An overview of the management of adult ependymomas with emphasis on relapsed disease

Ependymomas are rare neoplasms of the central nervous system. Disease-free survival after relapse is poor and approaches to treatment in recurrent disease often palliative. This overview summarises the management of primary disease for which broad consensus exists. We also extensively review treatment options in relapsed disease for which approaches to treatment are varied due to the paucity of literature evidence. Incorporated in this overview is a survey of UK neuro-oncology units to form a snapshot of current UK practise with respect to preferred systemic therapy regimens for patients with recurrent ependymoma. The outcome reflects a preference for mainly oral-based regimens. Universal guidance is lacking in the management of non-operable irradiated recurrent ependymoma and there are worthy therapeutic avenues for further investigation, in particular the role of radical re-irradiation and also the potential of bevacizumab in advanced disease. It is hoped that advances can be achieved by multicentre collaboration in future studies to overcome the difficulties posed by achieving meaningful data in such a rare tumour with extensive natural history.