Background: Ependymomas are rare tumors of the central nervous system whose management is controversial. This population-based study of adults and children with ependymoma aims to (1) identify clinical and treatment-related factors that impact survival and (2) determine if postoperative radiotherapy (RT) can improve survival of patients with subtotal resection (STR) to levels similar to patients who had gross total resection (GTR). Methods: This retrospective population-based study evaluated 158 patients with ependymoma diagnosed between 1975-2007 in Alberta, Canada. Results: Younger patients (<7 years of age) were more likely to be diagnosed with grade III tumors compared with adults in whom grade I tumors were more common (p=0.003). Adults were more likely to have spinally located tumors compared to young children whose tumors were typically found in the brain. Overall, young children with ependymoma were more likely to die than older children or adults (p=0.001). An equivalent number of patients underwent GTR as compared with STR (48% vs 45%, respectively). Overall, older age, spinal tumor location, lower grade, and GTR were associated with improved progression free survival but only GTR was associated with significant improvement in overall survival. Median survival after STR and RT was 82 months compared with 122 months in patients who had GTR (p=0.0022). Conclusions: This is the first Canadian population-based analysis of patients with ependymoma including adults and children. Extent of resection appears to be the most important factor determining overall survival. Importantly, the addition of RT to patients initially treated with STR does not improve survival to levels similar to patients receiving GTR.

Spinal cord ependymomas are relatively rare tumours and their management is not well established. This retrospective analysis was performed to examine the outcome of patients treated for this malignancy at our institution between 1982 and 2004.

Hospital charts of 14 consecutive patients (10 men and four women) aged 8 to 58 years (median 32 years) were retrospectively analysed. This series included nine extramedullary and five intramedullary well differentiated (eight – G1, six – G2) ependymomas. All patients were treated with primary surgery. Postoperative radiotherapy was administered in 12 patients after subtotal excision. Total dose ranged from 35Gy in 17 fractions to 50.4Gy in 28 fractions. One patient experienced spinal axis failure despite complete resection of the primary tumour and was managed with salvage radiotherapy and chemotherapy following surgical excision of the relapse. There have been no radiotherapy-related serious side effects.

One patient was lost to follow-up. Of the remaining 13 patients, at a median follow-up of 5.5 years (3 months to 20 years), 12 were alive including 11 without progression.

Conclusion: In our group of patients radiotherapy following subtotal resection or used as a single modality was well tolerated and resulted in 92% long-term survival.

Superficial siderosis of the central nervous system (CNS) is a rare disease resulting in the accumulation of haemosiderin in the meninges, the brain surface, the spinal cord and the cranial nerves. The pigment is deposited as a result of chronic bleeding in the subarachnoid space. This produces a clinical picture of deafness, ataxia, cranial nerve deficits and in the latest stages dementia. In some cases the source of bleeding can be identified, whilst in others it can not. Despite its rarity the disease should be considered in the differential diagnosis of sensorineural deafness, particularly as it is a progressive and in some cases curable disease which is easily diagnosed by magnetic resonance imaging (MRI). In this case report the haemosiderin was derived from an ependymoma of the fourth ventricle with extension into the cerebello-pontine angle. The first symptom was a worsening sensorineural hearing loss.