Abstract
Background
Myxopapillary ependymoma (MPE) is a rare subtype of ependymoma that develops almost exclusively within the spinal cord. Despite its benign biological nature, MPE has a propensity to recur locally or distantly. Although variables influencing the prognosis, such as age, the extent of surgery and radiotherapy, have been widely discussed, no definitive standard has been established. Compared to other spinal tumors, many fewer histological markers have been elucidated to assist the determination of the prognosis.
Methods
Twenty-seven patients who underwent resection of MPE were enrolled. We determined their demographic features, imaging characteristics, clinical presentations and outcomes, surgical procedures and histological properties by chart review, telephone contact, reviewing of surgical notes, pre-/postoperative imaging and immunohistological staining.
Results
GTR (gross total resection) was achieved in 18 patients (66.7 %) and STR (subtotal resection) in 9 (33.3 %). Although GTR rendered a better disease control rate, the difference was not significant. Pediatric patients suffered from a greater risk of recurrence as well as a shorter period to disease relapse. In the majority of cases, we observed the overexpression of platelet-derived growth factor receptor α (PDGFRα), matrix metalloproteinase-2 (MMP2) and matrix metalloproteinase-14 (MMP14). Epidermal growth factor receptor (EGFR) was observed in the tumors of 7 of 23 nonrecurrent patients, but not in any recurrent tumors.
Conclusions
The results of the present study indicate that the extent of resection and age are major factors related to tumor recurrence. Therefore, gross total resection is recommended whenever possible unless following neurological dysfunction is predictable. Moreover, pediatric patients need considerable attention after surgery, particularly in the early stages. PDGFRα, MMP2 and MMP14 may be new diagnostic and therapeutic targets and EGFR a potential predictor of improved prognosis for MPE.
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References
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
Akyurek S, Chang EL, Yu TK, Little D, Allen PK, McCutcheon I, Mahajan A, Maor MH, Woo SY (2006) Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at MD Anderson Cancer Center. J Neurooncol 80:177–183
Al-Habib A, Al-Radi OO, Shannon P, Al-Ahmadi H, Petrenko Y, Fehlings MG (2011) Myxopapillary ependymoma: correlation of clinical and imaging features with surgical resectability in a series with long-term follow-up. Spinal Cord 49:1073–1078
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
Bagley CA, Kothbauer KF, Wilson S, Bookland MJ, Epstein FJ, Jallo GI (2007) Resection of myxopapillary ependymomas in children. J Neurosurg 106:261–267
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, discussion 334
Barton VN, Donson AM, Kleinschmidt-DeMasters BK, Birks DK, Handler MH, Foreman NK (2010) Unique molecular characteristics of pediatric myxopapillary ependymoma. Brain Pathol 20:560–570
Bodey B, Kaiser HE, Siegel SE (2005) Epidermal growth factor receptor (EGFR) expression in childhood brain tumors. In Vivo 19:931–941
Chakraborti S, Govindan A, Alapatt JP, Radhakrishnan M, Santosh V (2012) Primary myxopapillary ependymoma of the fourth ventricle with cartilaginous metaplasia: a case report and review of the literature. Brain Tumor Pathol 29:25–30
Chinn DM, Donaldson SS, Dahl GV, Wilson JD, Huhn SL, Fisher PG (2000) Management of children with metastatic spinal myxopapillary ependymoma using craniospinal irradiation. Med Pediatr Oncol 35:443–445
Cho HY, Lee M, Takei H, Dancer J, Ro JY, Zhai QJ (2009) Immunohistochemical comparison of chordoma with chondrosarcoma, myxopapillary ependymoma, and chordoid meningioma. Appl Immunohistochem Mol Morphol 17:131–138
de Jonge T, Slullitel H, Dubousset J, Miladi L, Wicart P, Illes T (2005) Late-onset spinal deformities in children treated by laminectomy and radiation therapy for malignant tumours. Eur Spine J 14:765–771
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
Fuller GN, Scheithauer BW (2007) The 2007 Revised World Health Organization (WHO) Classification of Tumours of the Central Nervous System: newly codified entities. Brain Pathol 17:304–307
Ilhan A, Furtner J, Birner P, Rossler K, Marosi C, Preusser M (2011) Myxopapillary ependymoma with pleuropulmonary metastases and high plasma glial fibrillary acidic protein levels. J Clin Oncol 29:e756–e757
Ilhan-Mutlu A, Berghoff AS, Furtner J, Dieckmann K, Slavc I, Czech T, Marosi C, Wagner L, Preusser M (2013) High plasma-GFAP levels in metastatic myxopapillary ependymoma. J Neurooncol 113:359–363
Joaquim AF, Cheng I, Patel AA (2012) Postoperative spinal deformity after treatment of intracanal spine lesions. Spine J 12:1067–1074
Kernohan JW (1932) Primary tumors of the spinal cord and intradural filum terminale. In: Penfield W (ed) Cytology and cellular pathology of the nervous system, vol 3. Paul B Hoeber, New York, pp 993–1025
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
Kukreja S, Ambekar S, Sin AH, Nanda A (2014) Cumulative survival analysis of patients with spinal myxopapillary ependymomas in the first 2 decades of life. J Neurosurg Pediatr 13:400–407
Marquez A, Wu R, Zhao J, Tao J, Shi Z (2004) Evaluation of epidermal growth factor receptor (EGFR) by chromogenic in situ hybridization (CISH) and immunohistochemistry (IHC) in archival gliomas using bright-field microscopy. Diagn Mol Pathol 13:1–8
Mridha AR, Sharma MC, Sarkar C, Suri V, Rishi A, Garg A, Suri A (2007) Myxopapillary ependymoma of lumbosacral region with metastasis to both cerebellopontine angles: report of a rare case. Childs Nerv Syst 23:1209–1213
Pica A, Miller R, Villa S, Kadish SP, Anacak Y, Abusaris H, Ozyigit G, Baumert BG, Zaucha R, Haller G, Weber DC (2009) 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 74:1114–1120
Rao IS, Kapila K, Aggarwal S, Ray R, Gupta AK, Verma K (2002) Subcutaneous myxopapillary ependymoma presenting as a childhood sacrococcygeal tumor: a case report. Diagn Cytopathol 27:303–307
Schild SE, Nisi K, Scheithauer BW, Wong WW, Lyons MK, Schomberg PJ, Shaw EG (1998) The results of radiotherapy for ependymomas: the Mayo Clinic experience. Int J Radiat Oncol Biol Phys 42:953–958
Snuderl M, Chi SN, De Santis SM, Stemmer-Rachamimov AO, Betensky RA, De Girolami U, Kieran MW (2008) Prognostic value of tumor microinvasion and metalloproteinases expression in intracranial pediatric ependymomas. J Neuropathol Exp Neurol 67:911–920
Sonneland PR, Scheithauer BW, Onofrio BM (1985) Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer 56:883–893
Tripp SR, Willmore-Payne C, Layfield LJ (2005) Relationship between EGFR overexpression and gene amplification status in central nervous system gliomas. Anal Quant Cytol Histol 27:71–78
Tzerakis N, Georgakoulias N, Kontogeorgos G, Mitsos A, Jenkins A, Orphanidis G (2004) Intraparenchymal myxopapillary ependymoma: case report. Neurosurgery 55:981
Verma A, Zhou H, Chin S, Bruggers C, Kestle J, Khatua S (2012) EGFR as a predictor of relapse in myxopapillary ependymoma. Pediatr Blood Cancer 59:746–748
Wang H, Zhang S, Rehman SK, Zhang Z, Li W, Makki MS, Zhou X (2014) Clinicopathological features of myxopapillary ependymoma. J Clin Neurosci 21:569–573
Weber DC, Wang Y, Miller R, Villa S, Zaucha R, Pica A, Poortmans P, Anacak Y, Ozygit G, Baumert B, Haller G, Preusser M, Li J (2014) 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 17:588–595
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** Chen and Chao Li contributed equally to this work. The manuscript was written with contributions of all authors. All authors have given approval to the final version of the manuscript.
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Chen, X., Li, C., Che, X. et al. Spinal myxopapillary ependymomas: a retrospective clinical and immunohistochemical study. Acta Neurochir 158, 101–107 (2016). https://doi.org/10.1007/s00701-015-2637-8
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DOI: https://doi.org/10.1007/s00701-015-2637-8