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Pediatric spinal intramedullary anaplastic myxopapillary ependymoma: a case report

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Abstract

A 6-year-old girl presented with a 1-week history of progressive upper and lower extremity weakness and bilateral upper extremity dysesthesia. Imaging demonstrated a 4.7 × 1.2-cm enhancing intramedullary lesion in the cervical spine from level C2 to C5 with associated cystic components and syringomyelia. The patient underwent a C2–C5 laminoplasty, with gross total resection of the intramedullary lesion. Histological analysis showed small to medium-sized epithelioid cells, with predominantly a solid architecture focally infiltrating into the adjacent spinal cord tissue. Focal papillary differentiation was present along with peri-vascular pseudorosettes, mucin microcysts, and globules of dense collagen. Focal anaplasia was noted with mitosis (5/10 HPF), focal necrosis, and elevated Ki67 10–15%. These findings were consistent with a myxopapillary ependymoma with anaplastic features. CSF cytology was negative for tumor cells. MYCN amplification was not present. She was treated with targeted proton-beam radiation therapy. This is the fourth case of an intramedullary anaplastic myxopapillary ependymoma to date, and the first case in the cervical spine reported in the literature.

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References

  1. 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. https://doi.org/10.1016/j.ijrobp.2012.05.001

    Article  PubMed  Google Scholar 

  2. 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. https://doi.org/10.1002/pbc.22614

    Article  PubMed  Google Scholar 

  3. Amsbaugh MJ, Grosshans DR, McAleer MF, Zhu R, Wages C, Crawford CN, Palmer M, De Gracia B, Woo S, Mahajan A (2012) Proton therapy for spinal ependymomas: planning, acute toxicities, and preliminary outcomes. Int J Radiat Oncol Biol Phys 83:1419–1424. https://doi.org/10.1016/j.ijrobp.2011.10.034

    Article  PubMed  Google Scholar 

  4. Awaya H, Kaneko M, Amatya VJ, Takeshima Y, Oka S, Inai K (2003) Myxopapillary ependymoma with anaplastic features. Pathol Int 53:700–703. https://doi.org/10.1046/j.1440-1827.2003.01546.x

    Article  PubMed  Google Scholar 

  5. 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. https://doi.org/10.1007/s10143-009-0190-8

    Article  PubMed  Google Scholar 

  6. Bandopadhayay P, Silvera VM, Ciarlini P, Malkin H, Bi WL, Bergthold G, Faisal AM, Ullrich NJ, Marcus K, Scott RM, Beroukhim R, Manley PE, Chi SN, Ligon KL, Goumnerova LC, Kieran MW (2016) Myxopapillary ependymomas in children: imaging, treatment and outcomes. J Neurooncol 126:165–174. https://doi.org/10.1007/s11060-015-1955-2

    Article  PubMed  Google Scholar 

  7. Beschorner R, Wehrmann M, Ernemann U, Bonin M, Horber V, Oehl-Jaschkowitz B, Meyermann R, Dufke A (2007) Extradural ependymal tumor with myxopapillary and ependymoblastic differentiation in a case of Schinzel-Giedion syndrome. Acta Neuropathol 113:339–346. https://doi.org/10.1007/s00401-006-0179-0

    Article  PubMed  Google Scholar 

  8. Capper D, Jones DTW, Sill M, Hovestadt V, Schrimpf D, Sturm D, Koelsche C, Sahm F, Chavez L, Reuss DE, Kratz A, Wefers AK, Huang K, Pajtler KW, Schweizer L, Stichel D, Olar A, Engel NW, Lindenberg K, Harter PN, Braczynski AK, Plate KH, Dohmen H, Garvalov BK, Coras R, Hölsken A, Hewer E, Bewerunge-Hudler M, Schick M, Fischer R, Beschorner R, Schittenhelm J, Staszewski O, Wani K, Varlet P, Pages M, Temming P, Lohmann D, Selt F, Witt H, Milde T, Witt O, Aronica E, Giangaspero F, Rushing E, Scheurlen W, Geisenberger C, Rodriguez FJ, Becker A, Preusser M, Haberler C, Bjerkvig R, Cryan J, Farrell M, Deckert M, Hench J, Frank S, Serrano J, Kannan K, Tsirigos A, Brück W, Hofer S, Brehmer S, Seiz-Rosenhagen M, Hänggi D, Hans V, Rozsnoki S, Hansford JR, Kohlhof P, Kristensen BW, Lechner M, Lopes B, Mawrin C, Ketter R, Kulozik A, Khatib Z, Heppner F, Koch A, Jouvet A, Keohane C, Mühleisen H, Mueller W, Pohl U, Prinz M, Benner A, Zapatka M, Gottardo NG, Driever PH, Kramm CM, Müller HL, Rutkowski S, von Hoff K, Frühwald MC, Gnekow A, Fleischhack G, Tippelt S, Calaminus G, Monoranu CM, Perry A, Jones C, Jacques TS, Radlwimmer B, Gessi M, Pietsch T, Schramm J, Schackert G, Westphal M, Reifenberger G, Wesseling P, Weller M, Collins VP, Blümcke I, Bendszus M, Debus J, Huang A, Jabado N, Northcott PA, Paulus W, Gajjar A, Robinson GW, Taylor MD, Jaunmuktane Z, Ryzhova M, Platten M, Unterberg A, Wick W, Karajannis MA, Mittelbronn M, Acker T, Hartmann C, Aldape K, Schüller U, Buslei R, Lichter P, Kool M, Herold-Mende C, Ellison DW, Hasselblatt M, Snuderl M, Brandner S, Korshunov A, von Deimling A, Pfister SM (2018) DNA methylation-based classification of central nervous system tumours. Nature 555:469–474. https://doi.org/10.1038/nature26000

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Chakraborti S, Kini H, Pai KG, Upadhyaya V (2012) Sacrococcygeal myxopapillary ependymoma with anaplastic ependymoma component in an infant. J Pediatr Neurosci 7:218–220. https://doi.org/10.4103/1817-1745.106485

    Article  PubMed  PubMed Central  Google Scholar 

  10. Ellison DW, Aldape KD, Capper D, Fouladi M, Gilbert MR, Gilbertson RJ, Hawkins C, Merchant TE, Pajtler K, Venneti S, Louis DN (2020) cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Brain Pathol. https://doi.org/10.1111/bpa.12866

  11. Fassett DR, Pingree J, Kestle JR (2005) The high incidence of tumor dissemination in myxopapillary ependymoma in pediatric patients. Report of five cases and review of the literature. J Neurosurg 102:59–64. https://doi.org/10.3171/ped.2005.102.1.0059

    Article  PubMed  Google Scholar 

  12. Ghasemi DR, Sill M, Okonechnikov K, Korshunov A, Yip S, Schutz PW, Scheie D, Kruse A, Harter PN, Kastelan M, Wagner M, Hartmann C, Benzel J, Maass KK, Khasraw M, Sträter R, Thomas C, Paulus W, Kratz CP, Witt H, Kawauchi D, Herold-Mende C, Sahm F, Brandner S, Kool M, Jones DTW, von Deimling A, Pfister SM, Reuss DE, Pajtler KW (2019) MYCN amplification drives an aggressive form of spinal ependymoma. Acta Neuropathol 138:1075–1089. https://doi.org/10.1007/s00401-019-02056-2

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Huynh TR, Lu C, Drazin D, Lekovic G (2018) Myxopapillary ependymoma with anaplastic features: a case report with review of the literature. Surg Neurol Int 9:191. https://doi.org/10.4103/sni.sni_422_17

    Article  PubMed  PubMed Central  Google Scholar 

  14. Lee JC, Sharifai N, Dahiya S, Kleinschmidt-DeMasters BK, Rosenblum MK, Reis GF, Samuel D, Siongco AM, Santi M, Storm PB, Ferris SP, Bollen AW, Pekmezci M, Solomon DA, Tihan T, Perry A (2019) Clinicopathologic features of anaplastic myxopapillary ependymomas. Brain Pathol 29:75–84. https://doi.org/10.1111/bpa.12673

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Rudà R, Reifenberger G, Frappaz D, Pfister SM, Laprie A, Santarius T, Roth P, Tonn JC, Soffietti R, Weller M, Moyal EC (2018) EANO guidelines for the diagnosis and treatment of ependymal tumors. Neuro Oncol 20:445–456. https://doi.org/10.1093/neuonc/nox166

    Article  PubMed  Google Scholar 

  16. Sonneland PR, Scheithauer BW, Onofrio BM (1985) Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer 56:883–893. https://doi.org/10.1002/1097-0142(19850815)56:4<883::aid-cncr2820560431>3.0.co;2-6

    Article  CAS  PubMed  Google Scholar 

  17. Swanson AA, Raghunathan A, Jenkins RB, Messing-Jünger M, Pietsch T, Clarke MJ, Kaufmann TJ, Giannini C (2019) Spinal cord ependymomas with MYCN amplification show aggressive clinical behavior. J Neuropathol Exp Neurol 78:791–797. https://doi.org/10.1093/jnen/nlz064

    Article  PubMed  Google Scholar 

  18. Trivedi D, Xiong Z (2017) Anaplastic myxopapillary ependymoma in an infant: case report and literature review. Intractable Rare Dis Res 6:128–131. https://doi.org/10.5582/irdr.2017.01015

    Article  PubMed  PubMed Central  Google Scholar 

  19. Weber DC, Wang Y, Miller R, Villà S, Zaucha R, Pica A, Poortmans P, Anacak Y, Ozygit G, Baumert B, Haller G, Preusser M, Li J (2015) 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. https://doi.org/10.1093/neuonc/nou293

    Article  PubMed  Google Scholar 

  20. Wu J, Armstrong TS, Gilbert MR (2016) Biology and management of ependymomas. Neuro Oncol 18:902–913. https://doi.org/10.1093/neuonc/now016

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Correspondence to Tord D. Alden.

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Trybula, S.J., Wadhwani, N.R., Mohammad, L.M. et al. Pediatric spinal intramedullary anaplastic myxopapillary ependymoma: a case report. Childs Nerv Syst 38, 223–227 (2022). https://doi.org/10.1007/s00381-021-05171-2

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