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

A systematic review

Restricted access

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.

Abbreviations used in this paper:GTR = gross-total resection; MPE = myxopapillary ependymoma; STR = subtotal resection.
Article Information

Contributor Notes

Address correspondence to: Andrew T. Parsa, M.D., Ph.D., Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, 676 N. St. Clair, Ste. 2210, Chicago, IL 60611. email: aparsa@nmff.org.Please include this information when citing this paper: published online August 23, 2013; DOI: 10.3171/2013.6.SPINE12927.

© AANS, except where prohibited by US copyright law.

Headings
References
  • 1

    Agbahiwe HCWharam MBatra SCohen KTerezakis SA: Management of pediatric myxopapillary ependymoma: the role of adjuvant radiation. Int J Radiat Oncol Biol Phys 85:4214272013

    • Search Google Scholar
    • Export Citation
  • 2

    Akyurek SChang ELYu TKLittle DAllen PKMcCutcheon I: Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neurooncol 80:1771832006

    • Search Google Scholar
    • Export Citation
  • 3

    Al-Habib AAl-Radi OOShannon PAl-Ahmadi HPetrenko YFehlings MG: Myxopapillary ependymoma: correlation of clinical and imaging features with surgical resectability in a series with long-term follow-up. Spinal Cord 49:107310782011

    • Search Google Scholar
    • Export Citation
  • 4

    Al-Halabi HMontes JLAtkinson JFarmer JPFreeman CR: Adjuvant radiotherapy in the treatment of pediatric myxopapillary ependymomas. Pediatr Blood Cancer 55:6396432010

    • Search Google Scholar
    • Export Citation
  • 5

    Anderson MS: Myxopapillary ependymomas presenting in the soft tissue over the sacrococcygeal region. Cancer 19:5855901966

  • 6

    Bagley CAKothbauer KFWilson SBookland MJEpstein FJJallo GI: Resection of myxopapillary ependymomas in children. J Neurosurg 106:4 Suppl2612672007

    • Search Google Scholar
    • Export Citation
  • 7

    Bagley CAWilson SKothbauer KFBookland MJEpstein FJallo GI: Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev 32:3213342009

    • Search Google Scholar
    • Export Citation
  • 8

    Boström Avon Lehe MHartmann WPietsch TFeuss MBoström JP: Surgery for spinal cord ependymomas: outcome and prognostic factors. Neurosurgery 68:3023092011

    • Search Google Scholar
    • Export Citation
  • 9

    Carter MNicholson JRoss FCrolla JAllibone RBalaji V: Genetic abnormalities detected in ependymomas by comparative genomic hybridisation. Br J Cancer 86:9299392002

    • Search Google Scholar
    • Export Citation
  • 10

    Chan HSBecker LEHoffman HJHumphreys RPHendrick EBFitz CR: Myxopapillary ependymoma of the filum terminale and cauda equina in childhood: report of seven cases and review of the literature. Neurosurgery 14:2042101984

    • Search Google Scholar
    • Export Citation
  • 11

    Chao STKobayashi TBenzel EReddy CAStevens GHPrayson RA: The role of adjuvant radiation therapy in the treatment of spinal myxopapillary ependymomas. Clinical article. J Neurosurg Spine 14:59642011

    • Search Google Scholar
    • Export Citation
  • 12

    Chinn DMDonaldson SSDahl GVWilson JDHuhn SLFisher PG: Management of children with metastatic spinal myxopapillary ependymoma using craniospinal irradiation. Med Pediatr Oncol 35:4434452000

    • Search Google Scholar
    • Export Citation
  • 13

    Clover LLHazuka MBKinzie JJ: Spinal cord ependymomas treated with surgery and radiation therapy. A review of 11 cases. Am J Clin Oncol 16:3503531993

    • Search Google Scholar
    • Export Citation
  • 14

    Combs SEKelter VWelzel TBehnisch WKulozik AEBischof M: Influence of radiotherapy treatment concept on the outcome of patients with localized ependymomas. Int J Radiat Oncol Biol Phys 71:9729782008

    • Search Google Scholar
    • Export Citation
  • 15

    Davis CBarnard RO: Malignant behavior of myxopapillary ependymoma. Report of three cases. J Neurosurg 62:9259291985

  • 16

    Fassett DRPingree JKestle JR: The high incidence of tumor dissemination in myxopapillary ependymoma in pediatric patients. Report of five cases and review of the literature. J Neurosurg 102:1 Suppl59642005

    • Search Google Scholar
    • Export Citation
  • 17

    Gagliardi FMCervoni LDomenicucci MCelli PSalvati M: Ependymomas of the filum terminale in childhood: report of four cases and review of the literature. Childs Nerv Syst 9:361993

    • Search Google Scholar
    • Export Citation
  • 18

    Gavin Quigley DFarooqi NPigott TJFindlay GFPillay RBuxton N: Outcome predictors in the management of spinal cord ependymoma. Eur Spine J 16:3994042007

    • Search Google Scholar
    • Export Citation
  • 19

    Jeibmann AEgensperger RKuchelmeister KSepehrnia AStolke DBruns B: Extent of surgical resection but not myxopapillary versus classical histopathological subtype affects prognosis in lumbo-sacral ependymomas. Histopathology 54:2602622009

    • Search Google Scholar
    • Export Citation
  • 20

    Kucia EJMaughan PHKakarla UKBambakidis NCSpetzler RF: Surgical technique and outcomes in the treatment of spinal cord ependymomas: part II: myxopapillary ependymoma. Neurosurgery 68:1 Suppl Operative90942011

    • Search Google Scholar
    • Export Citation
  • 21

    Lin YHHuang CIWong TTChen MHShiau CYWang LW: Treatment of spinal cord ependymomas by surgery with or without postoperative radiotherapy. J Neurooncol 71:2052102005

    • Search Google Scholar
    • Export Citation
  • 22

    Louis DNOhgaki HWiestler ODCavenee WKBurger PCJouvet A: The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:971092007

    • Search Google Scholar
    • Export Citation
  • 23

    Lukashova-v Zangen IKneitz SMonoranu CMRutkowski SHinkes BVince GH: Ependymoma gene expression profiles associated with histological subtype, proliferation, and patient survival. Acta Neuropathol 113:3253372007

    • Search Google Scholar
    • Export Citation
  • 24

    Merchant TEKiehna ENThompson SJHeideman RSanford RAKun LE: Pediatric low-grade and ependymal spinal cord tumors. Pediatr Neurosurg 32:30362000

    • Search Google Scholar
    • Export Citation
  • 25

    Nagib MGO'Fallon MT: Myxopapillary ependymoma of the conus medullaris and filum terminale in the pediatric age group. Pediatr Neurosurg 26:271997

    • Search Google Scholar
    • Export Citation
  • 26

    Nakamura MIshii KWatanabe KTsuji TMatsumoto MToyama Y: Long-term surgical outcomes for myxopapillary ependymomas of the cauda equina. Spine (Phila Pa 1976) 34:E756E7602009

    • Search Google Scholar
    • Export Citation
  • 27

    Pica AMiller RVillà SKadish SPAnacak YAbusaris H: The results of surgery, with or without radiotherapy, for primary spinal myxopapillary ependymoma: a retrospective study from the rare cancer network. Int J Radiat Oncol Biol Phys 74:111411202009

    • Search Google Scholar
    • Export Citation
  • 28

    Prayson RA: Myxopapillary ependymomas: a clinicopathologic study of 14 cases including MIB-1 and p53 immunoreactivity. Mod Pathol 10:3043101997

    • Search Google Scholar
    • Export Citation
  • 29

    Quraishi NAWolinsky JPBydon AWitham TGokaslan ZL: Giant destructive myxopapillary ependymomas of the sacrum. Clinical article. J Neurosurg Spine 12:1541592010

    • Search Google Scholar
    • Export Citation
  • 30

    Rousseau AIdbaih ADucray FCrinière EFèvre-Montange MJouvet A: Specific chromosomal imbalances as detected by array CGH in ependymomas in association with tumor location, histological subtype and grade. J Neurooncol 97:3533642010

    • Search Google Scholar
    • Export Citation
  • 31

    Sakai YMatsuyama YKatayama YImagama SIto ZWakao N: Spinal myxopapillary ependymoma: neurological deterioration in patients treated with surgery. Spine (Phila Pa 1976) 34:161916242009

    • Search Google Scholar
    • Export Citation
  • 32

    Schild SENisi KScheithauer BWWong WWLyons MKSchomberg PJ: The results of radiotherapy for ependymomas: the Mayo Clinic experience. Int J Radiat Oncol Biol Phys 42:9539581998

    • Search Google Scholar
    • Export Citation
  • 33

    Schild SEWong WNisi K: In regard to the radiotherapy of myxopapillary ependymomas. Int J Radiat Oncol Biol Phys 53:7872002

  • 34

    Schittenhelm JBecker RCapper DMeyermann RIglesias-Rozas JRKaminsky J: The clinico-surgico-pathological spectrum of myxopapillary ependymomas—report of four unusal cases and review of the literature. Clin Neuropathol 27:21282008

    • Search Google Scholar
    • Export Citation
  • 35

    Sonneland PRScheithauer BWOnofrio BM: Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer 56:8838931985

    • Search Google Scholar
    • Export Citation
  • 36

    Stephen JHSievert AJMadsen PJJudkins ARResnick ACStorm PB: Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases. Clinical article. J Neurosurg Pediatr 9:6466532012

    • Search Google Scholar
    • Export Citation
  • 37

    Verma AZhou HChin SBruggers CKestle JKhatua S: EGFR as a predictor of relapse in myxopapillary ependymoma. Pediatr Blood Cancer 59:7467482012

    • Search Google Scholar
    • Export Citation
TrendMD
Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 443 442 55
Full Text Views 168 136 5
PDF Downloads 324 185 2
EPUB Downloads 0 0 0
PubMed
Google Scholar