Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases

Stephen, James H.; Sievert, Angela J.; Madsen, Peter J; Judkins, Alexander R.; Resnick, Adam; Storm, Phillip B.; Rushing, Elisabeth J.; Santi, Mariarita

doi:10.3171/2012.2.peds11285

Cited by 48 publications

(33 citation statements)

References 21 publications

(30 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…8 In a 2012 retrospective single-institution study (n = 11 Grade II ependymomas), Stephen et al reported 100% PFS and OS after a mean of 51 months of follow-up. 62 In a 2013 literature review of more than 30 series (n = 43 Grade II ependymomas), Safaee et al reported a 5-year OS of greater than 90% and a PFS of approximately 80%. 59 This study found that females were much more likely to survive than males among pediatric subjects with Grade II spinal ependymoma.…”

Section: Characteristics and Demographicsmentioning

confidence: 99%

“…7,42 They are slow-growing, indolent tumors with a benign course; in the literature, rates of both overall survival (OS) and progression-free survival (PFS) at 5 years have been reported at 90%-100%. 7,42,62 Maximal resection is generally agreed to be the cornerstone of treatment for pediatric spinal ependymomas; 2,7,8,34,42,47,50 however, the addition of adjuvant radiation therapy after surgery is the subject of more debate. 2,7,8,48 While there are many studies on spinal ependymomas in adults, 11,12,[16][17][18]21,24,25,38,41,45,51,56,57,61,65 there are few papers on these tumors in children, and those that do exist tend to be small, single-institution retrospective studies that are not powered for statistical analysis stratified by tumor grade.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Treatment of pediatric Grade II spinal ependymomas: a population-based study

Lin

Jea

Melkonian

et al. 2015

PED

View full text Add to dashboard Cite

OBJECT Grade II spinal cord ependymomas occurring in pediatric patients are exceptionally rare neoplasms. In this paper the authors use a national cancer database to determine patient demographics, treatment patterns, and associated outcomes of this cohort. METHODS The Surveillance Epidemiology and End Results (SEER) database was used to analyze subjects younger than 18 years with histologically confirmed diagnoses of Grade II spinal cord ependymoma from the years 1973 to 2008. Descriptive data on the demographic characteristics of this cohort and the associated treatment patterns are shown. The Kaplan-Meier method was used to estimate overall survival at 1, 2, 5, and 10 years. RESULTS This cohort comprised 64 pediatric subjects with Grade II spinal ependymoma. The median age was 13 years, nearly half of the patients were male, and most were white (84%). The median follow-up was 9.2 years. Overall survival at 5 and 10 years was 86% and 83%, respectively. Gross-total resection was achieved in 57% of subjects, and radiation therapy was administered to 36%. Radiation therapy was administered to 78% of subjects after subtotal resection but only to 19% of patients after gross-total resection; this difference was significant (p < 0.001). In a multivariate regression model analyzing sex, age at diagnosis, year of diagnosis, radiotherapy, and extent of resection, female sex was found to be an independent predictor of decreased mortality (HR 0.15 [95% CI 0.02–0.94], p = 0.04). CONCLUSIONS These data show long-term outcomes for pediatric patients with Grade II spinal ependymoma. Radiotherapy was more likely to be administered in cases of subtotal resection than in cases of gross-total resection. Female sex is associated with decreased mortality, while other demographic or treatment modalities are not.

show abstract

Section: Characteristics and Demographicsmentioning

confidence: 99%

mentioning

confidence: 99%

Treatment of pediatric Grade II spinal ependymomas: a population-based study

Lin

Jea

Melkonian

et al. 2015

PED

View full text Add to dashboard Cite

show abstract

“…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%

“…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%

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

Feldman

Clark²,

Safaee³

et al. 2013

SPI

View full text Add to dashboard Cite

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.

show abstract

“…A recent literature review on pediatric spinal cord myxopapillary ependymomas reported GTR in 60% of cases, but similar recurrence rates when comparing GTR (40%) to STR (43%) at an average follow-up of 77 months. 48 Furthermore, a recent review of our institutional data suggests that extent of resection is associated with improved tumor control among Grade II ependymomas, but not Grade I ependymomas. 49 The study also reported 7 deaths (8%), while we had no deaths due to surgery or disease among both Grade I and Grade II ependymomas.…”

Section: Discussionmentioning

confidence: 99%

Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults

Safaee¹,

Mummaneni

et al. 2014

PED

View full text Add to dashboard Cite

Object Ependymomas are a common type of CNS tumor in children, although only 13% originate from the spinal cord. Aside from location and extent of resection, the factors that affect outcome are not well understood. Methods The authors performed a search of an institutional neuropathology database to identify all patients with spinal cord ependymomas treated over the past 20 years. Data on patient age, sex, clinical presentation, symptom duration, tumor location, extent of resection, use of radiation therapy, surgical complications, presence of tumor recurrence, duration of follow-up, and residual symptoms were collected. Pediatric patients were defined as those 21 years of age or younger at diagnosis. The extent of resection was defined by the findings of the postoperative MR images. Results A total of 24 pediatric patients with spinal cord ependymomas were identified with the following pathological subtypes: 14 classic (Grade II), 8 myxopapillary (Grade I), and 2 anaplastic (Grade III) ependymomas. Both anaplastic ependymomas originated in the intracranial compartment and spread to the spinal cord at recurrence. The mean follow-up duration for patients with classic and myxopapillary ependymomas was 63 and 45 months, respectively. Seven patients with classic ependymomas underwent gross-total resection (GTR), while 4 received subtotal resection (STR), 2 received STR as well as radiation therapy, and 1 received radiation therapy alone. All but 1 patient with myxopapillary ependymomas underwent GTR. Three recurrences were identified in the Grade II group at 45, 48, and 228 months. A single recurrence was identified in the Grade I group at 71 months. The mean progression-free survival (PFS) was 58 months in the Grade II group and 45 months in the Grade I group. Conclusions Extent of resection is an important prognostic factor in all pediatric spinal cord ependymomas, particularly Grade II ependymomas. These data suggest that achieving GTR is more difficult in the upper spinal cord, making tumor location another important factor. Although classified as Grade I lesions, myxopapillary ependymomas had similar outcomes when compared with classic (Grade II) ependymomas, particularly with respect to PFS. Long-term complications or new neurological deficits were rare. Among patients with long-term follow-up, those who underwent GTR had a recurrence rate of 20% compared with 40% among those with STR or biopsy only, suggesting that extent of resection is perhaps a more important prognostic factor than histological grade in predicting PFS, which has been suggested by other data in the literature. Given the relative paucity of these lesions, collaborative multiinstitutional studies are needed, and such efforts should also focus on molecular and genetic analysis to refine the current classification system.

show abstract

Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases

Cited by 48 publications

References 21 publications

Treatment of pediatric Grade II spinal ependymomas: a population-based study

Treatment of pediatric Grade II spinal ependymomas: a population-based study

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

Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults

Contact Info

Product

Resources

About