Myxopapillary Ependymoma of the Conus medullaris and Filum terminale in the Pediatric Age Group

Nagib, Mahmoud G.; O’Fallon, M.Therese

doi:10.1159/000121154

Cited by 47 publications

(37 citation statements)

References 12 publications

(30 reference statements)

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“…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%

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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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Section: Article Selectionmentioning

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%

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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“…Myxopapillary ependymomas are slow-growing tumors that are most frequently diagnosed in the third or fourth decades of life although they are found less commonly in the pediatric age group [5]. If encapsulated and confined to the filum terminale, the tumor can be totally excised.…”

Section: Discussionmentioning

confidence: 99%

“…Occasionally, they have also been found as extradural masses in the sacrococcygeal region [1,2,3,4,5]. It is hypothesized that myxopapillary ependymomas arise from neoplastic transformation of residual ependymal embryonic precursor cells [6].…”

Section: Introductionmentioning

confidence: 99%

Unexpected Myxopapillary Ependymoma within a Filum Terminale Tethering the Spinal Cord

2007

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We present a 9-month-old neurologically normal infant with cutaneous markers for a closed neural tube defect consisting of two sacral dimples and associated tuft of hair. Magnetic resonance imaging showed that her spinal cord was tethered at S2 and associated with a large syrinx. A myelotomy was performed to address the hydrosyringomyelia and the filum terminale resected to untether the spinal cord. Histopathologic examination of the filum terminale specimen revealed the presence of an unexpected myxopapillary ependymoma. The association of a myxopapillary ependymoma with a closed neural tube defect appears to be coincidental. This patient may have presented at some future date with a clinically symptomatic myxopapillary ependymoma. The presence of microscopic myxopapillary ependymoma cells in this infant’s filum supports the concept that these tumors arise from embryonic rests of ependymal cells.

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“…After perusing the full content of these articles, 20 publications were selected for final inclusion in this systematic review (Table 1). 1,2,4,5,7,8,10,[12][13][14]16,17,[19][20][21][22][23]26,31,32 …”

Section: Search Strategymentioning

confidence: 99%

Cumulative survival analysis of patients with spinal myxopapillary ependymomas in the first 2 decades of life

Kukreja¹,

Ambekar²,

Sin³

et al. 2014

PED

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Object Reports of myxopapillary ependymomas (MPEs) of the spinal cord in pediatric patients are scarce. In the literature, various authors have shared their experiences with small groups of patients, which makes it difficult to create a consensus regarding the treatment approach for spinal MPEs in young patients. The aim of this study was to perform a survival analysis of patients in the first 2 decades of life whose cases were selected from the published studies, and to examine the influence of various factors on outcomes. Methods A comprehensive search of studies published in English was performed on PubMed. Patients whose age was ≤ 20 years were included for integrative analysis. Information about age, treatment characteristics, critical events (progression, recurrence, and death), time to critical events, and follow-up duration was recorded. The degree of association of the various factors with the survival outcome was calculated by using Kaplan-Meier estimator and Cox proportional hazard model techniques. Results A total of 95 patients were included in the analysis. The overall rate of recurrence (RR) was 34.7% (n = 33), with a median time to recurrence of 36 months (range 2–100 months). Progression-free survival (PFS) and overall survival rates at 5 years were 73.7% and 98.9%, respectively. Addition of radiotherapy (RT) following resection significantly improved PFS (log-rank test, p = 0.008). In patients who underwent subtotal resection (STR), administering RT (STR + RT) improved outcome with the lowest failure rates (10.3%), superior to patients who underwent gross-total resection (GTR) alone (RR 43.1%; log-rank test, p < 0.001). Addition of RT to patients who underwent GTR was not beneficial (log-rank test, p = 0.628). In patients who had disseminated tumor at presentation, adjuvant RT controlled the disease effectively. High-dose RT (≥ 50 Gy) did not change PFS (log-rank test, p = 0.710). Conclusions Routine inclusion of RT in the treatment protocol for spinal MPEs in young patients should be considered. Complete resection is always the goal of tumor resection. However, when complete resection does not seem to be possible in complex lesions, RT should be used as an adjunct to avoid aggressive resection and to minimize inadvertent injury to the surrounding neural tissues. High-dose RT (≥ 50 Gy) did not provide additional survival benefits, although this association needs to be evaluated by prospective studies.

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Myxopapillary Ependymoma of the Conus medullaris and Filum terminale in the Pediatric Age Group

Cited by 47 publications

References 12 publications

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

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

Unexpected Myxopapillary Ependymoma within a Filum Terminale Tethering the Spinal Cord

Cumulative survival analysis of patients with spinal myxopapillary ependymomas in the first 2 decades of life

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