Adolescent- and adult-onset neuroblastic tumor: A retrospective multicenter observational study of patients diagnosed in France between 2000 and 2020.

BACKGROUND: Adult- and adolescent-onset neuroblastomas are rare, with no established therapy. In addition, rare pheochromocytomas may harbor neuroblastic components. This study was designed to collect epidemiological, diagnostic and therapeutic data in order to better define the characteristics of malignant peripheral neuroblastic tumors (MPNT) and composite pheochromocytomas (CP) with MPNT. PROCEDURE: Fifty-nine adults and adolescents (aged over 15 years) diagnosed with a peripheral or composite neuroblastic tumor, who were treated in one of 17 institutions between 2000 and 2020, were retrospectively studied. RESULTS: Eighteen patients with neuroblastoma (NB) or ganglioneuroblastoma (GNB) had locoregional disease, and 28 patients had metastatic stage 4 NB. Among the 13 patients with CP, 12 had locoregional disease. Fifty-eight percent of the population were adolescents and young adults under 24 years of age. The probability of 5-year event-free survival (EFS) was 40% (confidence interval: 27%-53%). CONCLUSIONS: Outcomes were better for patients with localized tumor than for patients with metastases. For patients with localized tumor, in terms of survival, surgical treatment was the best therapeutic option. Multimodal treatment with chemotherapy, surgery, radiotherapy, and immunotherapy-based maintenance allowed long-term survival for some patients. Adolescent- and adult-onset neuroblastoma appeared to have specific characteristics associated with poorer outcomes compared to pediatric neuroblastoma. Nevertheless, complete disease control improved survival. The presence of a neuroblastic component in pheochromocytoma should be considered when making therapeutic management decisions. The development of specific tools/resources (Tumor Referral Board, Registry, biology, and trials with new agents or strategies) may help to improve outcomes for patients.

Anatomo-functional study of the cerebellum in working memory in children treated for medulloblastoma.

INTRODUCTION: Medulloblastoma is the most common malignant cerebral tumor during childhood, arising in the posterior fossa. Children treated for medulloblastoma often experience working memory (WM) deficits, affecting their quality of life and school performance. The aim of the present study undertaken to describe the cerebellar involvement in WM deficits observed in these children. MATERIAL AND METHODS: 23 healthy children and 11 children treated for medulloblastoma were included into study. All subjects performed a detailed neuropsychological examination, an anatomical and functional MRI. Stimuli were presented to the participants with alternating sensory modality and nature of communication in a block design during functional magnetic resonance imaging acquisitions. Non-parametric tests were used for analyzing neuropsychological and behavioral data. SPM8 and SUIT (Spatially Unbiased Atlas Template) were used for anatomical and functional MRI data analyses. RESULTS: Patients had cerebellar resections mainly located in the left posterior lobe. Patients had significantly reduced intelligence quotient, central executive and visuospatial WM. In healthy children group, fMRI showed activations for non-verbal and visuospatial WM in the left posterior cerebellar lobe. CONCLUSION: This study provides further evidence that left posterior cerebellar lobe plays a critical role in WM. Indeed, lesions of left posterior cerebellar lobe were associated with WM impairment in children treated for cerebellar medulloblastoma. Additionally, fMRI using WM tasks showed activation in the left posterior cerebellar lobe in healthy children. Taken together, these findings may help for improving treatment and rehabilitation of children referred for cerebellar tumor.

Feticide Before Termination of Pregnancy in Singleton Pregnancy - Trends in England and Wales 2012-2020, a Cross-sectional Study.

Feticide is the practice of inducing fetal demise before the termination of pregnancy. In England and Wales, it is recommended for terminations of pregnancy beyond 21+6 weeks of gestation. This project analyses the trends in feticide in singleton pregnancy in England and Wales between 2012 and 2020. This project was a retrospective study that analysed data extracted from the Health and Social Act 4 (HSA4) forms submitted to the Department of Health and Social Care (DHSC). The data extracted by the DHSC included the prevalence of feticide, methods of feticide and termination, statutory grounds, gestation, service provider, maternal age, ethnicity and obstetric history. In addition, data analysis was carried out to identify trends. Between 2012 and 2020, there were 9310 feticides in England and Wales, undertaken in 0.5% of all abortions. The prevalence of feticide fluctuated; however, there was an overall decrease from 1084 cases in 2012 to 1000 cases in 2020. Intracardiac injection of potassium chloride was the most frequent method of achieving feticide (67.2%). Just over half (55.8%) of feticides took place under Ground E of the Abortion Act 1967, with the main indication being congenital malformations of the nervous system. Two-fifths (40.2%) of feticides took place at 23 weeks, 22.8% at 22 weeks and 13.5% between 20 and 21 weeks. The remainder occurred at later gestations: 17.5% at 24-29 weeks and 5.9% beyond 29 weeks. During our study period, it was more common for feticides to be carried out as part of a medical termination than a surgical termination and 60.3% occurred in NHS hospitals. Women undergoing feticide were mostly aged 30-34 years (38.3%) and of White ethnicity (78.6%). Feticide is an essential component of comprehensive abortion care for women undergoing late second and third-trimester abortions. This study provides insight into how feticide is carried out in England and Wales and demonstrates the effect of the COVID-19 pandemic on reducing feticide prevalence. Future research should analyse in more detail the use of the different methods of feticide.

Management of a Composite Pheochromocytoma (Pheochromocytoma/Neuroblastoma) in Adult Patient Recurring After Several Years: A Complex Case Report.

Pheochromocytoma/neuroblastoma composite tumors are rare entities for which little is known. We report an atypical case of a 39-year-old man with secondary bone locations of a composite tumor, 7 years after resection of adrenal neuroblastoma, with constitutional alteration of SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily A, member 4 whose role is unknown. The diagnosis of a peripheral neuroblastic tumor in adulthood is difficult and even more so when it is a composite tumor. In the absence of a standard of care, management is varied and discussions about treatment modalities for these patients are complex.

Adolescents and Young Adults with Primary Brain Tumor: Description of the Care Pathway of Patients Diagnosed in Grenoble Between 2013 and 2019.

Purpose: This study was undertaken to describe the Adolescents and Young Adults' (AYA-aged patients') neuro-oncology care pathways at Grenoble Alpes University Hospital according to the French health recommendations. Methods: A retrospective monocentric observational study was conducted between 2020 and 2021, on patients 13-29 years of age, diagnosed from January 2013 to 2019 in our institute with a primary brain tumor, excluding meningiomas, schwannomas, and neurinomas. The detailed analysis took into account the type of care unit (adult or pediatric), whether or not a dedicated AYA support team was involved, and various pretherapeutic actions. Results: Sixty patients were included in this study. Three of the 9 key stages of the care process were completed regardless of the type of management. Management in a pediatric oncology department seems to improve multidisciplinary discussions and access to the AYA team. Collaboration with an AYA team seems to improve the recourse to pediatric advice and supportive care. Conclusions: Increased vigilance at all key steps as defined in the recommendations of the Directorate General of Healthcare Services would significantly improve the overall quality of care for this specific population. In the absence of a dedicated AYA hospitalization unit, interdisciplinary cooperation between the different professionals of the adult and pediatric services is essential, and the involvement of the AYA mobile team is a factor that favors links and exchanges and ultimately an overall improvement in patient care and life. We propose our vision of an ideal AYA neuro-oncology care pathway.

Attitudes and practices of ophthalmology doctors in London (UK) on the importance of discussing work as a clinical outcome with patients during consultations: A cross sectional survey.

BACKGROUND: Limited research suggests that non-occupational health doctors rarely discuss occupation with their patients. There is a gap in research regarding the attitudes and practices of doctors towards discussing patient occupation and return to work. The aim of this work was to explore the attitudes of ophthalmology doctors towards work as a clinical outcome and assess the need for occupational health training among participants (doctors). METHODS: A cross-sectional survey among doctors working in ophthalmology in two London teaching hospitals. The survey focused on the attitudes of doctors towards 'work' as a clinical outcome, their practices of asking patients about occupation, their perceived level of competency in this area of clinical practice and the level of training doctors had received in this field. Descriptive data analysis was undertaken and results presented as frequencies and proportions. RESULTS: The response rate was 30/72 (42%). Approximately a quarter (8/30;27%) of doctors 'always' discussed return to work during care planning whilst the majority (25/30;87%) of doctors agreed or strongly agreed that this should always be the case. Over half of the doctors had received no formal OH training on how to discuss or assess the impact of health on work and only 17/30 (57%) considered themselves competent in discussing these work outcomes with patients. Over half agreed that additional training would be useful, with the majority believing that it would be most useful at all stages of medical training. CONCLUSION: We found the majority of ophthalmology doctors regard 'return to work' as an important clinical outcome yet most do not routinely discuss work outcomes with patients to inform care planning. Majority of doctors lack training in how to discuss issues relating to work and would benefit from additional OH training.

Presenting features of neuroblastoma with spinal canal invasion. A prospective study of the International Society of Pediatric Oncology Europe - Neuroblastoma (SIOPEN).

Introduction: Between 5 and 15% of children with neuroblastoma (NB) present with or develop spinal canal invasion (SCI). The majority of these children have symptoms of epidural compression of spinal cord and/or spinal nerves. Treatment of NB-SCI is considered an emergency but its modalities are not yet well-established. Independently of treatment, NB-SCI may result in significant long-term disabilities. We report on the first prospective study of NB-SCI focused on presenting characteristics of both symptomatic and asymptomatic patients and correlation between SCI-related symptoms and imaging features. Materials and methods: This SIOPEN prospective NB-SCI study opened in June 2014. Patient data including SCI symptoms evaluated by standardized measures and spinal cord imaging studies were collected for each patient. For the purpose of this study data entry was locked on July 2021. Results: Of the 208 NB-SCI patients registered, 196 were evaluable for this analysis of whom 67% were symptomatic and 33% asymptomatic. Median age was 11 months. The thorax was the commonest primary tumor site. The median intervals between initial symptoms and diagnosis and between first medical visit and diagnosis were 14 and 3 days, respectively. The was no statistical difference in frequency of presenting characteristics between symptomatic and asymptomatic patients. Presenting features of NB-SCI patients differed from other NBs for older median age, prevalence of thoracic vs. abdominal primary site, prevalence of localized vs. metastatic disease and lower incidence of MYCN gene amplification. The most common SCI features were motor deficit in the younger and pain in the older patients that correlated on imaging with both transverse and longitudinal extent but not with the level of intraspinal tumor. Spinal cord T2-hyperintensity was more frequently detected in symptomatic patients (not significant). Conclusion: This prospective study confirms that children with NB-SCI differ from NBs without SCI. Compared to previous studies, it provides more detailed information regarding presenting symptoms, time intervals between SCI symptoms, medical visit and diagnosis, and correlations between symptoms and imaging features.

Cognitive disorders in pediatric medulloblastoma: what neuroimaging has to offer.

Medulloblastomas are the most common malignant childhood brain tumors arising in the posterior fossa. Treatment improvements for these tumors have meant that there are a greater number of survivors, but this long-term patient survival has increased the awareness of resulting neurocognitive deficits. Impairments in attention, memory, executive functions, and intelligence quotient demonstrate that the cerebellum likely plays a significant role in numerous higher cognitive functions such as language, cognitive, and emotional functions. In addition, children with medulloblastoma not only have cerebellar lesions but also brain white matter damages due to radiation and chemotherapy. Functional neuroimaging, a noninvasive method with many advantages, has become the standard tool in clinical and cognitive neuroscience research. By reviewing functional neuroimaging studies, this review aims to clarify the role of the cerebellum in cognitive function and explain more clearly cognitive sequelae due to polytherapy in children with medulloblastoma. This review suggests that the posterior cerebellar lobes are crucial to maintaining cognitive performance. Clinical investigations could help to better assess the involvement of these lobes in cognitive functions.