Lumbosacral Ependymomas: A Review of the Management of Intradural and Extradural Tumors

Daniel R. Fassett, M.D.; Meic H. Schmidt, M.D.


Neurosurg Focus. 2003;15(5) 

In This Article

Abstract and Introduction

Object: The goal of this study was to review the management of intra- and extradural ependymomas. Spinal ependymomas most commonly occur as intramedullary tumors throughout the spinal axis. In the lumbosacral region, ependymomas are most commonly associated with the conus medullaris and cauda equina, but can also occur extradurally in the sacrum, presacral tissues, or subcutaneous tissues over the sacrum. These two tumor locations produce different management concerns. Intradural ependymomas, especially those in the lumbosacral region, are now recognized for their potential to spread throughout the central nervous system (CNS), whereas extradural tumors elicit more concern for their association with extraneural metastases.
Methods: The authors have reviewed the literature regarding both of these distinct tumors and have summarized recommendations for the management of intra- and extradural lumbosacral ependymomas. For both tumors, it appears that gross-total resection is the treatment of choice when feasible. The role of radiation therapy has not been adequately studied for either tumor location, but most clinicians use this modality in patients with subtotal resection of intradural ependymomas, local recurrence, or CNS dissemination. Data supporting the use of radiation therapy for extradural ependymomas are lacking. There does not appear to be a significant role for chemotherapy in either tumor location.
Conclusions: Despite the risk for local recurrence and CNS dissemination, the prognosis for intradural lumbosacral ependymomas is good, with a greater than 90% 10-year patient survival in most series. The prognosis for extradural ependymomas does not appear to be as good. Much depends on extradural tumor location, however; the outlook is better for dorsal sacral tumors than presacral tumors.

Spinal ependymomas are very rare tumors, with approximately 227 intradural spinal ependymomas diagnosed each year in the US. Of the intradural ependymomas, approximately 50% are intramedullary, above the conus, and the remaining 50% occur in association with the terminal filum, cauda equina, and conus medullaris.[3,6,22] In the lumbosacral region, ependymomas account for 90% of intradural tumors.[10] In addition to occurring intradurally, ependymomas can also sometimes occur extradurally around or in the sacrum.

Spinal ependymomas are most common in the fourth decade of life and are more common in male patients.[5,33,37] Sonneland, et al.,[32] reported a 1.7:1.0 male/female ratio and a mean age at onset of 36.4 years (range 6–82 years) in the largest series (77 patients) of myxopapillary ependymomas reported in the literature. All of the tumors were intradural in this series, with the majority occurring in the lumbosacral region.

Bimodal peaks have been suggested for extraspinal ependymomas in the sacral region, with one peak at less than 8 years of age and one in the fourth decade of life.[20,21] Others have suggested that extraspinal ependymomas occur in younger patients compared with intraspinal ependymomas.[1,15,17,21,25] There appears to be no significant sex predominance for extradural ependymomas.[15,21,25]