ReviewEpendymoma in adults: Local experience with an uncommon tumour
Introduction
Ependymomas are uncommon brain and spinal tumours, accounting for a mere 3–5% of adult intracranial neoplasms and 8–10% of childhood tumours of the central nervous system (CNS) [1], [2], [3], [4], [5]. These tumours are of neuroectodermal origin and were previously thought to arise from the ependymal cells lining the central canal of the spinal cord, cerebral ventricles and choroid plexus, but have more recently been postulated to be of glial origin [2], [6], [7], [8]. The World Health Organization (WHO) classifies ependymal tumours based their histological subtype into Grade I (myxopapillary ependymoma or subependymoma), II (ependymoma) or III (anaplastic ependymoma) [9], however, the prognostic relevance of Grade II versus III remains controversial [2], [5], [10], [11].
Due to the low incidence of ependymomas, outcome studies on prognostic factors and treatment modalities are limited and are based on retrospective data. Despite this, surgical resection is the standard therapeutic intervention [2], most importantly, to confirm histological diagnosis. Additionally, the extent of resection appears to be predictive of recurrence and overall survival (OS) and, thus, a safe maximal resection is usually recommended [12], [13], [14]. The role of radiotherapy as an adjuvant treatment for higher grade tumours has been established [2], [15], [16], [17] with a reduction shown in recurrence rate and improved OS. The role of chemotherapy remains controversial with limited data available, predominantly amongst the paediatric population [18], [19], [20], [21].
We reviewed our experience with ependymomas in adults over a 6 year period. By retrospectively analysing our prospectively collected data, we sought to present an Australian tertiary experience on incidence, treatment and prognosis of this uncommon CNS tumour to contribute and further elaborate on the expanding literature and research in the area.
Section snippets
Study population
The Australian Comprehensive Cancer Outcomes and Research Database is a multi-institutional database of cancer patient demographics, treatment and clinical outcomes managed by BioGrid Australia (Melbourne, VIC, Australia). It includes longitudinal data on multiple tumour types including brain, breast, lung, colorectal and haematological malignancies. The CNS tumour database, from this group of datasets, prospectively enrols all patients with CNS tumours treated at the Royal Melbourne Hospital
Clinical data
A total of 39 patients with ependymoma were diagnosed between 2008 and 2013, yielding an incidence of approximately 6.5 events per year. Demographic, tumour, and treatment characteristics are summarised in Table 1. The mean age at diagnosis was 44 years (range: 18–85), reflecting the entirely adult population treated at RMH/MPH, however, the mean age at presentation did not differ significantly between spinal (44 years), supratentorial (47 years) and infratentorial (40 years) tumours. The male to
Discussion
Our study reviews a single centre tertiary hospital experience with ependymomas in 39 adults across a 6 year period. This is the first paper, to our knowledge, to describe the diagnosis and clinical course of adult patients with ependymal tumours treated in Australia. Consistent with the reported literature in adults across several large retrospective reviews [1], [3], [6], [22], almost 75% of the diagnosed patients had spinal ependymomas, in contrast to the intracranial predominance seen in
Conclusion
Ependymomas are uncommon and their management is complex. Our study demonstrates the demographic and treatment data of a single, large, tertiary Australian hospital in dealing with this complex CNS tumour. Our findings are largely consistent with the current literature, however, a clear gap exists for a large, multi-centred review or meta-analysis. Further, there is an urgent need to establish validated predictive and prognostic molecular biomarkers for these tumours.
Conflicts of Interest/Disclosures
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
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