bluuebunny/crossref_metadata_2025_split huggingface dataset:
QOL-23. ASSESSING THE IMPACT OF METHYLPHENIDATE ON LATE COGNITIVE EFFECTS IN PAEDIATRIC BRAIN TUMOUR SURVIVORS: A SERVICE-BASED EVALUATION
Authors: Verity Sarah , Hill Rebecca , Halliday Gail , Ryles Jade , Bailey Simon 
Published: December 2020

Abstract

OBJECTIVE
One of the most disabling side effects of treatment in survivors of brain tumours is the resultant reduction in level of processing speed and attention. This study aimed to evaluate intellectual and psychological benefit of short-acting methylphenidate to survivors of brain tumour.

METHODS
Paediatric BT patients attending a UK specialist treatment centre received assessment of cognitive performance. All patients identified with attentional difficulties were screened for contraindications to methylphenidate. Participants (N=23), mean age 11.09 years, completed a 6-month trial of methylphenidate. Measures of attention (Test of Everyday Attention for Children 2; SNAP-IV), side-effects (Stimulant Side-Effects Rating Scale), Health-Related Quality of Life (PEDS-QL), and experience of methylphenidate questionnaire (purpose-developed semi-structured questionnaire) were administered prior to medication and after six months.

RESULTS
Participants showed improvement in selective attention (t(18)=-5.4, p=&lt;.001, d=.93) and processing speed (t(16)=-3.0, p=.01) at follow up. Family ratings of attention were significant (t(17)=14.46, p&lt;.001, d=-1.19). Change in subjective measures of Health-Related Quality of Life (HRQoL) was also statistically significant as reported by children (t(16)=3.91, p=.001, d=-.99), and on a parental-report measure of child HRQoL (t(15)=-8.19, p&lt;.001, d=-1.09). HRQoL measures show improvement to physical, academic, and emotional domains as reported by participants.

CONCLUSIONS
Paediatric BT survivors showed benefit from provision of methylphenidate in terms of reduced attentional and processing deficit, and in terms of emotional wellbeing. Treatment was well tolerated. Continued follow-up of the current participants in a longitudinal study aims to evidence longer-term benefit to participants.
QOL-24. DIFFERENTIAL IMPACT OF TUMOR LOCATION, LOCAL AND CRANIOSPINAL IRRADIATION ON NEUROPSYCHOLOGICAL LONG-TERM OUTCOME IN CHILDREN WITH MEDULLOBLASTOMA, EPENDYMOMA AND SUPRATENTORIAL PNET: A LONGITUDINAL MULTICENTER OUTCOME ASSESSMENT OF CHILDREN FROM THE HIT-2000 AND HIT-REZ TRIALS
Authors: Ottensmeier Holger , Schlegel Paul G , Eyrich Matthias , Zimolong Bernhard , Mynarek Martin , von Hoff Katja , Frahsek Stefanie , Schmidt Rene , Faldum Andreas , Wolff Johannes , Fleischhack Gudrun , Warmuth-Metz Monika , Krauss Jürgen , Kortmann Rolf-Dieter , Galley Niels , Kühl Joachim , Rutkowski Stefan 
Published: December 2020

Abstract
Neurocognitive deficits are frequent in childhood brain tumor survivors and affect mental intelligence, psychomotor and executive abilities. The differential impact of factors such as disease (location, histology) or treatment (local (LI) vs. craniospinal irradiation (CSI)) on these parameters is not fully understood. Between 2007–2011 and 2013–2017 300 testings were performed on-site by one neuropsychologist. Of these, 274 tests from n=208 children with medulloblastoma (MB), ependymoma (EP) and supratentorial embryonal tumors (SET) &lt;4 years at diagnosis are currently included into the analysis. Applied tests included the Bayley II, WUEP-KD, K-ABC, tapping speed (TS), CPT_Hits/CPT_DT, and, as a new score, CPT_Power which integrates the latter. Treatment consisted of surgery and chemotherapy ± LI/CSI. All children receiving CSI and MB children with LI showed substantial deficits in general intelligence scores. In contrast, children with MB or SET without CSI/LI and those with EP receiving LI performed surprisingly well after 2 and 5 years follow-up. Motor function (TS) was reduced in all children except those with SET without irradiation. Of note, mental processing speed (as measured in CPT_Power) was not essentially reduced in MB and EP patients, indicating that mental processing is less affected than motor speed (TS) in children with infratentorial tumors. In conclusion, our data show that besides the established detrimental effects of CSI on general intelligence, infratentorial tumor location is a main risk factor for motor dysfunction irrespective of irradiation. Appropriate sensitive testing tools are warranted to assess cognitive function without the interfering influence of motor dysfunction.
QOL-28. NEUROCOGNITIVE PROFILE OF PEDIATRIC MEDULLOBLASTOMA PATIENTS PRIOR TO RADIATION THERAPY
Authors: Baig Muhammad , Osthoorn Ineke , McGovern Susan , Grosshans David , McAleer Mary , Woodhouse Kristina , Paulino Arnold , Yang Grace , Stavinoha Peter , Zaky Wafik 
Published: December 2020

Abstract
Neurocognitive late effects are unfortunately common following treatment for pediatric medulloblastoma. While radiation therapy is an essential component of treatment for most pediatric medulloblastoma patients, it is associated with neurocognitive compromise. Effects include deficits in cognitive speed and performance efficiency, aspects of attention, as well as working memory. Yet long after treatment it is difficult to tease apart relative contributions of other risk factors to neurocognitive functioning beyond radiation. We examined neurocognitive functioning in a sample of pediatric medulloblastoma patients prior to radiation therapy, including investigation of neurocognitive risk factors such as hydrocephalus, presence of posterior fossa syndrome, and duration of neurological symptoms prior to diagnosis. Results indicated that the sample functioned in the average range in terms of overall IQ (n=34, X̅=103). Patients also functioned in the normal range in terms of language-based ability (X̅=106), nonverbal ability (X̅=104), and working memory (X̅=103). However, the sample performed statistically significantly lower than the general population in terms of cognitive speed and efficiency (z=-2.026, p=.043). The sample was also rated by parents as exhibiting more attention problems relative to the general population (z=1.988, p=.047). There was no specific association with hydrocephalus, duration of symptoms, or history of posterior fossa syndrome. Results suggest weaknesses in attention and processing speed may exist in some pediatric medulloblastoma patients prior to radiation therapy secondary to tumor and related complications. Implications for future research are presented, along with difficulties inherent to “baseline” assessment with pediatric brain tumor survivors.
QOL-32. THE PROMOTE STUDY: HEALTH-RELATED QUALITY OF LIFE COMMUNICATION NEEDS OF CHILDREN, ADOLESCENTS, AND THEIR FAMILIES ATTENDING OUTPATIENT CONSULTATIONS AFTER TREATMENT FOR A BRAIN TUMOUR
Authors: Stubley Shelly , Freeman Anita , Liossi Christina , Darlington Anne-Sophie , Grootenhuis Martha , Hargrave Darren , Morris Christopher , Walker David , Kennedy Colin , Bull Kim 
Published: December 2020

Abstract

BACKGROUND
Childhood brain tumours and their treatment can reduce health-related quality of life (HRQoL) and cause anxiety and depression, withdrawal, and social isolation. Improved communication within outpatient consultations may allow early identification and treatment of these issues. We explored family communication needs in survivors of childhood brain tumours receiving six-monthly follow-up outpatient review within the English NHS.

METHODS
Semi-structured interviews were conducted with 18 families whose child aged 8–17 years had finished treatment for a brain tumour within the preceding five years. Thematic analysis used the Framework Method.

RESULTS
Adjusting to change and finding a “new normal” was the overarching theme to emerge. HRQoL issues included fatigue, coping with physical changes, challenges at school, isolation, and adjusting to changes in abilities. Survivors described a need for greater knowledge about and more support with changes in cognitive functioning. Parents spoke about the impact on the wider family and their changed role in supporting the child’s HRQoL. Communication barriers included short-term memory loss, shyness, and the need to suppress or regulate emotions evoked by these issues. Communication needs included more information regarding recovery and rehabilitation and/or help managing anxiety or emotional health.

CONCLUSION
The above communication needs and barriers should be addressed. Having a digital record to document and monitor this information systematically could improve service planning and provide patients and their families with the resources to reach their full potential and experience a better HRQoL.
QOL-34. CAREER FAIR AND RESOURCE EXPO: ADVOCATING FOR THE LONG TERM SUCCESS OF BRAIN TUMOR SURVIVORS
Authors: Hoerig Clay , Allen Karlie , Noskoff Kara , Frediani Jamie , Pathare Jody , Koerner Casey , DeRosa Veronica , Madrid Nina , Miller Kristin , Mucci Grace , Abongwa Chenue , Plant Ashley 
Published: December 2020

Abstract
Pediatric cancer survivors have increased unemployment and lower educational attainment rates. This is most significant in brain tumor survivors who show five-fold relative odds increase in unemployment over other pediatric cancer survivors. The long-term effects of brain tumor treatment potentiate the difficulty with work and school reintegration seen in the broader Adolescent and Young Adult (AYA) population. To address this, our team designed an annual job fair for AYA Neuro-Oncology survivors. Vendors were invited representing disability advocacy groups, legal services, scholarship organizations, and employers with strong disability services, several who offered on-site interviews. Additionally, brain tumor survivors served as inspirational speakers for the event. Between thirty to forty survivors have attended each event. Pre- and post-surveys, as well as 3- and 6- month follow up was obtained. Universally, the day was engaging and motivating, both for survivors and staff, and stimulated conversation for pursuing career or academic success within families and the care team. While all the patients took applications, none of the patients completed the on-site interviews, finding them overwhelming. Even at the 3- and 6-month follow-ups following the first event, the survivors continued to be at varying levels of application completion; no one who was previously unemployed attained new employment. This improved after pre-event meetings were held with survivors to participate in resume building and interview preparation. Currently, two survivors obtained employment and are still employed at 1 year and five survivors were able to advocate for their disabilities services in college with help of a non-profit legal assistant.
QOL-46. LATE EFFECTS CARE FOR CHILDHOOD BRAIN TUMOUR SURVIVORS: A QUALITY IMPROVEMENT PROJECT
Authors: Cacciotti Chantel , Fleming Adam , Duckworth JoAnn , Tseitlin Hanna , Anderson Loretta , Marjerrison Stacey 
Published: December 2020

Abstract

BACKGROUND
Childhood and adolescent brain tumor survivors are at risk for considerable late morbidity and mortality from their disease and the treatment they receive. Surgery, chemotherapy, radiation therapy and tumor location all have the potential to impact the physical, psychological, functional and social health of these survivors. Comprehensive late effects care may mitigate these risks, but the necessary elements of this care model is unclear. We describe a quality-improvement initiative to improve the long-term follow-up (LTFU) care provided to brain tumour survivors at the McMaster Children’s Hospital.

METHODS
An anonymous needs assessment circulated to health providers was used to evaluate the LTFU practices. Utilizing this feedback as well as the LTFU guidelines from the Children’s Oncology Group a care plan was made for these survivors.

RESULTS
17 of 33 (52%) health care staff responded to the survey, this included 70% physicians or nurse practitioners, and 30% nurses and allied health staff. Improvements suggested included consistent inclusion of additional care providers (i.e. social work, dietitians, endocrinology) reported by 76%, as well as a need for improved patient education and surveillance for late effects of therapy.

CONCLUSION
Treatment summaries with surveillance care plans and LTFU resources were created for all survivors of childhood brain tumours at risk of treatment-related complications. Late effects counselling with distribution of these materials is ongoing as part of this quality improvement initiative. To provide comprehensive management, a neuro-oncology specific late effects programs with multi-disciplinary support is essential for the care of brain tumour survivors.
QOL-49. THE IMPACT OF OTOTOXICITY AND VISUAL IMPAIRMENT ON EDUCATION IN CHILDREN TREATED FOR CNS TUMOURS
Authors: Dulson Harriet , McAndrew Rachel , Brougham Mark 
Published: December 2020

Abstract

INTRODUCTION
Children treated for CNS tumours experience a very high burden of adverse effects. Platinum-based chemotherapy and cranial radiotherapy can cause ototoxicity, which may be particularly problematic in patients who have impaired vision and cognition as a result of their tumour and associated treatment. This study assessed the prevalence of impaired hearing and vision and how this may impact upon education.

METHODS
53 patients diagnosed with solid tumours in Edinburgh, UK between August 2013–2018 were included in the study. Patients were split into three groups according to treatment received: Group 1 – cisplatin-based chemotherapy and cranial radiotherapy; Group 2 - platinum-based chemotherapy, no cranial radiotherapy; Group 3 – benign brain tumours treated with surgery only. Data was collected retrospectively from patient notes.

RESULTS
Overall 69.5% of those treated with platinum-based chemotherapy experienced ototoxicity as assessed by Brock grading and 5.9% of patients had reduced visual acuity. Patients in Group 1 had the highest prevalence of both. 44.4% of patients in Group 1 needed increased educational support following treatment, either with extra support in the classroom or being unable to continue in mainstream school. 12.5% of Group 2 patients required such support and 31.3% in Group 3.

CONCLUSIONS
Children with CNS tumours frequently require support for future education but those treated with both platinum-based chemotherapy and cranial radiotherapy are at particular risk, which may be compounded by co-existent ototoxicity and visual impairment. It is essential to provide appropriate support for this patient cohort in order to maximise their educational potential.
QOL-51. LISTENING BEFORE WE SPEAK: A PATIENT-CENTERED APPROACH TO DEVELOPING RESOURCES FOR PEDIATRIC BRAIN TUMOR SURVIVORS AND THEIR FAMILIES
Authors: Riley Kathy 
Published: December 2020

Abstract
In the United States, more than 28,000 children and teenagers live with the diagnosis of a primary brain tumor (Porter, McCarthy, Freels, Kim, & Davis, 2010). In 2017, an estimated 4,820 new cases of childhood primary brain and other central nervous system tumors were expected to be diagnosed in children ages 0 – 19 in the United States (Central Brain Tumor Registry of the United States, 2017). Survivors suffer from lifelong side effects caused by their illness or by various treatments. Commonly identified late effects of treatment include a decline in intellectual functioning and processing speed, performance IQ deficits, memory deficits, psychological difficulties, deficits in adaptive functioning (daily life skills), and an overall decrease in health-related quality of life (Castellino, Ullrich, Whelen, & Lange, 2014). To address the ongoing challenges these survivors and their families face, the Pediatric Brain Tumor Foundation (PBTF) met extensively with working groups comprised of survivors and caregivers to develop the outline for a comprehensive Survivorship Resource Guidebook. In 2019, the PBTF published the guidebook which categorizes survivor and caregiver needs into three primary areas: physical and mental health, quality of life, and working the system. Expert authors included survivors and caregivers themselves in addition to medical and mental health professionals. Key outcomes discovered during the creation and production of this resource highlight how caregivers, survivors and professionals can collaborate to provide needed information and practical help to one segment of the pediatric cancer population who experience profound morbidities as a result of their diagnosis and treatment.
RARE-02.RE-IRRADIATION FOR RECURRENT CRANIOPHARYNGIOMA
Authors: Foran Sarah , Laperriere Normand , Edelstein Kim , Janzen Laura , Ramaswamy Vijay , Shultz David , Gentili Fred , Bouffet Eric , Tsang Derek 
Published: December 2020

Abstract

PURPOSE
Patients with recurrent craniopharyngioma after radiotherapy (RT) have few treatment options. At our institution, re-irradiation has been offered to selected individuals with recurrent craniopharyngioma not suitable for further surgery, intracystic therapy or targeted agents.

METHODS
A retrospective study was performed of patients with craniopharyngioma treated with two courses of fractionated RT. First RT (RT1) prescriptions ranged from 50–54 Gy in 25–30 fractions; re-irradiation (RT2) prescriptions were 54 Gy in 30 fractions with full, in-field overlap of dose. The maximum dose to organs-at-risk (brainstem, optic structures) were maintained at or below the prescription dose. There was no cumulative dose limit to any structure.

RESULTS
We identified four patients. Median RT1-to-RT2 interval was 5.8 years (range, 4.7–20.4). Cumulative maximum doses to optic chiasma and nerves were &gt;100 Gy in all four patients. With a median follow-up of 33 months after RT2, three patients had disease control and are alive at 9, 23 and 42 months from RT2; one patient developed progressive disease and died 33 months after RT2. In three evaluable patients, vision remained stable or improved after RT2; the remaining one patient had no light perception prior to re-irradiation. Two patients had neuropsychological testing before and after RT2; neurocognitive domains were generally stable in one patient but working memory declined in the second patient.

CONCLUSIONS
Despite exceeding usual tolerances for optic chiasm and nerves, visual outcomes were stable in all living patients. Re-irradiation should be discussed as a treatment option for patients with recurrent craniopharyngioma but without other therapeutic options.
RARE-03. AGGRESSIVE RESECTION FOR PEDIATRIC CRANIOPHARYNGIOMAS VIA ENDOSCOPIC ENDONASAL APPROACH
Authors: Morisako Hiroki , Goto Takeo , Ohata Hiroki , Sakamoto Hiroaki , Ohata Kenji 
Published: December 2020

Abstract

OBJECTIVE
In recent years, the endoscopic endonasal approach (EEA) has been increasingly used for pediatric craniopharyngiomas. We here present our experience and the outcomes of the EEA resection of pediatric craniopharyngiomas.

MATERIALS AND METHODS
Between April 2014 and December 2019, 16 cases of pediatric craniopharyngiomas were operated at the Osaka city university (OCU) hospital. Eight patients were diagnosed with primary craniopharyngiomas while 8 had a recurrent tumor. There were 5 males and 11 females, with a mean age of 10.7 years (3–17 years). EEA was selected in all patients and a case of large muti-lobulated tumor was resected by combination of microscopic transcranial approach.

RESULTS
Gross total resection was achieved in 14 patients and near total resection in other 2. Post-op CSF leak occurred in 3 patients, which was treated with re-exploration. Pituitary stalk was preserved intraoperatively in 4 cases, and 15 patients developed diabetes insipidus and anterior hormonal replacement therapy was required in 15 patients at last follow-up. Visual improvement was noted in 4 patients while vision remained unchanged in the rest. Neuropsychological function status was preserved in all patients, and there was no new-onset obesity postoperatively. The mean follow-up duration was 35.1 months (2 – 69 months) and 4 of 8 recurrent cases had re-recurrence during this period, however there was no recurrent in 8 primary cases.

CONCLUSIONS
EEA should be the surgical modality of choice for treating pediatric craniopharyngiomas. It results in better visual and cognitive outcomes with a significantly increased extent of resection.
RARE-09. PRESERVATION OF ENDOCRINE FUNCTION AFTER OMMAYA RESERVOIR INSERTION IN CHILDREN WITH CYSTIC CRANIOPHARYNGIOMA
Authors: Lohkamp Laura-Nanna , Kulkarni Abhaya , Drake James , Rutka James T , Dirks Peter , Taylor Michael , Ibrahim George , Baroni Lorena , Hamilton Jill , Bartels Ute Katharina 
Published: December 2020

Abstract

INTRODUCTION
Children with craniopharyngiomas (CP) can be subjected to significant morbidities caused by radical surgery and/or radiation with severe long-term consequences. Ommaya reservoir Insertion (ORI) into cystic CP represents a minimally invasive procedure that aims to preserve endocrine, hypothalamic and neurocognitive function. The purpose of this study was to determine the relevance of upfront ORI (+/- intracystic treatment) for preservation of endocrine function.

METHODS
A retrospective chart review of children with CP treated at the Hospital for Sick Children between 01/01/2000 and 15/01/2020 was undertaken. Endocrine function was reviewed at the time of initial ORI or surgical resection and throughout the course of follow-up. Event free survival (EFS) was defined as the time to additional surgical resection or irradiation.

RESULTS
Fifty-five patients with sufficient endocrine follow-up data were included. The median age of diagnosis was 8.3 years (range 2.1–18.0 years), 31 were males. ORI was performed as upfront treatment in 30 patients, gross total or partial resection in 24 patients and radiation in 1 patient, respectively. Endocrine function remained stable after ORI with a median EFS of 19.2 (0 – 105.3) months while the majority of patients who underwent surgical resection had documented worsened endocrine function postoperatively (median of 0; range 0 – 29.4 months) (p&lt; 0.001). The event most commonly related to secondary endocrine deterioration was initial or delayed surgical resection.

CONCLUSIONS
Endocrine function was preserved in patients with upfront ORI (+/- intracystic treatment). Further studies will elucidate the implications of ORI with respect to ophthalmological, vascular and neurocognitive long-term outcome.
RARE-13. INFLAMMATORY MYOFIBROBLASTIC TUMOR MIMICKING DESMOPLASTIC INFANTILE GANGLIOGLIOMA (DIG) OF THE TEMPORAL LOBE
Authors: Abukhiran Ibrahim , Lora Gino Bardi , Kirby Patricia , Shelton Julia , Shilyanski Joel , Wilson Saul , Bossler Aaron , Sato Yutaka , Bashir Amani , Sato Mariko 
Published: December 2020

Abstract
Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm composed of fascicles of myofibroblastic spindle cells in a background of prominent inflammatory infiltrate. It is categorized as ‘intermediate, rarely metastasizing’ in the World Health Organization classification of tumors of soft tissue and bone. We present a novel case of concurrent brain and lung tumor with diagnosis of TFG-ROS1-rearranged IMT in a 14 year old female patient, in which targeted next-generation sequencing became a powerful tool for detection of genomic alterations in both lung and brain tumors. At age 9, the patient’s lung mass was incidentally found and investigated for various infectious diseases with negative result. At age 14, she presented with seizure and was noted to have a stable size lung mass and a left temporal lobe tumor. The left temporal lobe tumor showed a desmoplastic spindle cell neoplasm involving the meninges and cerebral cortex and Desmoplastic Infantile Ganglioglioma (DIG) was considered one of differentials. Subsequently, her right lung mass was resected and showed a similar spindle cell neoplasm with a background of dense fibrosis and chronic inflammation, consistent with Inflammatory Myofibroblastic Tumor. Molecular microdissection revealed that both tumors shares TFG-ROS1 fusion which is associated with (t(3;6) (q12;q22)), thus it is strongly suggestive that two tumors arose from the same origin. No predisposition syndrome was identified.