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| LETTER TO EDITOR |
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| Year : 2013 | Volume
: 61
| Issue : 6 | Page : 687-690 |
Ependymoma: Unusual differential for a totally extramedullary intraspinal tumor
Lars Gardner1, Manish K Kasliwal2, Nicholas Hempeck1, Manuel Utset1, Yogesh N Gandhi1
1 Department of Neurosurgery, John H. Stroger Jr. Hospital of Cook County, Chicago, IL, USA
2 Department of Neurosurgery, RUSH University Medical Center, Chicago, IL, USA
| Date of Submission | 24-Oct-2013 |
| Date of Decision | 18-Nov-2013 |
| Date of Acceptance | 18-Feb-2013 |
| Date of Web Publication | 20-Jan-2014 |
Correspondence Address:
Manish K Kasliwal
Department of Neurosurgery, RUSH University Medical Center, Chicago, IL
USA
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.125386
How to cite this article:
Gardner L, Kasliwal MK, Hempeck N, Utset M, Gandhi YN. Ependymoma: Unusual differential for a totally extramedullary intraspinal tumor. Neurol India 2013;61:687-90 |
Sir,
Benign nerve sheath tumors (NST) or meningiomas are the most common intradural extramedullary (IDEM) tumor in adults. [1] On the contrary, ependymoma is the most common primary intramedullary spinal tumor in adults. [2] Although the myxopapillary variant of ependymoma arising from the filum is extramedullary, IDEM location of this glial lesion outside filum is exceptionally rare. [3],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17],[18],[19]
A 27-year-old female patient presented with 2 years history of worsening band-like sensation across the chest and difficulty ambulating with urinary symptoms for 2 months with neurological examination consistent with thoracic myelopathy. Magnetic resonance imaging (MRI) demonstrated presence of a long segment IDEM lesion from T2 to T7 with diffuse enhancement and cystic component displacing the spinal cord to the left [Figure 1]. With a possible diagnosis of NST, a T2-T8 laminoplasty was performed. Intraoperatively, a soft-tissue tumor was seen with the spinal cord displaced but not infiltrated by the tumor. The tumor was totally extramedullary and could be dissected from the spinal cord permitting gross total resection. Histopathology confirmed the diagnosis of an ependymoma [Figure 2]. Post-operative MRI showed no residual tumor with imaging of the neuraxis demonstrating no other synchronous lesion [Figure 3]. The patients neurological symptoms resolved completely at her last follow-up at 8 months. Radiation therapy was withheld in view of complete resection but she has been kept under clinical surveillance in view of the diagnosis of ependymoma. | Figure 1: Magnetic resonance imaging (MRI) of the thoracic spine showing a long segment intradural extramedullary lesion from T2 to T7 with diffuse enhancement and cystic component displacing the spinal cord to the left. Left: Sagittal T1W MRI with contrast, middle: Axial T1W MRI with contrast and right: T2W sagittal MRI
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 | Figure 2: Densely cellular glial neoplasm with abundant perivascular pseudorossettes characteristic of ependymoma
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 | Figure 3: Sagittal T1W, T1W with contrast and T2W magnetic resonance imaging showing no obvious residual tumor with post-operative changes and well decompressed spinal cord
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Intraspinal tumor in adults can be intramedullary or IDEM. The diagnosis of IDEM tumors often connotes good prognosis as majority of these tumors are benign with excellent clinical outcome. Spinal ependymomas have been described exceptionally rare as extramedullary and only seventeen cases have been described so far as per our review of the literature [Table 1]. The plausible explanation for an IDEM ependymoma comes from the embryologic theory according to which a collection of ependymal cells are not contained within the anatomic central canal location of the spinal cord during neural tube allowing ectopic cells to be within the IDEM space after birth. [10],[11] Review of the literature shows that these tumors are usually benign and slow growing, with subtle symptoms present over a year before diagnosis. [2],[3],[4],[5],[6],[7],[8],[9],[10],[11],[14],[15],[16],[17],[18],[19],[20],[21] The treatment of choice is total resection, with radiation usually reserved for cases with incomplete resection and/or recurrence. [5],[8],[15],[16] Although radiotherapy has been proposed to prolong local control and survival after incomplete resection, [22] histological grade and surgical extent of resection remains the most important independent predictors of longer progression-free survival. [23] Nevertheless, even Grade II ependymomas may recur after complete excision at site different from the primary, as Robles, et al.[15] reported a recurrence of extramedullary ependymoma distal to the prior surgery site implicating that these tumors may not be benign as are usually thought and the natural history of other benign IDEM like NST and meningiomas cannot be extrapolated to extramedullary ependymomas. This highlights the importance of close imaging surveillance for these patients and though not conclusively proven, the possible role of avoiding tumor dissemination and spillage during surgery in cases of possible IDEM ependymoma to avoid recurrences at different CNS location. [15],[16] Though, it can be argued that the treatment of choice for all IDEM tumors is total resection; in recurrences/subtotal resections for benign tumors like meningiomas and NST, patients generally do not require spinal radiation, unlike ependymomas which may need post-operative radiation, if not excised completely or even upfront if they are Grade III. [10],[11],[13],[16],[22] | Table 1: Details of patients with intradural extramedullary ependymoma reported in the literature
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Though, IDEM ependymoma generally have a good neurologic recovery after complete resection; possibility of potential recurrence, distant metastasis due to CSF dissemination and anaplastic transformation should be watched for and these patients should be kept under close clinical and radiological surveillance. Awareness of this tumor as a differential of IDEM neoplasm with its possible clinical behavior may help pre-operative patients counseling and appropriate management.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1]
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