steps to be taken to expedite referral of affected children.
OTHR-07. LIFE THREATENING CATATONIA AND THE USE OF ELECTROCONVULSIVE THERAPY IN A PATIENT WITH RELAPSED MEDULLOBLASTOMA
Authors: Chaleff Stanley , Gerwin Roselyn , Wolfrum Lee , Bokat Pamela , Kastle Nadav
Published: June 2024

Medulloblastoma is one of the most common malignant brain tumors in the pediatric population and with multimodal therapy the majority of patients will be cured, but patients experience significant morbidity related to the disease and therapy. Catatonia is a life-threatening psychomotor disorder that has rarely been described in the pediatric population and is likely under recognized. Catatonia is a treatable condition using benzodiazepines as first line with electroconvulsive therapy (ECT) used for patients who do not respond or have life threatening malignant catatonia. In the medical literature there are very few papers to inform on the safety of using ECT in patients who have received radiation in the past or are undergoing chemotherapy. We describe a patient in remission of his medulloblastoma who presented with refractory catatonia who needed lifesaving ECT, then had metastatic recurrence of his medulloblastoma. The patient was initially diagnosed with group 4 medulloblastoma at 14 – he was treated with high-risk therapy to metastatic sites of disease at diagnosis as per COG ACNS 0332 with a combination of surgery, chemotherapy and craniospinal radiation and a boost to the primary tumor and metastatic sites of disease. Approximately 18 months after the end of therapy he was diagnosed with treatment refractory catatonia and needed to be started on ECT with a good response. 6 months after presentation of catatonia, the patient unfortunately had a relapse of his medulloblastoma in his left temporal lobe and is now actively getting ECT and chemotherapy at the same time without complications. The description of this patient will add to the sparse body of evidence that it is likely safe to be able to deliver chemotherapy at the same time as ECT.
OTHR-14. AN ADOLESCENT WITH A RARE SCHWANNOMA LOCALIZATION AND THE IMPORTANCE OF METHYLATION STUDIES
Authors: Luz Inês Romão , Gonçalves Raquel , Sousa Rita , Carvalho Sílvia , Novo Ana Margarida , Rebelo Olinda , Pinheiro João , Soares Gustavo , Frank Stephan , Hench Jürgen , Carvalho Alice
Published: June 2024

BACKGROUND

Schwannomas are benign nerve sheath tumours, accounting for about 8% of intracranial tumours, with different histologic patterns described. Olfactory nerves are not expected to have Schwann cells, several theories exist about the origin of these tumours.

METHODS/RESULTS

A 16-year-old boy was admitted to the emergency department due to a first, self-limited, isolated epileptic event. He scored 15 in the GCS and no focal neurological deficits were observed. At that time, he complained about headaches, lasting for 3 months. Brain CT showed a frontobasal heterogeneous mass. MR characterized it as a 43X34X38 mm well-circumscribed mass, with solid and cystic areas, having an intimate relationship with lamina cribosa, with extensive vasogenic oedema surrounding it, causing deviation of adjacent structures. At that time, he was COVID-19 positive. He was submitted to a craniotomy with a transcortical approach, with an apparent total resection. Retrospectively, he became aware of an improved sense of smell after intervention. The histopathological study revealed a tumour composed of sheets and lobules of large, round cells showing immunopositivity for SOX-10 and S100 and with a Ki-67 index of 5-6%. We proceed to a molecular diagnosis, as the morphology was elusive, finding a methylation pattern highly close to those of schwannomas. With this presumptive diagnosis, clinical and radiological follow-up has been done. After two years, he is doing well, without signs of recurrence.

CONCLUSIONS

Although rare, an olfactory nerve schwannoma should be considered when there is a frontobasal tumour and impaired sense of smell, although in this case, the COVID-19 infection could be responsible for hyposmia. The clinical picture, imaging and histopathology description were not enough to make the diagnosis and, therefore, to plan the post-surgical approach. We emphasize the importance of a comprehensive molecular study, with a methylation classifier, as this tumour exhibits a typical DNA methylation pattern.
OTHR-22. SUCCESSFUL DEVELOPMENT OF RESOURCES FOR DMG/DIPG PATIENT FAMILIES THROUGH A NETWORK OF FAMILIES, RESEARCHERS, AND CARE TEAMS
Authors: Lyons Nicole , Ward Lisa
Published: June 2024

The DIPG community has been severely affected by a lack of resources and awareness due to the relative rarity of the disease. In recent years, this has led to a network of patient families, researchers, and clinicians who have worked together to develop resources that improve clinical care, research, and patient family support. These resources address issues in care equity such as access to clinical trials, accurate information, and consults with leading physicians in the field. These initiatives span across multiple institutions and are predominantly little to no cost for the families. This relationship is mutually beneficial; data from patient families inform researchers and clinicians. In turn, researchers and clinicians use these findings to support other families. Over the last five years, many of the resources developed and/or utilized by the DIPG community have been successful. Since May 2021, 42% of post-mortem tissue donations through Gift From a Child have been DIPG or DMG. Data from this tissue is shared openly through the Children’s Brain Tumor Network, ensuring that a diverse patient population is represented in future research projects. DIPG One-link helps patient families navigate vetted resources for care at any point after diagnosis. Resources include patient navigators, information about research and clinical trials, emotional and grief support, places to safely share their data, and referrals to the DIPG/DMG National Tumor Board. All of these resources have shown an overall increase in use since implementation. There has also been an increase in access by patient populations and care teams that previously would have been unable to access such important resources. The direct involvement of patient families ensures that patient voices are represented and valued. These resources are essential to removing institutional, financial, geographic, and health literacy barriers, and ensuring all future patients have equitable access to care.
OTHR-26. MINIMALLY-INVASIVE MOLECULAR DIAGNOSIS OF DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR (DLGNT) USING CEREBROSPINAL FLUID CELL-FREE DNA SEQUENCING IN PEDIATRIC AND YOUNG ADULT PATIENTS
Authors: Larsen Alexandra M Giantini , Hill Katherine , Hickman Richard , Bale Tejus A , Holle Bridget , Mellinghoff Ingo , Dunkel Ira , Souweidane Mark , Greenfield Jeffrey , Sait Sameer Farouk , Miller Alexandra M , Karajannis Matthias A
Published: June 2024

BACKGROUND

Diffuse leptomeningeal glioneuronal tumor (DLGNT) presents with nodular leptomeningeal disease throughout the neuroaxis and is characterized by the presence of KIAA1549::BRAF fusion and chromosome arm 1p deletion. Concomitant gain of chromosome arm 1q is associated with poor prognosis. Diagnosis by meningeal biopsy is often non-diagnostic due to sampling error or yields insufficient material for molecular testing. We hypothesized that analysis of cell free DNA (cfDNA) from cerebrospinal fluid (CSF) could represent a superior diagnostic modality.

METHODS

We analyzed CSF samples from 9 suspected DLGNT patients, median age of 19.5 years with a 5:4 male:female ratio using MSK-IMPACT, a hybridization capture-based next-generation DNA sequencing panel, including matched germline.

RESULTS

Prior tissue biopsy was done in 7 patients; 6 were negative for the KIAA1549:BRAF fusion and 1 was positive. Of the 6 biopsies negative for the fusion, analysis of CSF cfDNA detected KIAA1549::BRAF fusion in 3/6 (50%) of patients, who were all subsequently started on MEK inhibitor therapy. The other 3 patients were negative for KIAA1549:BRAF fusion on both biopsy and CSF cfDNA. One patient received biopsy confirmation of KIAA1549::BRAF fusion after CSF cfDNA analysis detected the mutation. In addition, in one patient with prior tissue-confirmed KIAA1549::BRAF fusion, the fusion was not detected in CSF cfDNA during/after treatment with a MEK inhibitor, but additional somatic alterations and DNA copy number profiles were similar to those found in prior biopsies. Additional somatic alterations were identified in ATRX, BCOR, RARA, STAT5A, SPOP, PPM1D, MED12, KMT2C, RECQL andSETD2, including likely oncogenic secondary driver alterations that have not been previously reported in DLGNT.

CONCLUSIONS

Given the high molecular diagnostic yield and concordance with open biopsy, cfDNA CSF sequencing should be considered as a first line diagnostic maneuver for patients with suspected DLGNT, followed by surgical biopsy only if CSF testing is non-diagnostic.
PATH-03. FOXR2-OVEREXPRESSING CENTRAL NERVOUS SYSTEM (CNS) TUMORS EXHIBIT SUBSTANTIAL DIVERSITY ACROSS HISTOLOGICAL, MOLECULAR, AND CLINICAL DIMENSIONS
Authors: Hanzlik Emily , Siskar Alexa N , Eldomery Mohammad K , Pinto Soniya , Tinkle Christopher L , Zhang Qunyu , Cardenas Maria F , Dhanda Sandeep K , Li Xiaoyu , Robinson Giles W , Wheeler David A , Blackburn Patrick R , Chiang Jason
Published: June 2024

BACKGROUND

FOXR2 activation is regarded as the pathognomonic molecular signature of CNS neuroblastoma. However, a comprehensive understanding of the landscape for CNS tumors exhibiting FOXR2 activation is lacking.

METHODS

Integrated histopathologic, molecular, and clinical data analysis of 36 FOXR2-overexpressing CNS tumors identified through screening of an institutional dataset.

RESULTS

FOXR2-overexpressing CNS tumors showed a broad anatomic distribution throughout the neuraxis. Of the 36 tumors, 17 (47.2%) were gliomas and 17 (47.2%) were embryonal tumors. The gliomas included 16 high-grade gliomas (eight histone H3 K27M-mutant diffuse midline gliomas, seven radiation-associated tumors, and one with MN1::PATZ1 fusion) and one pilocytic astrocytoma. The embryonal tumors included ten CNS neuroblastomas, six pineoblastomas, and one medulloblastoma. Two were difficult-to-classify high-grade neuroepithelial tumors. The gliomas and embryonal tumors demonstrated comparable FOXR2 expression based on normalized RNA-sequencing data. FOXR2-overexpressing gliomas, but not embryonal tumors, displayed diverse concomitant genetic alterations. The most common mechanisms of FOXR2 activation involved structural alterations leading to proximity effects, frequently incorporating non-canonical non-coding exons, followed by activation of the exon -3 promoter. Tumor location, histology, and concomitant alterations correlated with patient outcomes. While FOXR2-overexpressing gliomas were aggressive with a 5-year overall survival of 13.3%, FOXR2-overexpressing embryonal tumors showed a diverse prognosis. FOXR2-activated CNS neuroblastoma showed a 5-year overall survival of 100%. Pineoblastomas with FOXR2 activation in the PB-FOXR2 DNA methylation group showed a 3-year survival of 0% despite multimodal therapy. The one medulloblastoma patient in the cohort is alive at three years post-diagnosis.

CONCLUSION

FOXR2-overexpressing CNS tumors manifest significant histological, molecular, and clinical diversity and can occur as post-radiation tumors. Glioma histology and concomitant genetic alterations correlate with treatment resistance and inferior prognosis, while FOXR2-overexpressing embryonal tumors show more heterogeneous outcomes. Integrating histologic and molecular diagnostic approaches along with therapeutic interventions and clinical outcomes is imperative for accurate prognostication and optimal therapeutic decision-making.
PATH-05. MACHINE LEARNING-BASED CYTOLOGICAL ANALYSIS OF CEREBROSPINAL FLUID IN MEDULLOBLASTOMA
Authors: Maack Lennart , Kresbach Catena , Neumann Julia , Tischendorf Jacqueline , Wefers Annika , Seegerer Philipp , Schüller Ulrich , Schlaefer Alexander , Bockmayr Michael
Published: June 2024

BACKGROUND

Metastatic dissemination occurring via the cerebrospinal fluid contributes to the poor prognosis in many medulloblastoma patients. It has high prognostic relevance, and its accurate detection is critical for adequate therapy stratification. However, cytological examination is a difficult, time-consuming task and frequently unreliable. We, therefore, propose a machine-learning pipeline to solve this diagnostic challenge.

METHODS

A dataset of 303 digitized images of cerebrospinal fluid preparations from 81 medulloblastoma patients was assembled. Based on these data, over 50000 digitized objects, including more than 5000 patches with tumor cells, were classified by experts into 13 clinically relevant diagnostic categories and used for training. The classification was performed using convolutional neural networks, trained with nested-cross validation and different transfer learning strategies.

RESULTS

The classification into the 13 diagnostic categories was feasible, with an overall accuracy of 75% on the test set and a tumor-specific F1 score of 89%. Most misclassifications occurred between morphologically similar cell types, mainly lymphocytes and activated lymphocytes, monocytes and activated monocytes, as well as tumor cells and atypical cells with borderline cytomorphology. Grouping these cytologically and/or biologically closely related groups increased the overall accuracy to 91%. Pretraining on published cytological datasets of cerebrospinal fluid and bone marrow further increased classification accuracy.

CONCLUSIONS

Deep learning can reliably detect tumor cells in digitized cytological images from cerebrospinal fluid preparations in medulloblastoma. Implementing explanation methods, an easy-to-use user interface and validation on additional cohorts will further strengthen the robustness of the presented approach.
PATH-13. LEARNED RESIZING WITH EFFICIENT TRAINING (LRET) FACILITATES IMPROVED PERFORMANCE OF LARGE-SCALE BRAIN TUMOR HISTOLOGY IMAGE CLASSIFICATION MODELS
Authors: Orr Brent A , Alom Zahangir , Tran Quyhn T
Published: June 2024

BACKGROUND

Histologic examination is vital in oncology research and diagnostics. The adoption of digital scanning of whole slide images (WSI) has created an opportunity to leverage deep learning-based image classification methods to enhance diagnosis and risk stratification. However, technical limitations prevent training and deployment of accurate comprehensive multiclass deep convolutional neural networks (DCNN) models for histopathology image classification. The input dimensions of DCNN architectures are small compared to the typical pathologist field of view, degrading performance by excluding important architectural features. Furthermore, data requirements for comprehensive models are sufficiently large to overwhelm the system memory during training.

METHODS

A method termed Learned Resizing with Efficient Training (LRET) was developed to address the main limitations of traditional histopathology classification model training. The LRET method couples efficient training techniques with image resizing to facilitate seamless integration of larger histology image patches into state-of-the-art classification models while preserving important structural information. The LRET method was coupled with two distinct resizing techniques to train three diverse histology image datasets using five different DCNN architectures. Performance metrics were compared on cross validation and hold out test sets.

RESULTS

LRET-trained models were flexible to multiple input patch dimensions and DCNN models. We demonstrated performance improvement across all datasets while significantly reducing the training time and resources over traditional methods. Using a large-scale, multiclass brain tumor classification dataset consisting of 74 distinct histopathologic classes, LRET-trained models outperformed existing methods by 15-28% in accuracy, yielding 94% accuracy for the best model.

CONCLUSION

The LRET method for DCNN training significantly enhances the performance of large-scale multiclass histopathology image classification. The implications of this work extend to broader applications within medical imaging and beyond, where efficient integration of high-resolution images into deep learning pipelines is paramount for driving advancements research and clinical practice.