Intraspinal extradural myxopapillary ependymoma of the sacrum arising from the filum terminale externa

Case report

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✓ Extradural ependymomas of the sacrococcygeal region are very rare, with most arising from the soft tissues of the presacral area or from the regions dorsal to the sacrum. In even rarer circumstances, the tumor may arise within the sacral canal, likely as a result of ependymal cells of the extradural filum terminale. Because of bone erosion caused by extension of the tumor into the pelvis or dorsal to the sacrum, a truly intraspinal extradural ependymoma in this region has until now never been clearly demonstrated. The authors present a patient with a myxopapillary ependymoma arising from the filum terminale externa in which there was no involvement of the intradural filum or extension outside the sacral canal. A review of the literature is presented, with emphasis on the pathogenesis and clinical management of these rare tumors.

Article Information

Address reprint requests to: Ziya L. Gokaslan, M.D., Department of Neurosurgery-64, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030. email: zgokasla@notes.mdacc.tmc.edu.

© AANS, except where prohibited by US copyright law.

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Figures

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    Axial neuroimaging studies. Left: Gadolinium-enhanced T1-weighted MR image revealing a well-circumscribed neoplasm arising within the sacrum. Right: Bone-density computerized tomography scan demonstrating expansion of the sacral canal with thinning of the sacral laminae.

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    Coronal T1-weighted gadolinium-enhanced (left) and T2-weighted (right) MR images of the neoplasm, revealing patchy enhancement and inhomogeneous signal intensity.

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    Photograph showing gross pathological specimen of the tumor. A portion of the extradural filum terminale can be seen at either end (arrows).

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    Photomicrograph showing characteristic features of myxopapillary ependymoma, including small clusters of ependymal cells surrounded by mucin pools. Also seen in this field is the highly characteristic finding of mucin cuffs surrounding blood vessels (arrow). H & E, original magnification × 200.

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    Sagittal gadolinium-enhanced T1-weighted MR image obtained 10 months after resection of the tumor, demonstrating no evidence of residual or recurrent tumor.

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