Surgical Technique and Outcomes in the Treatment of Spinal Cord Ependymomas: Part II: Myxopapillary Ependymoma

Kucia, Elisa J.; Maughan, Peter H.; Kakarla, U Kumar; Bambakidis, Nicholas C.; Spetzler, Robert F.

doi:10.1227/neu.0b013e3181fdf912

Cited by 26 publications

(46 citation statements)

References 15 publications

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“…Males outnumbered females at a ratio of 1.5:1. The most common presenting symptom was back pain, 6,13,23,35 mostly of dull intensity and restricted to the lower back. Some patients reported a pseudoradicular component as well.…”

Section: Discussion Clinical Presentation and Diagnosismentioning

confidence: 99%

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Klekamp

2015

FOC

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OBJECT Ependymomas of the filum terminale provide specific surgical challenges due to their often enormous size, contact with nerve roots of the cauda equina and conus, and potential for subarachnoid dissemination. This study presents treatment results for these tumors over a 30-year period. METHODS Among 1447 patients with tumors of the spinal canal treated between 1980 and 2014, 618 patients presented with extramedullary tumors. Of these, 42 patients (25 males, 17 females) demonstrated a myxopapillary ependymoma in the lumbosacral region. Thirty-four patients underwent 36 operations for 39 such tumors. The mean patient age was 38 ± 14 years (range 11−73 years), with an average clinical history of 37 ± 67 months. Patients were followed through outpatient visits and questionnaires, with a mean follow-up of 10 years (127 ± 100 months). Twenty-seven operations were performed to treat de novo tumors and the remainder were undertaken on recurrent tumors. Short-term results were determined for individual symptoms, and tumor recurrence rates were calculated with Kaplan-Meier statistical analyses. RESULTS Subarachnoid dissemination was observed in 11 patients and was related to previous surgery in 9 patients and associated with extensive tumors in 2 patients. Gross-total resections (GTR) were achieved in 28 operations (77.7%) and subtotal resections in the remainder. Subtotal resections were restricted to unencapsulated ependymomas (61.5%). Radiotherapy was employed after 6 operations on unencapsulated tumors, with 5 of these also demonstrating subarachnoid seeding. Permanent surgical morbidity affected 3 patients who experienced permanent worsening of bladder function, while 7 patients showed no postoperative changes, and the remaining 26 operations were followed by improvements. Long-term outcome depended on the amount of resection and the presence of a tumor capsule. Eight of 9 tumor recurrences affected unencapsulated tumors, of which 3 had undergone GTR. The overall recurrence rates were 6.6%, 19.0%, and 37.0% after 1, 10, and 20 years, respectively. For unencapsulated ependymomas, the corresponding rates were 15.6%, 32.5%, and 66.2% after 1, 10, and 20 years, respectively, with significantly lower rates of 9.1% after 10 and 20 years for encapsulated tumors. Postoperative radiotherapy tended to prolong the recurrence-free interval for patients with unencapsulated tumors. Five patient deaths occurred during follow-up, of which 2 deaths were tumor related and occurred at 216 and 287 months after surgery. CONCLUSION Extramedullary ependymomas are slow-growing tumors in the lumbosacral region, sometimes with an indolent course for long periods of time. Despite their delicate location and often enormous size, surgical morbidity in experienced hands is low, with good chances for postoperative clinical improvements and very low recurrence rates after GTR for encapsulated tumors. The role of postoperative radiotherapy remains controversial. Radiotherapy may be considered after incomplete resections of unencapsulated tumors and/or for patients with subarachnoid dissemination.

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Section: Discussion Clinical Presentation and Diagnosismentioning

confidence: 99%

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Klekamp

2015

FOC

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“…Akyurek and colleagues 2 found no difference in the overall survival rate or progression free-survival rate between patients treated by GTR and those treated by STR. Likewise, Kucia and colleagues 20 reported no difference in the outcomes of patients treated by GTR alone versus STR followed by radiotherapy. In contrast, Sonneland and colleagues 35 showed that GTR was associated with lower Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children overall recurrence rates and improved overall survival than was STR alone.…”

Section: ©Aans 2013mentioning

confidence: 96%

“…1,6,16,23,[27][28][29]32 In total, 28 articles with 475 patients were included in this integrative analysis. [2][3][4][5][7][8][9][10][11][12][13][14][15][17][18][19][20][21][23][24][25][26]30,31,[34][35][36][37] …”

Section: Article Selectionmentioning

confidence: 99%

“…Of the 475 patients included in this analysis, only 27 presented initially with recurrent disease. For 24 of these cases, 7 the assigned age was the age at initial presentation; for the remaining 3, 20 it was the age at recurrence because this was the only age available. Tumor location was dichotomized into thoracolumbar and lumbosacral.…”

Section: Data Extractionmentioning

confidence: 99%

“…Six modern articles reported pre-and postoperative MRI scans on all included patients and were included in a subgroup analysis of MRI-era studies. 12,20,25,30,31,34 Six articles specifically described the location of the tumor with respect to the filum terminale and were included in a separate subgroup analysis. 9,10,13,17,35,36 …”

Section: Data Extractionmentioning

confidence: 99%

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Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children

Feldman

Clark²,

Safaee³

et al. 2013

SPI

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Object Myxopapillary ependymomas (MPEs) are rare WHO Grade I tumors found in the conus medullaris, cauda equina, and filum terminale. Treatment generally consists of resection with or without adjuvant radiotherapy. Evidence-based guidelines for surgical management are lacking due to the rarity of this tumor. Methods An English-language PubMed search was performed using the key words “myxopapillary” and “ependymoma.” Reports describing fewer than 3 patients or those lacking data on the extent of resection or radiotherapy were excluded. A total of 28 articles describing 475 patients met the authors' inclusion criteria. Patients were grouped by extent of resection and whether or not they underwent adjuvant radiotherapy. Differences in recurrence rates were assessed by chi-square test. Results The overall recurrence rate was 15.5% in patients treated by gross-total resection (GTR) and 32.6% in patients treated by subtotal resection (STR), irrespective of whether they underwent adjuvant therapy (p < 0.001). Regardless of the extent of resection, adjuvant radiotherapy was not associated with a decrease in recurrence rates. The overall recurrence rate was 15.6% in patients who underwent GTR and radiotherapy compared with 15.9% in patients who underwent GTR alone (p = 0.58), and it was 29.3% in patients who underwent STR and radiotherapy compared with 35.1% in those who underwent STR alone (p = 0.53). The difference between recurrence rates for patients who underwent GTR alone versus STR and radiotherapy was statistically significant (p = 0.02). Subgroup analysis demonstrated significantly higher recurrence rates in pediatric patients compared with adults (40.5% vs 23.4%, respectively; p = 0.02). Even in the setting of GTR alone, recurrence rates were higher in pediatric patients (65% vs 7.6%; p < 0.001). Conclusions Gross-total resection alone is associated with decreased recurrence rates compared with STR with or without radiotherapy. The authors' results suggest that treatment goals should include attempted GTR whenever possible. The observation that children benefitted from radiation therapy to a greater extent than did adults suggests that biological differences between tumors in these patient populations warrants more rigorous scientific studies.

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Clinical course of adult patients with ependymoma

Armstrong

Vera-Bolaños

Gilbert

2011

Cancer

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BACKGROUND: Ependymomas are a rare tumor in adults, and there are limited reports of the clinical course, treatment, and current health status of patients. METHODS: Patients with ependymoma completed an online survey regarding their diagnosis, treatment course, and current health status. Descriptive statistics were used to report the characteristics and degree of symptom severity. Correlations between demographic and clinical characteristics were explored with univariate analysis. RESULTS: One hundred eighteen adults participated, and there were more women (n ¼ 68) than men (n ¼ 50). The median age was 48 years (range, 22-77 years). Fifty-nine participants (48%) reported that they had not been able to work since their diagnosis, and 31% reported receiving disability benefits. Patients who had been treated for spine tumors had symptoms longer than those who had been treated for brain lesions (chi-square statistic, 7.294; P ¼ .026), and the majority had 3 symptoms before diagnosis. Most patients reported undergoing complete resection (brain lesions, 62%; spine tumors, 58%), and patients with spine tumors were less likely to have received additional treatment (chi-square statistic, 9.687; P ¼ .008). The majority had not had a recurrence and reported consulting a neurosurgeon for surveillance. Despite having stable disease and not receiving active treatment, most patients described moderate to severe symptoms, including fatigue (44%), numbness/tingling (39%), pain (36%), and disturbed sleep (34%) overall. Brain lesions were associated with altered vision (25%), difficulty concentrating (25%), weakness (19%), irritability (19%), difficulty speaking (19%), and understanding (17%); and spine lesions were associated with extremity weakness (55%), sexual dysfunction (48%), radiating pain (37%), and change in bowel pattern (35%). CONCLUSIONS: Treatment for adult patients with ependymoma is not standardized. Despite the low recurrence rate, patients reported significant symptoms and disability. Cancer 2011;117:5133-

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Surgical Technique and Outcomes in the Treatment of Spinal Cord Ependymomas: Part II: Myxopapillary Ependymoma

Cited by 26 publications

References 15 publications

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Spinal ependymomas. Part 2: Ependymomas of the filum terminale

Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children

Clinical course of adult patients with ependymoma

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