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ORIGINAL ARTICLE
Year : 2013  |  Volume : 9  |  Issue : 1  |  Page : 60-63

Spinal ependymomas: Prognostic factors and treatment results


Department of Neurosurgery, University Hospital of Ioannina, Ioannina, Greece

Date of Web Publication10-Apr-2013

Correspondence Address:
George A Alexiou
PO box 103, Neohoropoulo, Ioannina, 45500
Greece
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.110371

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 > Abstract 

Objective: We retrospectively analyzed patients with spinal ependymomas who were treated in our institute. We correlated outcome and recurrence with clinical and pathological features.
Materials and Methods: Between January 2000 and January 2010, we treated 14 patients with spinal ependymoma (10 males, 4 females; mean age: 48.3±18 years, range: 18-79 years). All patients were operated and received standard postoperative care. The outcome was estimated based on Frankel scale.
Results: The most common presenting symptom was pain. All tumors were operated through a posterior approach and gross total resection was performed in 13/14 cases. Histopathological examination revealed the presence of one anaplastic ependymoma, nine grade II ependymomas, and four myxopapillary ependymomas. The mean Ki-67 index was 1.5%. All the patients were followed up postoperatively for an average of 5.1 years. One patient was reoperated because of recurrent disease and another received radiotherapy due to dissemination of disease. No association was found between extent of resection, tumor location, Ki-67 index, and recurrence of disease. There was a trend toward a higher risk of recurrence in myxopapillary ependymomas. Eight patients improved postoperatively. Interestingly, during the follow-up period, four patients developed a secondary neoplasia.
Conclusion: Early intervention and gross total resection of spinal ependymomas are associated with a favorable outcome. Further studies are needed to clarify the incidence of the development of a second cancer in these patients.

Keywords: Ependymoma, outcome, spine


How to cite this article:
Voulgaris S, Alexiou GA, Zigouris A, Fotakopoulos G, Michos E, Katsiafas I, Savvanis G, Pachatouridis D. Spinal ependymomas: Prognostic factors and treatment results . J Can Res Ther 2013;9:60-3

How to cite this URL:
Voulgaris S, Alexiou GA, Zigouris A, Fotakopoulos G, Michos E, Katsiafas I, Savvanis G, Pachatouridis D. Spinal ependymomas: Prognostic factors and treatment results . J Can Res Ther [serial online] 2013 [cited 2019 Nov 13];9:60-3. Available from: http://www.cancerjournal.net/text.asp?2013/9/1/60/110371


 > Introduction Top


Compared with intracranial ependymomas, spinal ependymomas are less prevalent and exhibit a better prognosis. [1],[2],[3] Spinal ependymomas account for 50-60% of adult spinal tumors. The average age of the patients is between 35 and 45 years, and there is a slight male predominance. [1],[2],[3] Spinal ependymomas are classified as myxopapillary ependymoma [World Health Organization (WHO) grade I], ependymoma (WHO grade II), and anaplastic ependymoma (WHO grade III). Low back pain is the most common presenting symptom. Other symptoms are leg weakness, changes in gait, sensory changes, and disturbances of bowel and bladder function. [1] In the present study, we retrospectively analyzed patients with spinal ependymomas who were treated in our institute.


 > Materials and Methods Top


Between January 2000 and January 2010, 14 consecutives patients (10 males, 4 females; mean age: 48.3±18 years, range: 18-79 years) with spinal ependymoma were treated in our institute. All patients were assessed preoperatively by neurological examination, computed tomography (CT), and magnetic resonance imaging (MRI) of the spine. All patients were operated via a posterior approach. Transcranial motor evoked potentials (TcMEP), somatosensory evoked potentials (SSEP), and free-running electromyography (EMG) were monitored intraoperatively by an experienced neurophysiologist in nine cases. All tumors were classified according to the WHO 2007 scheme. Post operation, each patient had an MRI within a month after surgery, approximately six months postoperatively, and then annually. The extent of resection was defined based on the postoperative MRI findings. The outcome was estimated based on the Frankel scale. The study was approved by the Institutional Review Board.

Technique for TcMEP, SSEP, and EMG

Multipulse transcranial electrical stimulations were generated with an XLTEK stimulator. The electrodes were placed in the C1/C2 position of the International 10-20 system. Intensity of the stimulus was gradually increased from 0 to 200 mA and the duration from 0.2 to 0.5 milliseconds. The first recording was stored as baseline and was compared to the postoperative value. Regarding interpretation of the TcMEP, we used the total loss of amplitude as suggestive of damage. Free-running EMG monitoring was performed during the operation. The EMG electrodes were placed on the muscles (Netter's dermatomes) according to the spinal segment operated on, to record the corresponding nerve roots.

Statistical analysis

The functional outcome was categorized as 'good' versus 'fair' and 'poor'. The relationship between pre and postoperative Frankel grade was assessed using Fisher's exact test. To investigate whether tumor location, tumor grade, Ki-67 index, and extent of resection correlated with tumor recurrence and postoperative functional outcome, we used the paired sample t-test. A two-sided p value <0.05 was considered statistically significant.


 > Results Top


Patient characteristics are presented in [Table 1]. The most common presenting symptom was pain (71%), followed by gait changes (50%) and urine incontinence (28%). The mean duration of symptoms was 5.4 months (range: 1 month to 2 years). Two patients presented with Frankel grades D-E, whereas the remaining patients presented with Frankel grades A-C. The tumors were located in the cervical segment (n = 4), thoracic segment (n = 2), conus medullaris (n = 2), and cauda equina (n =6). In one case, there were multiple tumors. All tumors were operated through a posterior approach and gross total resection was performed in 13/14 cases [Figure 1], [Figure 2]. During the operation, free-running EMG monitoring prompted a surgical alert in case of an irritation in the recordings of the spinal section. In such cases, we ran the TcMEP to identify changes in the amplitude. In all the cases, no change in TcMEP amplitude was recorded, suggesting no neurological deterioration. SSEPs were also unchanged. Histopathological examination revealed the presence of one anaplastic ependymoma, nine grade II ependymomas, and four myxopapillary ependymomas. The mean Ki-67 index was 1.5% (range: 0.5 to 3%). After a mean follow-up period of 5.1 years (range: 2 months to 10 years), 10 patients were alive. In one case, there was a postoperative cerebrospinal fluid (CSF) leak. Within the follow-up period, one patient was reoperated because of recurrent disease, and another received radiotherapy due to dissemination of disease. No association was found between age, tumor location, Ki-67 index, and recurrence of disease. There was a trend toward a higher risk of recurrence in myxopapillary ependymomas (p = 0.053) and in subtotal tumor excision (p = 0.1). Regarding functional outcome, eight patients improved postoperatively, whereas the remaining six remain unchanged. Patients with a good preoperative Frankel grade and shorter duration of symptoms tended to have a better functional status postoperatively; however, the difference was not statistically significant. Interestingly, during the follow-up period, four patients developed a secondary neoplasia. There were two cases of gastric cancer, one case of lung cancer, and one case of a brain anaplastic astrocytoma. Furthermore, one patient developed multiple sclerosis.
Figure 1: Case #2. A 21-year-old female patient with lumbar pain for over a month; (a) preoperative T2-weighted magnetic resonance imaging (MRI) and gadolinium-enhanced T1-weighted MRI (b) revealing a tumor in the conus medullaris; (c) postoperative MRI revealing complete tumor excision

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Figure 2: Case #4. A 38-year-old male with unsteadiness of gait and urine incontinence; (a) preoperative T1-weighted and gadolinium-enhanced T1-weighted MRI (b) revealing a cauda equina tumor; (c) T2-weighted MRI six years later revealing no tumor recurrence

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Table 1: Detailed data of patients

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 > Discussion Top


The present study demonstrated a cohort of spinal ependymomas with a long follow-up. We found that gross total excision is associated with a favorable outcome. Myxopapillary ependymomas are probably associated with a higher risk of recurrence. Patients with a shorter duration of symptoms tended to have a better functional status. Interestingly, during the follow-up period, four patients developed a secondary neoplasia. Intraoperative monitoring enabled the surgeon to feel more comfortable in proceeding with tumor debulking, by reducing the risk of neurological deficits.

Spinal cord tumors account for 5-10% of all primary central nervous system tumors. They are classified as extradural, intradural, extramedullary, and intramedullary tumors. The most common intramedullary neoplasm in adults is ependymoma, which accounts for 50-60% of spinal neuroepithelial tumors. [1] There is a male predominance according to all studies. [1],[2],[3],[4],[5] In our study, males accounted for 70%. The diagnosis of an ependymoma is usually late, because of slow progression of the tumor. The patient suffers from back pain for about a year, especially at night, and may present with sensory, motor, and sphincter disturbances. [1],[6],[7] The duration of symptoms ranged in our study between one month and two years, a delay that seems to be confirmed by all studies. [1],[5],[6],[8],[9]

Gross total excision should be the goal in spinal ependymomas. In the present study, complete resection of the tumor via posterior laminectomy was achieved in 13/14 patients. Radical excision has been reported in 65 to 100% of the cases. [1],[6],[10],[11] The clear intraoperative plane of an ependymoma allows for gross total resection in majority of the cases. Chang et al. reported that tumor location and histology may also influence extent of resection. [7] Regarding preoperative and postoperative functional status, a shorter duration of preoperative symptoms and better functional grade are associated with better prognosis. Boström et al., after performing a multivariate logistic regression analysis, reported that the preoperative neurological status was an independent predictor factor of postoperative functional outcome. [1] Chang et al., after evaluating 31 patients with spinal ependymoma, concluded that the extent of removal had a prognostic significance. [7] In our study also, extent of resection was associated with a favorable outcome. Nevertheless, Kochbati et al. suggested that aggressive surgery is not necessary. In this study, the authors studied 16 patients, nine of whom had a subtotal resection. The myxopapillary subtype had a five-year survival rate of 100%. Thus, they concluded that histologic subtype had a prognostic significance. [4] Other investigators also found that tumor grade had a prognostic significance despite postoperative irradiation in patients with spinal ependymomas. [5],[12],[13] In another study of 41 spinal ependymomas, tumor grade, preoperative functional status, and age of the patient were associated with an overall survival of 60%. [14] The dissemination of ependymoma in the entire neuraxis was the indication for one patient in our study to receive radiotherapy. McLaughlin et al. observed that postoperative radiotherapy resulted in 100% local control in incompletely excised ependymomas. [9] In the present study, no postoperative radiotherapy was used. The secondary neoplasia that occurred in four of our patients is an interesting observation. A similar finding has also been reported from previous studies. [1] Recently, Halvorsen et al. retrospectively studied 86 patients with intraspinal ependymomas. All patients underwent surgery and 14 patients received additional adjuvant radiotherapy. During the follow-up period, five patients developed a second cancer. [15] This observation has not been reported in other tumor types and deserves further investigation.

This study has several limitations. It was performed in a single institution and the number of patients was relatively small; thus, no survival analysis could be performed. Furthermore, Ki-67 index was not available in all the cases. In conclusion, we believe that early intervention and gross total resection of spinal ependymoma is associated with a favorable outcome. A longer duration of symptoms before treatment is associated with a poorer functional outcome. Further studies are needed to clarify the incidence of the development of a second cancer in these patients.

 
 > References Top

1.Boström A, von Lehe M, Hartmann W, Pietsch T, Feuss M, Boström JP, et al. Surgery for spinal cord ependymomas: Outcome and prognostic factors. Neurosurgery 2011;68:302-8.  Back to cited text no. 1
    
2.Schwartz TH, McCormick PC. Intramedullary ependymomas: Clinical presentation, surgical treatment strategies and prognosis. J Neurooncol 2000;47:211-8.  Back to cited text no. 2
    
3.Reni M, Gatta G, Mazza E, Vecht C. Ependymoma. Crit Rev Oncol Hematol 2007;63:81-9.  Back to cited text no. 3
    
4.Kochbati L, Nasr C, Frikha H, Gargouri W, Benna F, Besbes M, et al. Primary intramedullary ependymomas: Retrospective study of 16 cases. Cancer Radiother 2003;7:17-21.  Back to cited text no. 4
    
5.Waldron JN, Laperriere NJ, Jaakkimainen L, Simpson WJ, Payne D, Milosevic M, et al. Spinal cord ependymomas: A retrospective analysis of 59 cases. Int J Radiat Oncol Biol Phys 1993;27:223-9.  Back to cited text no. 5
    
6.Eroes CA, Zausinger S, Kreth FW, Goldbrunner R, Tonn JC. Intramedullary low grade astrocytoma and ependymoma. Surgical results and predicting factors for clinical outcome. Acta Neurochir (Wien) 2010;152:611-8.  Back to cited text no. 6
    
7.Chang UK, Choe WJ, Chung SK, Chung CK, Kim HJ. Surgical outcome and prognostic factors of spinal intramedullary ependymomas in adults. J Neurooncol 2002;57:133-9.  Back to cited text no. 7
    
8.Garcés-Ambrossi GL, McGirt MJ, Mehta VA, Sciubba DM, Witham TF, Bydon A, et al. Factors associated with progression-free survival and long-term neurological outcome after resection of intramedullary spinal cord tumors: Analysis of 101 consecutive cases. J Neurosurg Spine 2009;11:591-9.  Back to cited text no. 8
    
9.McLaughlin MP, Marcus RB Jr, Buatti JM, McCollough WM, Mickle JP, Kedar A, et al. Ependymoma: Results, prognostic factors and treatment recommendations. Int J Radiat Oncol Biol Phys 1998;40:845-50.  Back to cited text no. 9
    
10.Alkhani A, Blooshi M, Hassounah M. Outcome of surgery for intramedullary spinal ependymoma. Ann Saudi Med 2008;28:109-13.  Back to cited text no. 10
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11.McLaughlin MP, Buatti JM, Marcus RB Jr, Maria BL, Mickle PJ, Kedar A. Outcome after radiotherapy of primary spinal cord glial tumors. Radiat Oncol Investig 1998;6:276-80.  Back to cited text no. 11
    
12.Stüben G, Stuschke M, Kroll M, Havers W, Sack H. Postoperative radiotherapy of spinal and intracranial ependymomas: Analysis of prognostic factors. Radiother Oncol 1997;45:3-10.  Back to cited text no. 12
    
13.Grabenbauer GG, Barta B, Erhardt J, Buchfelder M, Thierauf P, Beck JD, et al. Prognostic factors and results after the combined surgical and radiotherapy treatment of ependymomas. Strahlenther Onkol 1992;168:679-85.  Back to cited text no. 13
    
14.Garrett PG, Simpson WJ. Ependymomas: Results of radiation treatment. Int J Radiat Oncol Biol Phys 1983;9:1121-4.  Back to cited text no. 14
    
15.Halvorsen CM, Kolstad F, Hald J, Johannesen TB, Krossnes BK, Langmoen IA, et al. Long-term outcome after resection of intraspinal ependymomas: Report of 86 consecutive cases. Neurosurgery 2010;67:1622-31.  Back to cited text no. 15
    


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