MYXOPAPILLARY EPENDYMOMA OF THE SPINAL CORD IN ADULTS: A REPORT OF PERSONAL SERIES AND REVIEW OF LITERATURE

Abstract

Myxopapillary ependymomas (MPE) of the spinal cord are slow-growing benign tumors most frequently found in adults between 30 and 50 years of age. They arise from the ependyma of the filum terminale and are located in the area of the medullary conus and cauda. The recommended treatment option is gross total resection, while patients undergoing subtotal resection usually require radiotherapy. Complete resection without capsular violation can be curative and is often accomplished by simple resection of the filum above and below the tumor mass. Nevertheless, dissemination and distant treatment failure may occur in approximately 30% of the cases. In this paper, we propose an original MPE classification, which is based upon our personal series report concerned with tumor location and its correlation with the extent of resection. We also provide literature review, discussing surgical technique, tumor recurrence rate and dissemination, and adjuvant treatment. In conclusion, our findings suggest that MPE management based on the proposed 5-type tumor classification is favorable when total surgical resection is performed in carefully selected patients. Yet, further studies on a much broader model is obligatory to confirm this.

Keywords: Gross total resection; Myxopapillary ependymoma; Surgical technique; Tumor classification.

Fig. 1

Preoperative sagittal post-contrast T1-weighted MRI of the spine showing a cystic contrast-enhancing tumor at medullary conus (L1-2) level (arrow) with syringomyelia at T10-12 level (a) and sagittal T2-weighted MRI visualizing the cyst and syringomyelia (arrow) better (b). Postoperative sagittal T2-weighted MRI revealing complete tumor resection with syringomyelia resolution (c).

Fig. 2

Preoperative sagittal T2-weighted spinal MRI revealing spinal cord cystic tumor with intratumoral hemorrhage at L1-L3 level (arrow), and subdural hematoma at S1 level (asterisk) (a). Axial T2-weighted MRI demonstrating the tumor cyst of the vertebral canal ventral part at L1 level (arrow) (b) and subdural hematoma of the dorsal part of the canal at S1 level (asterisk) (c). Postoperative sagittal T2-weighted MRI confirming complete tumor resection, hematoma evacuation, and spinal T12-L3 stabilization (d,e).

Fig. 3

Preoperative sagittal contrast-enhanced T1-weighted spinal MRI demonstrating an intradural-extramedullary tumor mass at L4 level (a) confined to filum terminale and adherent to the nerve roots (a). Preoperative T2-weighted sagittal (arrow) (b) and axial (arrow) MRI scans (c) visualizing the tumor confined to the filum terminale and adherent to the nerve roots. Postoperative sagittal T2-weighted MRI showing gross total tumor resection (d).

Fig. 4

Preoperative sagittal post-contrast T1-weighted MRI of the lumbar spine showing an enhancing solid tumor at L3 level with engrossment of filum terminale and cauda equina (a), also shown on post-contrast axial T1-weighted (b) and sagittal T2-weighted MRI (c) (arrows). Postoperative post-contrast T1-weighted sagittal (d), axial (e) and sagittal T2-weighted MRI (f) confirming total tumor resection.

Fig. 5

Preoperative post-contrast sagittal T2-weighted (a) and T1-weighted (b) lumbar spine MRI demonstrating a solid tumor mass at L3 level (arrows). Postoperative sagittal T2-weighted MRI confirming complete tumor resection (c).

Fig. 6

Preoperative post-contrast sagittal T2-weigted (a) and T1-weighted (b) lumbar MRI revealing a large cystic tumor at L2 level with hydromyelia and involvement of medullary conus. Postoperative sagittal T2-weighted MRI confirming complete tumor resection with cyst drainage and hydromyelia resolution (c).

Fig. 7

Preoperative post-contrast sagittal T2-weighted MRI of the lumbar spine demonstrating a large cystic tumor at L1-L2 level with involvement of medullary conus and filum terminale (asterisk) (a). Sagittal T1-weighted MRI revealing two additional lesions at L3 level, and S1 segment as the site of primary seeding (arrows) (b). Axial post-contrast T1-weighted MRI depicting separate tumors at L1 (c) and L3 levels (d) (arrows). Postoperative sagittal T2-weighted (e) and post-contrast T1-weighted MRI (f) confirming total resection of all the three tumors.

Fig. 8

Schematic drawing of 5-type spinal cord MPE classification.