Abstract
Primary spinal myxopapillary ependymomas (MPE) in children are extremely rare. We examined the patient demographics, treatment modalities, and the associated outcomes of children with MPE using the Surveillance, Epidemiology, and End Results (SEER) national cancer database to gain a better understanding of these tumors. The SEER database (1973–2012) was used to analyze patients 21 years of age and younger with histologically confirmed MPE localized to the spinal cord or cauda equina. We analyzed patient demographics, extent of surgical resection, and radiation treatment. Overall survival was calculated using the Kaplan–Meier method. Statistical significance was defined as p < 0.05, with all data analyzed in IBM SPSS Statistics 21. 122 pediatric patients met inclusion criteria. The median age was 16 years (range 0–21) with 63 % male and 87 % Caucasian. The mean follow-up time was 4.5 years (95 % CI 3.93–5.07). Overall survival at 5 and 10 years was 97 and 95 %, respectively. We found 37 % underwent gross-total resection (GTR), 36 % subtotal resection (STR), and 27 % biopsy only. Patients who received GTR alone (n = 37) had a statistically significant increase in overall survival compared to those who received STR plus adjuvant radiation (n = 20) (Χ2 = 5.9, p < 0.05). To our knowledge, this is the largest survival analysis of pediatric patients with MPE. Overall survival is excellent at the 5 and 10-year time points; however, GTR should be the goal of treatment when possible. For patients with MPE, future studies should focus on longer follow-up, the role of radiation, and the therapeutic approach at tumor recurrence.
Similar content being viewed by others
References
Mack SC, Agnihotri S, Bertrand KC, Wang X, Shih DJ, Witt H, Hill N, Zayne K, Barszczyk M, Ramaswamy V, Remke M, Thompson Y, Ryzhova M, Massimi L, Grajkowska W, Lach B, Gupta N, Weiss WA, Guha A, Hawkins C, Croul S, Rutka JT, Pfister SM, Korshunov A, Pekmezci M, Tihan T, Philips JJ, Jabado N, Zadeh G, Taylor MD (2015) Spinal myxopapillary ependymomas demonstrate a warburg phenotype. Clin Cancer Res. doi:10.1158/1078-0432.CCR-14-2650
Safaee M, Oh MC, Mummaneni PV, Weinstein PR, Ames CP, Chou D, Berger MS, Parsa AT, Gupta N (2014) Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults. J Neurosurg Pediatr 13:393–399. doi:10.3171/2013.12.PEDS13383
Bagley CA, Wilson S, Kothbauer KF, Bookland MJ, Epstein F, Jallo GI (2009) Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev 32:321–334. doi:10.1007/s10143-009-0190-8 (discussion 334)
Al-Halabi H, Montes JL, Atkinson J, Farmer JP, Freeman CR (2010) Adjuvant radiotherapy in the treatment of pediatric myxopapillary ependymomas. Pediatr Blood Cancer 55:639–643. doi:10.1002/pbc.22614
Lin Y, Smith ZA, Wong AP, Melkonian S, Harris DA, Lam S (2015) Predictors of survival in patients with spinal ependymoma. Neurol Res 37:650–655. doi:10.1179/1743132815Y.0000000041
Bagley CA, Kothbauer KF, Wilson S, Bookland MJ, Epstein FJ, Jallo GI (2007) Resection of myxopapillary ependymoma in children. J Neurosurg 106(4 Suppl):261–267
Agbahiwe HC, Wharam M, Batra S, Cohen K, Terezakis SA (2013) Management of pediatric myxopapillary ependymoma: the role of adjuvant radiation. Int J Radiat Oncol Biol Phys 85:421–427. doi:10.1016/j.ijrobp.2012.05.001
Lin Y, Jea A, Melkonian SC, Lam S (2015) Treatment of pediatric Grade II spinal ependymomas: a population-based study. J Neurosurg Pediatr 15:243–249. doi:10.3171/2014.9.PEDS1473
Feldman WB, Clark AJ, Safaee M, Ames CP, Parsa AT (2013) Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. J Neurosurg Spine 19:471–476. doi:10.3171/2013.6.SPINE12927
Kukreja S, Ambekar S, Sharma M, Sin AH, Nanda A (2015) Outcome predictors in the management of spinal myxopapillary ependymoma: an integrative survival analysis. World Neurosurg 83:852–859. doi:10.1016/j.wneu.2014.08.006
Stephen JH, Sievert AJ, Madsen PJ, Judkins AR, Resnick AC, Storm PB, Rushing EJ, Santi M (2012) Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases. J Neurosurg Pediatr 9:646–653. doi:10.3171/2012.2.PEDS11285
Benesch M, Frappaz D, Massimino M (2012) Spinal cord ependymomas in children and adolescents. Childs Nerv Syst 28:2017–2028. doi:10.1007/s00381-012-1908-4
Benesch M, Weber-Mzell D, Gerber NU, von Hoff K, Deinlein F, Krauss J, Warmuth-Metz M, Kortmann RD, Pietsch T, Driever PH, Quehenberger F, Urban C, Rutkowski S (2010) Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database. J Neurosurg Pediatr 6:137–144 doi:10.3171/2010.5.PEDS09553
Lundar T, Due-Tonnessen BJ, Scheie D, Brandal P (2014) Pediatric spinal ependymomas: an unpredictable and puzzling disease. Long-term follow-up of a single consecutive institutional series of ten patients. Childs Nerv Syst 30:2083–2088. doi:10.1007/s00381-014-2491-7
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have no conflict of interests.
Ethical approval
This study used the publically available SEER database and is considered non-human subjects research. An IRB exemption letter was received from both Quinnipiac University School of Medicine and Yale University institutional review boards. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study formal consent is not required.
Rights and permissions
About this article
Cite this article
Lucchesi, K.M., Grant, R., Kahle, K.T. et al. Primary spinal myxopapillary ependymoma in the pediatric population: a study from the Surveillance, Epidemiology, and End Results (SEER) database. J Neurooncol 130, 133–140 (2016). https://doi.org/10.1007/s11060-016-2218-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11060-016-2218-6