Cauda equina ependymomas: surgical treatment and long-term outcomes in a series of 125 patients

View More View Less
  • 1 Department of Neurosurgery, University Hospital Borgo Trento, Verona, Italy; and
  • | 2 Centro interdipartimentale di documentazione economica, University of Verona, Italy
Restricted access

Purchase Now

USD  $45.00

Spine - 1 year subscription bundle (Individuals Only)

USD  $376.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
Print or Print + Online

OBJECTIVE

Cauda equina ependymoma (CEE) is a rare tumor for which little information is available on the oncological and clinical outcomes of patients. In this study the authors aimed to address functional, oncological, and quality-of-life (QOL) outcomes in a large series of consecutive patients operated on at their institution during the past 20 years.

METHODS

The records of 125 patients who underwent surgery between January 1998 and September 2018 were reviewed. Analyzed variables included demographic, clinical, radiological, surgical, and histopathological features. Neurological outcomes were graded according to the McCormick and Kesselring scales. The QOL at follow-up was evaluated by administering the EQ-5DL questionnaire.

RESULTS

On admission, 84% of patients had a McCormick grade of I and 76.8% had a Kesselring score of 0. At follow-up (clinical 8.13 years; radiological 5.87 years) most scores were unchanged. Sacral level involvement (p = 0.029) and tumor size (p = 0.002) were predictors of poor functional outcome at discharge. Tumor size (p = 0.019) and repeated surgery (p < 0.001) were predictors of poor outcome. A preoperative McCormick grade ≥ III and Kesselring grade ≥ 2 were associated with worse outcomes (p = 0.035 and p = 0.002, respectively). Myxopapillary ependymoma (MPE) was more frequent than grade II ependymoma (EII). The overall rate of gross-total resection (GTR) was 91.2% and rates were significantly higher for patients with EII (98%) than for those with MPE (84%) (p = 0.0074). On multivariate analysis, the only factor associated with GTR was the presence of a capsule (p = 0.011). Seventeen patients (13.7%) had recurrences (13 MPE, 4 EII; 76.4% vs 23.6%; p = 0.032). The extent of resection was the only factor associated with recurrence (p = 0.0023) and number of surgeries (p = 0.006). Differences in progression-free survival (PFS) were seen depending on the extent of resection at first operation (p < 0.001), subarachnoid seeding (p = 0.041), piecemeal resection (p = 0.004), and number of spine levels involved (3 [p = 0.016], 4 [p = 0.011], or ≥ 5 [p = 0.013]). At follow-up a higher proportion of EII than MPE patients were disease free (94.7% vs 77.7%; p = 0.007). The QOL results were inferior in almost all areas compared to a control group of subjects from the Italian general population. A McCormick grade ≥ 3 and repeated surgeries were associated with a worse QOL (p = 0.006 and p = 0.017).

CONCLUSIONS

An early diagnosis of CEE is important because larger tumors are associated with recurrences and worse functional neurological outcomes. Surgery should be performed with the aim of achieving an en bloc GTR. The histological subtype was not directly associated with recurrences, but some of the features more commonly encountered in MPEs were. The outcomes are in most cases favorable, but the mean QOL perception is inferior to that of the general population.

ABBREVIATIONS

BCR = bulbocavernosus reflex; CEE = cauda equina ependymoma; EII = grade II ependymoma; GTR = gross-total resection; HRQOL = health-related quality of life; IONM = intraoperative neurophysiological monitoring; MEP = muscle motor evoked potential; MPE = myxopapillary ependymoma; PFS = progression-free survival; RT = radiation therapy; SEP = somatosensory evoked potential; STR = subtotal resection; VAS = visual analog scale.

Spine - 1 year subscription bundle (Individuals Only)

USD  $376.00

JNS + Pediatrics + Spine - 1 year subscription bundle (Individuals Only)

USD  $612.00
  • 1

    Gilbert MR, Ruda R, Soffietti R. Ependymomas in adults. Curr Neurol Neurosci Rep. 2010;10(3):240247.

  • 2

    Xie TH, Chen XZ, Qian J, Lu YC, Jiang YK, Zhang L, et al. Surgery for primary filum terminale ependymomas: outcome and prognostic factors. CNS Neurosci Ther. 2014;20(2):131139.

    • Search Google Scholar
    • Export Citation
  • 3

    Louis DN, Ohgaki H, Wiestier OD, Cavenee WK, Ellison DW, Figarella-Branger D, et al. WHO Classification of Tumours of the Central Nervous System. Vol 1.World Health Organization;2016.http://www.amazon.co.uk/classification-tumours-central-nervous-system/dp/9283224302

    • Search Google Scholar
    • Export Citation
  • 4

    de Jong L, Calenbergh FV, Menten J, van Loon J, De Vleeschouwer S, Plets C, et al. Ependymomas of the filum terminale: the role of surgery and radiotherapy. Surg Neurol Int. 2012;3(1):76.

    • Search Google Scholar
    • Export Citation
  • 5

    McCormick PC, Torres R, Post KD, Stein BM. Intramedullary ependymoma of the spinal cord. J Neurosurg. 1990;72(4):523532.

  • 6

    Kesselring J. Multiple Sklerose. Kohlhammer;. 1989.

  • 7

    Herdman M, Gudex C, Lloyd A, Janssen M, Kind P, Parkin D, et al. Development and preliminary testing of the new five-level version of EQ-5D (EQ-5D-5L). Qual Life Res. 2011;20(10):17271736.

    • Search Google Scholar
    • Export Citation
  • 8

    Tarantino R, Donnarumma P, Nigro L, Rullo M, Santoro A, Delfini R. Surgery of intradural extramedullary tumors: retrospective analysis of 107 cases. Neurosurgery. 2014;75(5):509514.

    • Search Google Scholar
    • Export Citation
  • 9

    Viereck MJ, Ghobrial GM, Beygi S, Harrop JS. Improved patient quality of life following intradural extramedullary spinal tumor resection. J Neurosurg Spine. 2016;25(5):640645.

    • Search Google Scholar
    • Export Citation
  • 10

    Schwab JH, Janssen SJ, Paulino Pereira NR, Chen YLE, Wain JC, DeLaney TF, Hornicek FJ. Quality of life after resection of a chordoma of the mobile spine. Bone Joint J. 2017;99-B(7):979986.

    • Search Google Scholar
    • Export Citation
  • 11

    Scalone L, Cortesi PA, Ciampichini R, Cesana G, Mantovani LG. Health related quality of life norm data of the general population in Italy: results using the EQ-5D-3L and EQ-5D-5L instruments. Epidemiol Biostat Public Health. 2015;12(3):e11457.

    • Search Google Scholar
    • Export Citation
  • 12

    Gerstner ER, Pajtler KW. Ependymoma. Semin Neurol. 2018;38(1):104111.

  • 13

    Cervoni L, Celli P, Cantore G, Fortuna A. Intradural tumors of the cauda equina: a single institution review of clinical characteristics. Clin Neurol Neurosurg. 1995;97(1):812.

    • Search Google Scholar
    • Export Citation
  • 14

    Klekamp J. Spinal ependymomas. Part 2: Ependymomas of the filum terminale. Neurosurg Focus. 2015;39(2):E7.

  • 15

    Abdulaziz M, Mallory GW, Bydon M, Dela Garza Ramos R, Ellis JA, Laack NN, et al. Outcomes following myxopapillary ependymoma resection: the importance of capsule integrity. Neurosurg Focus. 2015;39(2):E8.

    • Search Google Scholar
    • Export Citation
  • 16

    Pica A, Miller R, Villà S, Kadish SP, Anacak Y, Abusaris H, et al. 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. 2009;74(4):11141120.

    • Search Google Scholar
    • Export Citation
  • 17

    Jeibmann A, Egensperger R, Kuchelmeister K, Sepehrnia A, Stolke D, Bruns B, et al. Extent of surgical resection but not myxopapillary versus classical histopathological subtype affects prognosis in lumbo-sacral ependymomas. Histopathology. 2009;54(2):260262.

    • Search Google Scholar
    • Export Citation
  • 18

    Wager M, Lapierre F, Blanc JL, Listrat A, Bataille B. Cauda equina tumors: a French multicenter retrospective review of 231 adult cases and review of the literature. Neurosurg Rev. 2000;23(3):119131.

    • Search Google Scholar
    • Export Citation
  • 19

    Sonneland PRL, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma A clinicopathologic and immunocytochemical study of 77 cases. Cancer. 1985;56(4):883893.

    • Search Google Scholar
    • Export Citation
  • 20

    Akyurek S, Chang EL, Yu TK, Little D, Allen PK, McCutcheon I, et al. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M. DAnderson Cancer Center. J Neurooncol. 2006;80(2):177183.

    • Search Google Scholar
    • Export Citation
  • 21

    Nakamura M, Ishii K, Watanabe K, Tsuji T, Matsumoto M, Toyama Y, Chiba K. Long-term surgical outcomes for myxopapillary ependymomas of the cauda equina. Spine (Phila Pa 1976).2009;34(21):E756E760.

    • Search Google Scholar
    • Export Citation
  • 22

    Tsai CJ, Wang Y, Allen PK, Mahajan A, McCutcheon IE, Rao G, et al. Outcomes after surgery and radiotherapy for spinal myxopapillary ependymoma: update of the MD Anderson Cancer Center experience. Neurosurgery. 2014;75(3):205214.

    • Search Google Scholar
    • Export Citation
  • 23

    Liu T, Yang C, Deng X, Li A, Xin Y, Yang J, Xu Y. Clinical characteristics and surgical outcomes of spinal myxopapillary ependymomas. Neurosurg Rev. 2020;43(5):13511356.

    • Search Google Scholar
    • Export Citation
  • 24

    Weber DC, Wang Y, Miller R, Villà S, Zaucha R, Pica A, et al. Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro Oncol. 2015;17(4):588595.

    • Search Google Scholar
    • Export Citation
  • 25

    Balasubramaniam S, Tyagi DK, Desai KI, Dighe MP. Outcome analysis in cases of spinal conus cauda ependymoma. J Clin Diagn Res. 2016;10(9):PC12PC16.

    • Search Google Scholar
    • Export Citation
  • 26

    Shin DA, Yang JW, Yi S, Ha Y, Yoon DH, Kim KN. Clinical features and surgical outcomes of primary cauda equina tumours. Acta Neurochir (Wien). 2013;155(10):19111916.

    • Search Google Scholar
    • Export Citation
  • 27

    Tonogai I, Sakai T, Tezuka F, Goda Y, Takata Y, Higashino K, Sairyo K. Spontaneous rupture and hemorrhage of myxopapillary ependymoma of the filum terminale: a case report and literature review. J Med Invest. 2014;61(3-4):430435.

    • Search Google Scholar
    • Export Citation
  • 28

    Yuh EL, Barkovich AJ, Gupta N. Imaging of ependymomas: MRI and CT. Childs Nerv Syst. 2009;25(10):12031213.

  • 29

    Koeller KK, Shih RY. Intradural extramedullary spinal neoplasms: radiologic-pathologic correlation. Radiographics. 2019;39(2):468490.

    • Search Google Scholar
    • Export Citation
  • 30

    Taskiran E, Ulu MO, Akcil EF, Hanci M. Intraoperative neuromonitoring in surgery of cauda equina and conus medullaris tumors. Turk Neurosurg. 2019;29(6):909914.

    • Search Google Scholar
    • Export Citation
  • 31

    Sala F, Palandri G, Basso E, Lanteri P, Deletis V, Faccioli F, Bricolo A. Motor evoked potential monitoring improves outcome after surgery for intramedullary spinal cord tumors: a historical control study. Neurosurgery. 2006;58(6):11291143.

    • Search Google Scholar
    • Export Citation
  • 32

    Barber SM, Fridley JS, Konakondla S, Nakhla J, Oyelese AA, Telfeian AE, Gokaslan ZL. Cerebrospinal fluid leaks after spine tumor resection: avoidance, recognition and management. Ann Transl Med. 2019;7(10):217.

    • Search Google Scholar
    • Export Citation
  • 33

    Kucia EJ, Maughan PH, Kakarla UK, Bambakidis NC, Spetzler RF. Surgical technique and outcomes in the treatment of spinal cord ependymomas: part II: myxopapillary ependymoma. Neurosurgery. 2011;68(1)(Suppl Operative):9094.

    • Search Google Scholar
    • Export Citation
  • 34

    Pesce A, Palmieri M, Armocida D, Frati A, Miscusi M, Raco A. Spinal myxopapillary ependymoma: the Sapienza University experience and comprehensive literature review concerning the clinical course of 1602 patients. World Neurosurg. 2019;129:245253.

    • Search Google Scholar
    • Export Citation
  • 35

    Lee SH, Chung CK, Kim CH, Yoon SH, Hyun SJ, Kim KJ, et al. Long-term outcomes of surgical resection with or without adjuvant radiation therapy for treatment of spinal ependymoma: a retrospective multicenter study by the Korea Spinal Oncology Research Group. Neuro Oncol. 2013;15(7):921929.

    • Search Google Scholar
    • Export Citation
  • 36

    Feldman WB, Clark AJ, Safaee M, Ames CP, Parsa AT. Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. J Neurosurg Spine. 2013;19(4):471476.

    • Search Google Scholar
    • Export Citation
  • 37

    Bagley CA, Wilson S, Kothbauer KF, Bookland MJ, Epstein F, Jallo GI. Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev. 2009;32(3):321334.

    • Search Google Scholar
    • Export Citation
  • 38

    Chan HSL, Becker LE, Hoffman HJ, Humphreys RP, Hendrick EB, Fitz CR, Chuang SH. Myxopapillary ependymoma of the filum terminale and cauda equina in childhood: report of seven cases and review of the literature. Neurosurgery. 1984;14(2):204210.

    • Search Google Scholar
    • Export Citation
  • 39

    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(6):16221631.

    • Search Google Scholar
    • Export Citation
  • 40

    Konovalov NA, Golanov AV, Shevelev IN, Nazarenko AG, Asyutin DS, Korolishin VA, et al. The outcomes of treatment of cauda equina ependymomas in adults. Article in Russian. Vopr Neirokhir. 2015;79(1):5867.

    • Search Google Scholar
    • Export Citation
  • 41

    Svoboda N, Bradac O, de Lacy P, Benes V. Intramedullary ependymoma: long-term outcome after surgery. Acta Neurochir (Wien). 2018;160(3):439447.

    • Search Google Scholar
    • Export Citation
  • 42

    Uchiyama T, Sakakibara R, Hattori T, Yamanishi T. Lower urinary tract dysfunctions in patients with spinal cord tumors. Neurourol Urodyn. 2004;23(1):6875.

    • Search Google Scholar
    • Export Citation
  • 43

    Xiao R, Miller JA, Abdullah KG, Lubelski D, Mroz TW, Benzel EC. Quality of life outcomes following resection of adult intramedullary spinal cord tumors. Neurosurgery. 2016;78(6):821828.

    • Search Google Scholar
    • Export Citation

Metrics

All Time Past Year Past 30 Days
Abstract Views 332 332 118
Full Text Views 54 54 8
PDF Downloads 65 65 8
EPUB Downloads 0 0 0