Improving our understanding of ependymomas in the spinal cord

Ependymoma is a disease in which cancer cells form in the tissues of the brain and spinal cord. There are mainly three types of ependymoma, based on the grade (I, II and III) of a tumour that describes how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. These tumours when treated identically based on similar grading, show different outcomes.

Ependymomas from the spinal cord are generally slow-growing tumours and commonly observed in adults, but in children, only 10% of ependymomas are found in the spinal cord. Moreover, children with spinal cord ependymoma (SEPN) are more likely to experience an aggressive disease course compared to adults. Surgery is the preferred form of treatment for children with SEPN followed by radiation therapy with or without chemotherapy. Despite advances in brain surgery, brain imaging and chemotherapy, SEPN in children have been associated with higher rates of recurrence after surgery. Furthermore, surgery is limited by the safety of operating within critical brain locations, radiotherapy can significantly damage the fragile developing brain, and several SEPN in children respond poorly to current available chemotherapy drugs. Therefore, a critical need exists to develop a therapy based on the biology of SEPN in children. Attempts to understand the biology of SEPN in children have been hampered by the lack of data to define the underlying biology.

The aim of this study is to perform a comprehensive analysis of tumour samples from children with SEPN to capture biological information in many different ways. We will assess these measured molecular profiles and identify patterns (molecular markers) that are unique in children with SEPN. In addition, we will explore the relationships between these molecular profiles in order to ultimately improve our understanding of SEPN biology.

At the end of this project we will have: 

1. Determined the molecular profiles of tumour samples from children with SEPN.
2. Explored the differences between children with SEPN and ependymomas from the brain as a first-step to identify molecular markers as novel targets for the most effective therapy, tailored to individual patients.
3. Identified the relationships between the molecular profiles to provide novel insights into the underlying biology of SEPN in children.
4. Evaluated candidate molecular markers to distinguish SEPN from ependymomas of the brain in children.