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Brain tumours are the second most common malignancy in children(1) (2), and despite some advancements being made over the last 2 decades, patient outcomes in general remain poor when compared with other childhood cancers. Optimal treatment of children with brain tumours is challenging and expertise and resources are not widely available in South Africa. This is important because the outcomes of children with brain tumours depend critically on the expertise and resources of a multidisciplinary team tasked with their treatment. Despite the importance of paediatric brain tumours though, little is known about childhood brain tumours in South Africa as limited data have been published and there have been no funded studies to support research in this area. In addition, we know very little about the resources available across the country to treat these children. In international centres of excellence the best outcomes are achieved by combining good epidemiological data, strong multidisciplinary teams, centralization or regionalization of services, available resources, and a research foundation. To start, we need to know more about the patients presenting to us with brain tumours. PURPOSE The overall aim of this project was to collect epidemiological data for childhood brain tumours at a tertiary paediatric hospital in South Africa with a dedicated multidisciplinary team. METHODS Study design: A retrospective review of records of patients diagnosed with a primary brain tumour and who presented to Red Cross Children’s Hospital (RCCH) system from 1 January 1996 to 31 December 2017. 2 Patient selection & data collection: Patients were identified by combining databases and admission logs from paediatric neurosurgery, oncology, radiotherapy, histopathology and radiology. Data collected included: age at diagnosis, sex, province of referral, tumour site and diagnosis. RESULTS A total of 554 paediatric patients with primary brain tumours were identified over the study period. Tumours were more common among males (55.4%) and were located in the supratentorial compartment in 52%. The median age at diagnosis was 5.92 years. The commonest tumours were astrocytomas (n=114 patients; 20.3%), followed by medulloblastomas (incl. PNETs) (n=107 patients; 19.1%), and craniopharyngiomas (n=55; 9.8%). As expected, most patients referred and seen at RCCH/GSH were from the expected drainage area in the Western Cape (73%), but a significant number of referrals (27%) were from outside the province referrals, especially in the last 10 years. CONCLUSION Our findings were largely consistent with the published literature in terms of histological diagnosis, sex profile and age ranges for children diagnosed with brain tumours with some small differences possibly related to referral bias. More patients than expected were referred from outside of the province, which emphasizes the need for establishing an ongoing tumour database registry and co-ordinating patient care across institutions. A follow-up study to assess patient management and outcomes is of critical importance to assess resource availability and patient outcomes.

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Purpose The purpose of this enquiry is to explore the symptom experience and the health-related quality of life (HRQL) of children who have survived a brain tumour. Design An emergent, mixed-method study design was used with three phases of inquiry. 1) A systematic review was undertaken of previous research on the HRQL outcomes and symptom experience of children who have survived a brain tumour. 2) A quantitative study of the relationship between symptom experience and HRQL in 50 children who have survived a pediatric brain tumour was completed. This study was followed by a qualitative study of 12 children that used an interpretive descriptive methodology to understand the perceived relationship between symptom experience, coping and quality of life. 3) The results of phases 1 and 2 were translated into an evidence-informed clinical practice framework. Results 1) Pediatric brain tumour survivors had poorer HRQL outcomes than other cancer survivors or healthy peers. Only two previous studies had explored the relationship between symptoms and HRQL. 2) Pediatric brain tumour survivors experience many symptoms following the completion of treatment. The most distressing symptoms were pain, headaches, fatigue and sleep problems. Survivors of pediatric brain tumours described multiple symptoms that affect their life; yet overall they described their quality of life as good. Survivors used a variety of coping strategies to help mitigate the negative effects of these symptoms. 3) The results of the above studies informed the development of a clinical practice framework (the Queen’s-Macartney Multidisciplinary Action Plan for Oncology Survivors - Q-MapS), that requires further testing. It is intended to optimize clinical practice, to encourage education and to stimulate further research. Conclusions Child survivors of brain tumours experience various symptoms that can affect their HRQL. Nurses play a pivotal role in the systematic assessment and management of the multidimensional symptom experience of children following treatment for a brain tumour. Q-MapS can empower patients and their families by increasing their awareness of potential or actual problems related to their symptom experience and HRQL. More research is needed to better understand the relationship between symptoms and HRQL in children surviving brain tumours. / Thesis (Ph.D, Nursing) -- Queen's University, 2013-05-01 21:12:57.809

Medulloblastoma (MB) is the commonest intracranial childhood malignancy and despite recent advances, current therapeutic approaches are still associated with high morbidity and mortality. A novel molecular classification has recently been proposed for these tumours – WNT Group (best prognosis), SHH Group (intermediate prognosis), Group 3 (worst prognosis) and Group 4 (intermediate prognosis). BMI1, a transcriptional repressor of the Polycomb group genes, is overexpressed in MB, most significantly in those of Group 4 MBs. Bone Morphogenetic Proteins (BMPs) are morphogens belonging to TGF-β superfamily of growth factors, and are known to inhibit MB cell proliferation and induce apoptosis in vitro, and to inhibit tumour growth in vivo. Our team have recently demonstrated that Bmi1 regulates cell adhesion properties during cerebellar development through repression of the BMP pathway. The aim of this project is to assess whether BMI1 overexpression may contribute to MB pathogenesis through repression of the BMP pathway. Here we demonstrate that BMI1 knock down derepresses BMP pathway, and using a novel xenograft model of human MB of Group 4, we show that BMI1 controls tumour volume and intraparenchymal invasion. In in vitro assays on MB cell lines we show that cell adhesion and motility is controlled by BMI1 in a BMP dependent manner and that deregulation of extracellular matrix proteins are key mediators of this effect. Furthermore, we demonstrate that BMP treatment to BMI1 overexpressing MB cells reduces cell proliferation and invasion, suggesting BMI1 as a possible biomarker for those tumours that could benefit from treatment with BMP agonist small molecules.