Survivors Of Childhood Brain Tumors Research Articles

OS10.6.A Supportive care needs of TYA childhood brain tumour survivors and their caregivers: a mixed methods study

Abstract BACKGROUND Childhood brain tumour survivors and their family caregivers can experience many late effects of treatment including social, cognitive and physical issues. Yet, the supportive care needs of survivors, now teenagers and young adults (TYAs), and their caregivers population are largely unknown. We aimed to gain an in-depth understanding of this populations’ supportive care needs. MATERIAL AND METHODS This study used a convergent mixed methods design including quantitative (survey) and qualitative data (in-depth semi-structured interviews). Participants were recruited from long-term follow-up clinics (in three NHS Trusts in England) and online. Participants included childhood brain tumour survivors, at least five years from diagnosis, currently aged 13–30 and their primary caregivers. The results from quantitative and qualitative data were integrated using a Joint Display Table. RESULTS 136 eligible survivors and caregivers (78 survivors/58 caregivers) were approached to take part in the survey. In total, 112 participants (69 survivors/43 caregivers) completed the survey. A further 22 participants took part in face-to-face semi-structured interviews (11 survivors/11 caregivers). The integrated findings indicate that both survivors and caregivers have unmet needs many years after diagnosis. TYA survivors specifically had high unmet needs in relation to their psychological health, social lives (including romantic relationships), employment, and independence. Caregivers experienced even more unmet needs - including regarding their own psychological well-being and survivors’ financial issues. Survivors further from diagnosis, unemployed survivors and single caregivers were more likely to report unmet needs. Barriers preventing survivors and caregivers accessing supportive services were highlighted, including (but not exclusive to) families not being aware of support available, location of services, and accessibility to information/support. CONCLUSION This research provides leads to improving supportive care and long-term follow-up services. Understanding unmet needs and recognising what services are required is critical to improving survivor and caregiver quality of long-term survival.

Functional Connectivity Networks and Their Recruitment During Working Memory Tasks in Adult Survivors of Childhood Brain Tumors.

Background: Assessments of functional connectivity of default mode network (DMN) and positive task-related networks (TRNs) using independent component analysis (ICA) may help describe long-term effects of childhood brain tumors and adjuvant treatments. Methods: Aiming to identify potential neuronal markers that may aid in prognosis and inform interventions to optimize outcomes, this study used ICA to evaluate the presence of functional connectivity networks and their recruitment during a letter n-back task in 23 adult survivors of childhood posterior fossa tumors (9 low grade, 14 high grade) at least 5 years past diagnosis compared with 40 age- and sex-matched healthy peers. Results: DMN components generally demonstrated increasing disengagement as task difficulty increased, and relationships between effective DMN disengagement and improved performance were observed in healthy controls (HCs). Low-grade brain tumor survivors (LGS) demonstrated unique patterns in DMN recruitment that suggested increased involvement of the medial prefrontal cortex in LGS during tasks. TRN components generally demonstrated increasing engagement, which was related to improved task performance in HCs for one executive control network (ECN) component. High-grade brain tumor survivors (HGS) demonstrated distinct challenges recruiting an ECN component at more difficult task levels and showed a relationship between recruitment of another ECN component and task performance, indicating a potential compensatory mechanism for some HGS. Conclusions: Findings suggest the importance of cognitive intervention in both survivor groups and the necessity to track LGS despite their cognitive abilities often resembling those of their healthy peers. Impact statement Distinct functional connectivity patterns were identified between both adult survivor of childhood brain tumor groups and peers during attention and working memory tasks, reflecting different damage and recovery from treatment. Survivors of low-grade tumors demonstrated unique patterns of recruitment of default mode network components in the context of similar cognitive abilities, whereas survivors of high-grade tumors demonstrated poorer cognitive abilities and may be utilizing compensatory executive control network components in the face of challenging tasks. Long-term clinical follow-up and cognitive remediation is warranted for both groups, including low grade cerebellar tumor patients who have traditionally not been monitored as closely.

Exploring evidence of fatigue in survivors of paediatric brain tumours: a systematic review

AimsAs the number of survivors of childhood brain tumor grows, fatigue is being increasingly recorded as a long-term consequence of both the cancer itself and the treatment received. Survivors of childhood brain tumour report more significant fatigue than children with other cancers, often impacting all aspects of life, including academic attainment, self-concept and social relationships with peers, leading to reduced health-related quality of life.This study aimed to systematically evaluate the evidence for fatigue in paediatric brain tumour survivors.MethodA systematic search using EMBASE, MEDLINE and PsycINFO identified 20 papers meeting the inclusion criteria. Scientific rigor was used throughout by following Scottish Intercollegiate Guidelines Network (2015) guidance for systematic reviews. Quality Assessment of Evidence Rating tool - Fatigue (QAERT) was developed with substantial inter-rater agreement found.Result19 of the 20 studies reviewed showed conclusive evidence of fatigue in survivors of paediatric brain tumour. One study offered adequate evidence that there was no difference in levels of fatigue in paediatric cancer survivors, including survivors of paediatric brain tumour, when compared to healthy controls. Three studies found that fatigue was worse in survivors of paediatric brain tumour when compared to survivors of other paediatric cancersConclusionThis review provides evidence for the presence of fatigue in survivors of paediatric brain tumour. However, the construct of fatigue was poorly defined throughout, with fatigue associated with physical effects of treatment and fatigue associated with long-term cognitive impairment not distinguished. This poor construct validity, coupled with a lack of comparison groups in 12 of the 20 studies, reduces the generalizability of the data and its usefulness for developing effective psychological interventions. Further research is needed, built on a clear fatigue construct definition, and including well defined exclusion criteria, to provide a sound basis for improving the quality of life of these children.

Long-term neuropsychological outcomes of survivors of young childhood brain tumors treated on the Head Start II protocol.

The Head Start treatment protocols have focused on curing young children with brain tumors while avoiding or delaying radiotherapy through using a combination of high-dose, marrow-ablative chemotherapy and autologous hematopoietic cell transplantation (AuHCT). Late effects data from treatment on the Head Start II (HS II) protocol have previously been published for short-term follow-up (STF) at a mean of 39.7 months post-diagnosis. The current study examines long-term follow-up (LTF) outcomes from the same cohort. Eighteen HS II patients diagnosed with malignant brain tumors <10 years of age at diagnosis completed a neurocognitive battery and parents completed psychological questionnaires at a mean of 104.7 months' post-diagnosis. There was no significant change in Full Scale IQ at LTF compared to baseline or STF. Similarly, most domains had no significant change from STF, including verbal IQ, performance IQ, academics, receptive language, learning/memory, visual-motor integration, and externalizing behaviors. Internalizing behaviors increased slightly at LTF. Clinically, most domains were within the average range, except for low average mathematics and receptive language. Additionally, performance did not significantly differ by age at diagnosis or time since diagnosis. Of note, children treated with high-dose methotrexate for disseminated disease or atypical teratoid/rhabdoid tumor displayed worse neurocognitive outcomes. These results extend prior findings of relative stability in intellectual functioning for a LTF period. Ultimately, this study supports that treatment strategies for avoiding or delaying radiotherapy using high-dose, marrow-ablative chemotherapy and AuHCT may decrease the risk of neurocognitive and social-emotional declines in young pediatric brain tumor survivors.

Prevalence and Risk Factors of Hypothalamic-Pituitary Dysfunction in Infant and Toddler Brain Tumor Survivors

Background: Childhood brain tumor survivors (CBTS) are at risk for hypothalamic-pituitary (HP) dysfunction, mainly caused by radiation exposure or tumor involvement of the HP-region. The risk for HP dysfunction (HPD) may vary between different age groups due to maturation of the brain and differences in oncologic treatment protocols. The aim of this study was to determine the prevalence and risk factors of HPD in infant (IBTS) and toddler brain tumor survivors (TBTS) compared to older childhood brain tumor survivors (OCBTS). Patients and Methods: A retrospective analysis in a nationwide cohort of CBTS was performed. Prevalence and risk factors for HPD were compared between IBTS (aged 0-1 years at diagnosis), TBTS (aged 1-3 years at diagnosis) and OCBTS (aged >3-18 years at diagnosis). Results: In 718 included CBTS, with a median follow-up time of 7.9 years, overall no differences in percentage of HPD were found between the three age groups. Treatment with radiotherapy (RT) (OR 15.41; 95%CI 8.33 to 28.48), suprasellar tumor location (OR 46.62; 95%CI 19.64 to 110.66) and younger age (OR 1.09; 95%CI 1.02 to 1.15) were associated with HP dysfunction. Because IBTS were significantly less often treated with RT, subanalyses were performed for all CBTS not treated with radiation (n=459). In non-irradiated CBTS, IBTS and TBTS were significantly more frequently diagnosed with TSH-, ACTH- and ADH deficiency, compared to ECBTS. IBTS and TBTS showed significantly more weight gain (p<0.0001) and smaller height SDS (p=0.001) during follow-up. Conclusion: Infant and toddler brain tumor survivors seem to be more vulnerable to develop HP dysfunction than when compared to older children. These results emphasize the importance of special infant and toddlers brain tumor treatment protocols and endocrine surveillance in children treated for a brain tumor at young age.

Unmet support needs in teenage and young adult childhood brain tumour survivors and their caregivers: \u201cit\u2019s all the aftermath, and then you\u2019re forgotten about\u201d

PurposeTeenage and young adult (TYA) survivors of childhood brain tumours and their family caregivers can experience many late effects of treatment that can hamper the transition to living independent lives. Yet, their long-term supportive care needs are largely unknown. We investigated the supportive care needs of TYA survivors and their caregivers and explored the role and perceived use of support.MethodsFace-to-face semi-structured interviews were conducted with survivors aged 16–30 (n = 11) who were ≥ 5 years after diagnosis and caregivers (n = 11). Interviews were recorded and transcriptions thematically analysed.ResultsFour themes emerged: (1) preferences for support and support services (unmet needs). Concerns regarding mental health, employment and financial uncertainty, the desire to live independently, and lack of support were emphasised. (2) Decline in support. Caregivers noted a drop-off in support available when transitioning to adult services. (3) Reasons for not obtaining adequate support. Several barriers to accessing support were raised, including distance and aging out of services. (4) The role of long-term hospital-based follow-up care. Participants highlighted the importance of, and reassurance from, long-term follow-up care but noted a more all-inclusive approach is required.ConclusionsEven many years after diagnosis, TYA childhood brain tumour survivors and their caregivers continue to have unmet supportive care needs. Both TYA survivors and their caregivers can benefit from support to meet their unique needs and improve long-term quality of life. Understanding unmet needs and recognising what services are required due to the late effects of treatment is critical to improving long-term quality of survival.

High Prevalence of Weight Gain in Childhood Brain Tumor Survivors and Its Association With Hypothalamic-Pituitary Dysfunction.

Childhood brain tumor survivors (CBTS) are at risk for developing obesity, which negatively influences cardiometabolic health. The prevalence of obesity in CBTS may have been overestimated in previous cohorts because of inclusion of children with craniopharyngioma. On the contrary, the degree of weight gain may have been underestimated because of exclusion of CBTS who experienced weight gain, but were neither overweight nor obese. Weight gain may be an indicator of underlying hypothalamic-pituitary (HP) dysfunction. We aimed to study prevalence of and risk factors for significant weight gain, overweight, or obesity, and its association with HP dysfunction in a national cohort of noncraniopharyngioma and nonpituitary CBTS. Prevalence of and risk factors for significant weight gain (body mass index [BMI] change ≥ +2.0 standard deviation score [SDS]), overweight, or obesity at follow-up, and its association with HP dysfunction were studied in a nationwide cohort of CBTS, diagnosed in a 10-year period (2002-2012), excluding all craniopharyngioma and pituitary tumors. Of 661 CBTS, with a median age at follow-up of 7.3 years, 33.1% had significant weight gain, overweight, or obesity. Of the CBTS between 4 and 20 years of age, 28.7% were overweight or obese, compared with 13.2% of the general population between 4 and 20 years of age. BMI SDS at diagnosis, diagnosis of low-grade glioma, diabetes insipidus, and central precocious puberty were associated with weight gain, overweight, or obesity. The prevalence of HP dysfunction was higher in overweight and obese CTBS compared with normal-weight CBTS. Overweight, obesity, and significant weight gain are prevalent in CBTS. An increase in BMI during follow-up may be a reflection of HP dysfunction, necessitating more intense endocrine surveillance.

Neurocognitive impairment, employment, and social status in radiotherapy-treated adult survivors of childhood brain tumors.

BackgroundLittle is known of the cognitive functions, employment, and social status in adult survivors of childhood brain tumor (BT). We aimed to determine the long-term neurocognitive profile of radiotherapy-treated adult survivors of childhood BT and the relationship between cognitive functions and employment and social status.MethodsNeurocognitive profiles of survivors were assessed in a Finnish national cohort of 71 radiotherapy-treated survivors of childhood BT (median follow-up time: 21 years [range: 5-33 years]) using a cross-sectional design. Neurocognitive outcomes were compared to control (n = 45) and normative values. Tumor- and treatment-related data were collected from the patient files. Information on employment and social status was gathered.ResultsSurvivors’ (median age: 27 years [range: 16-43 years]) median verbal and performance intelligence quotient (IQ) was 90 (range: 49-121) and 87 (range: 43-119), respectively. The cognitive domains with the greatest impairment were executive functions (median z score, −3.5 SD [range: −25.0 to 1.3 SD]), and processing speed and attention (median z score, −2.5 SD [range: −24.9 to 0.5 SD]). Executive functions were associated with employment, educational level, living independently, having an intimate relationship, and having a driving license. Processing speed and attention were related to educational level, living independently, having an intimate relationship, and having a driving license. Performance IQ was associated with educational level and employment status. Working memory was associated with educational level and living independently.ConclusionsRadiotherapy-treated adult survivors of childhood BT experience significant neurocognitive impairment, which is associated with difficulties related to employment and social status.

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

BACKGROUNDChildhood 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.METHODSSemi-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.RESULTSAdjusting 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.CONCLUSIONThe 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-46. LATE EFFECTS CARE FOR CHILDHOOD BRAIN TUMOUR SURVIVORS: A QUALITY IMPROVEMENT PROJECT

BACKGROUNDChildhood 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.METHODSAn 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.RESULTS17 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.CONCLUSIONTreatment 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-12. CLINICAL SIGNIFICANCE OF RADIATION-INDUCED CEREBROVASULAR DISEASE IN CHILDHOOD BRAIN TUMOR SURVIVORS

BACKGROUNDChildhood brain tumor survivors have a high risk of early cerebrovascular disease, but currently its clinical significance is unknown.METHODSIn a nation-wide study, we investigated 68 childhood brain tumor survivors treated with radiotherapy by using magnetic resonance imaging (MRI) and neuropsychological examination after median follow-up time of 20.6 years (range 5.0 – 33.1 years) since radiotherapy. Associations between imaging markers of cerebrovascular disease, white matter hyperintensities and the results of neuropsychological examination were investigated.RESULTSMajority (65 %) of the survivors was diagnosed with cerebrovascular disease at median age of 27.1 years (range16.2 – 43.8 years). The presence of imaging markers of cerebrovascular disease or white matter hyperintensities was associated with poorer performance in verbal (VIQ) and performance (PIQ) intelligent quotient, working and semantic memory, executive functions, visuospatial ability, and immediate and general auditive memory (P < 0.05). Survivors with microbleeds performed worse in PIQ, processing speed, executive functions, and visuospatial ability (P <0.05). Lacunar infarcts were associated with difficulties in visuospatial ability (P <0.05). Survivors with white matter hyperintensities in MRI had higher impairment of working and semantic memory, visuospatial ability, and general auditive memory (P < 0.05). Cerebrovascular and small-vessel disease burden associated with poorer neurocognitive performance.CONCLUSIONThe imaging markers of cerebrovascular disease and white matter hyperintensities were related to poorer cognitive performance in radiation-treated long-term survivors of childhood brain tumor. Longitudinal studies are urgently needed to investigate how cerebrovascular disease and related cognitive impairment progress in the survivors.

SWK-06. ANSWERING 900 VOICES: A NATIONAL NONPROFIT ORGANIZATION RESPONDS TO A NATIONWIDE COMMUNITY HEALTH NEEDS ASSESSMENT THAT ELUCIDATED KEY CHALLENGES FACED BY PEDIATRIC BRAIN TUMOR FAMILIES

The five-year relative survival rate for childhood primary brain and other central nervous system tumors is nearly 75 percent (Central Brain Tumor Registry of the United States, 2017). Nevertheless, childhood brain tumor survivors often suffer from lifelong side effects caused by their illness or treatments such as surgery, radiation and chemotherapy. To define the nature and extent of problems survivors and their families face, the Pediatric Brain Tumor Foundation (PBTF), the world’s largest nonprofit solely dedicated to children and teens with brain tumors, conducted a 2017 national community health needs assessment in collaboration with the Sol Price Center for Social Innovation at the University of Southern California. The assessment found that pediatric brain tumor patients and their families face key challenges in four general areas: 1) interpersonal and emotional support, 2) logistical and financial support, 3) information and medical education gathering, and 4) educational and vocational anxieties. In 2020, the PBTF’s response to the 900 assessment participants who represent the thousands living with the effects of this disease includes the launch of a national Peer to Peer Mentoring program to meet the ongoing challenges families face; the disbursement of emergency financial assistance to hundreds of families in the throes of treatment; and the distribution of a resource notebook for newly diagnosed families and a comprehensive guidebook for survivors and their families. The results of the needs assessment suggest additional clear, actionable areas for impact not only by the PBTF but by medical professionals, other nonprofit organizations and governmental agencies.

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

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) <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-02. PERCEPTIONS OF LATE EFFECTS CARE NEEDS AMONG SURVIVORS OF PEDIATRIC BRAIN TUMOURS

OBJECTIVESPediatric brain tumour survivors are at risk of long-term consequences of therapy. Comprehensive late effects care may mitigate these risks, but the best care model is unclear. We sought to describe the care experience and quality of life (QOL) of pediatric brain tumour survivors at the McMaster Children’s Hospital joint adult/pediatric Neuro-Oncology clinic.METHODSCross-sectional survey data were collected. Care needs were assessed with the Cancer Care Experience Questionnaire (CCEQ), Cancer Worry Scale (CWS), and Self-Management Skills Scale (SMSS). Quality of life was measured utilizing the PedsQL Brain Tumor Module. Data were analyzed descriptively.RESULTSThirty-two childhood brain tumor survivors and/or their parents participated. Their malignancies included embryonal tumors (medulloblastoma/ATRT) (62%), ependymoma (22%), and germ cell tumours (16%). Among 77%, therapy included chemotherapy, surgery and radiation. Most respondents reported high quality cancer care, although some could not recall discussions of late effects risks and health promotion. Mean cancer worry scores were low (71.8 (± 28.4)). Survivors reported limited self-management skills (58.5 (±18.2)), with support required in clinic visits, arranging medical appointments, filling prescriptions and tasks of daily living. Overall median QOL scores were in the ‘good’ range (parental report 72.3 (±17.7), survivor 68.2 (±16.6)).CONCLUSIONIn comparison to other childhood cancer survivor cohorts, this group of long-term brain tumour survivors appear to have similar QOL, fewer cancer worries, and increased need for aid with self-management. Given this, along with the positive care experience reported, this clinic model of care appears to meet the needs of this population.

Mothers’ and fathers’ views of family management and health-related quality of life for young adult survivors of childhood brain tumors

Purpose To examine associations between fathers’ and mothers’ appraisals of family management and physical and emotional health-related quality of life (QOL) for young adult survivors of childhood brain tumors. Design Cross-sectional. Sample 47 mothers and 39 fathers (39–67 years old); 47 survivors (18–33 years old) Methods Analyses evaluated relationships among family management (Survivor’s Daily Life, Condition Management Ability, Condition Management Effort, Family Life Difficulty, View of Condition Impact, Parental Mutuality), quality of life, and parental role. Findings Except for Parental Mutuality, family management ratings were not significantly different for mothers and fathers, and parental views of survivors’ physical and emotional QOL improved with better family management. Parental role moderated associations between physical and emotional QOL and Survivors’ Daily Life and between emotional QOL and Condition Management Ability, Condition Management Effort, and View of Condition Impact. Implications for Psychosocial Providers Assess and address survivor QOL through family management from multiple perspectives.

Treatment and Long-Term Sequelae in Childhood Brain Tumors

In children treated for brain tumors, important deficits in cognitive development have been described. The reduction of Intelligence Quotient (IQ) is correlated with multiple conditions such as tumor location, obstructive hydrocephalus, surgical intervention, and above all, the use of radiotherapy, especially in young children. Demyelinization represents the most striking microscopic alteration following radiation: cerebral white matter’s loss and failure to white matter development could partly account for changes in IQ score.Recently, combined chemo-radiotherapeutic approaches and the improvement of radiotherapy techniques have enabled the reduction of neurocognitive symptoms and improved the standard of life of childhood brain tumor survivors.

Systematic review of the incidence and risk factors for cerebral vasculopathy and stroke after cranial proton and photon radiation for childhood brain tumors.

The aim of our study is to determine the incidence, timing, and risk factors for cerebral vasculopathy after cranial proton and photon radiation for pediatric brain tumors. We performed a single-institution retrospective review of a cohort of children treated with proton radiation for brain tumors. MRA and/or MRI were reviewed for evidence of cerebral vascular stenosis and infarcts. Twenty-one similar studies (17 photon, 4 proton) were identified by systematic literature review. For 81 patients with median follow-up of 3 years, the rates of overall and severe vasculopathy were 9.9% and 6.2% respectively, occurring a median of 2 years post radiation. Dose to optic chiasm greater than 45 Gy and suprasellar location were significant risk factors. Results were consistent with 4 prior proton studies (752 patients) that reported incidence of 5% to 6.7%, 1.5 to 3 years post radiation. With significantly longer follow-up (3.7-19 years), 9 studies (1108 patients) with traditional photon radiation reported a higher rate (6.3%-20%) and longer time to vasculopathy (2-28 years). Significant risk factors were neurofibromatosis type 1 (NF-1; rate 7.6%-60%) and suprasellar tumors (9%-20%). In 10 studies with photon radiation (1708 patients), the stroke rate was 2% to 18.8% (2.3-24 years post radiation). Childhood brain tumor survivors need screening for vasculopathy after cranial radiation, especially with higher dose to optic chiasm, NF-1, and suprasellar tumors. Prospective studies are needed to identify risk groups, and ideal modality and timing, for screening of this toxicity.

Reassuring quality of life in younger childhood (&amp;lt;4 y) brain tumor survivors treated with proton beam therapy

Reassuring quality of life in younger childhood (&amp;lt;4 y) brain tumor survivors treated with proton beam therapy

The Neurological Predictor Scale Predicts Adaptive Functioning via Executive Dysfunction in Young Adult Survivors of Childhood Brain Tumor.

Survivors of childhood brain tumors experience neurological sequelae that disrupt everyday adaptive functioning (AF) skills. The Neurological Predictor Scale (NPS), a cumulative measure of tumor treatments and sequelae, predicts cognitive outcomes, but findings on its relation to informant-reported executive dysfunction (ED) and AF are mixed. Given known effects of frontal-subcortical system disruptions on AF, this study assessed the NPS' relationship with AF as mediated by frontal systems dysfunction, measured by the Frontal Systems Behavior Scale (FrSBe). 75 participants (Mage = 23.5, SDage = 4.5) were young adult survivors of childhood brain tumors at least 5 years past diagnosis. FrSBe and Scales of Independent Behavior-Revised (SIB-R), a measure of AF, were administered to informants. Parallel multiple mediator models included Apathy and ED as mediators, and age at diagnosis and time between diagnosis and assessment as covariates. More complex treatment and sequelae were correlated with poorer functioning. Mediation models were significant for all subscales: Motor Skills (MS), p=.0001; Social Communication (SC), p=.002; Personal Living (PL), p=.004; Community Living (CL), p=.007. The indirect effect of ED on SC and CL was significant; the indirect effect of Apathy was not significant for any subscales. More complex tumor treatment and sequelae were associated with poorer long-term AF via increased ED. Cognitive rehabilitation programs may focus on the role of executive function and initiation that contribute to AF, particularly SC and CL skills, to help survivors achieve comparable levels of independence in everyday function as their peers.

Long-term Functional Outcome in Young Adult Survivors of Childhood Brain Tumor

Background and Objective: The late effects of childhood brain tumors (BTs) are extensively described in the literature. However, their effect on independent living skills of survivors is not. Therefore, the aim of this study is to assess the impact of late effects of childhood BT and related treatment on the performance in daily living activities of survivors. Furthermore, we wanted to determine which factors are likely to be associated with performance limitation. Methods: A cross-sectional study was conducted. Setting: Follow-up clinic in a tertiary care university hospital. Patients: Young adult survivors of childhood BT. Intervention and Measurements: No intervention was delivered. Performance in activities of daily living (ADL) was measured by the Assessment of Motor and Process Skills 5 years or more after diagnosis. Sociodemographic and medical information was also collected. Results: Thirty-six young adults, mean age 21.0 years (range, 16-29 years) and mean time since completion of treatment 10.1 years (range, 4.0-18.0 years), participated in this study. Results showed that 55% of the participants had results under the motor cutoff and 36% under both the motor and process cutoffs representing the lower limit of competent ADL task performance. Lower level of functioning was associated with younger age at diagnosis for process skills and tumor location (odds ratio [OR] = 9.0; 95% confidence interval [CI], 1.97-41.08), female gender (OR = 5.14; 95% CI, 1.18-22.48), longer time since treatment (OR = 0.2; 95% CI, 0.05-0.08), and multiple chronic health conditions (OR = 0.06; 95%CI, 0.01-0.51) for motor skills. Limitations: The study design does not allow to make causal inference. Conclusions: Five years or more after diagnosis, survivors of pediatric BT show decreased motor and process skills affecting their performance in ADL. Recommendations from this study include the development of effective rehabilitation and prevention programs to optimize their functional outcome and to target patients at heightened risk for follow-up.