EC Paediatrics

Introduction: Myxopapillary ependymoma is a specific type of ependymoma that in very rare cases can occur in the extraspinal soft tissues. Categorized as grade I lesions myxopapillary ependymomas are considered benign but are capable of local recurrence, dissemination and a more aggressive disease course.

Case: The patient is a six year old boy with a formation in the sacral area with approximate dimensions 5 x 5 cm. Primary suspicion was hematoma because of history of repeated trauma to the buttocks. Ultrasonographic and computed tomography scans revealed the precise location, size and blood supply of the tumor. Gross- total resection was attempted.

Results: The sacrum and coccygeal bone were found to be intact and en-bloc resection was feasible. Histopathological result indicated extramedullary myxopapillary ependymoma. Postoperative period was uneventful. Follow-up magnetic resonance scan revealed normal postoperative finds and no signs of residual formation and/or recurrence.

Conclusion: Even though a very rare tumor, ME should be considered a possibility for sacral region tumors. Keeping in mind the potential of the tumor for local recurrence and metastases, it is advised that extradural subcutaneous ME be considered a low-grade malignant tumor and gross-total excision should be performed in all cases. Periodical postoperative examination is warranted for long term tumor control.     

 Keywords: Extradural Ependymoma; Myxopapillary; Sacral Tumor

1. Kernohan JW. “Primary tumors of the spinal cord and intradural filum terminale”. Cytology and Cellular Athology of the Nervous System 3 (1932): 993-1025.
2. Zaidi SN., et al. “Myxopapillary ependymoma masquerading as subcutaneous saccroccygeal non-healing ulcer: Case report”. Annals of Saudi Medicine 3 (2014): 262-264.
3. Shaw EG., et al. “Radiotherapeutic management of adult intraspinal ependymomas”. International Journal of Radiation Oncology, Biology, Physics 3 (1986): 323-327.
4. Wiestler OD., et al. “Ependymomas”. In Kleihues P, Cavernee WK (eds): Pathology and Genetics of Tumors of the Nervous System, ed 2. Lyon: IARC Press (2000): 50-56.
5. Chinn DM., et al. “Management of children with metastatic spinal myxopapillary ependymoma using craniospinal irradiation”. Medical and Pediatric Oncology 4 (2000): 443-445.
6. Gagliardi FM., et al. “Ependymomas of the filum terminale in childhood: report of four cases and review of the literature”. Child's Nervous System 1 (1993): 3-6.
7. Nagib MG and O’Fallon MT. “Myxopapillary ependymoma of the conus medullaris and filum terminale in the pediatric age group”. Pediatric Neurosurgery 1 (1997): 2-7.
8. Fassett DR and Schmidt MH. “Lumbosacral ependymomas: a review of the management of intradural and extradural tumors”. Neurosurgical Focus 5 (2003): E13.
9. Fourney DR., et al. “Intraspinal extradural myxopapillary ependymoma of the sacrum arising from the filum terminale externa. Case report”. Journal of Neurosurgery 2 (2000): 322-326.
10. Morantz RA., et al. “Extraspinal ependymomas. Report of three cases”. Journal of Neurosurgery 3 (1979): 383-391.
11. Helwig EB and Stern JB. “Subcutaneous sacrococcygeal myxopapillary ependymoma. A clinicopathologic study of 32 cases”. American Journal of Clinical Pathology 2 (1984): 156-161.
12. Lynch J., et al. “A sacrococcygeal extraspinal ependymoma in a 67-year-old man: a case report and review of the literature”. British Journal of Plastic Surgery 1 (2002): 80-82.
13. Morantz RA. “Ectopic ependymoma of the sacrococcygeal region”. In Doty JR, Rengachary SS (eds): Surgical Disorders of the Sacrum. New York: Theime Medical (1992): 177-179.
14. Schurmann K and Wallenfang T. “Rare sacral space-occupying lesions, their surgical management and reconstructive measures involved”. Acta Neurochirurgica 1-4 (1988): 106-117.
15. Gerston KF., et al. “Presacral myxopapillary ependymoma presenting as an abdominal mass in a child”. Journal of Pediatric Surgery 3 (1985): 276-278.
16. Pulitzer DR., et al. “Subcutaneous sacrococcygeal ("myxopapillary") ependymal rests”. American Journal of Surgical Pathology 9 (1988): 672-677.
17. Lemberger A., et al. “Sacrococcygeal extradural ependymoma”. Cancer5 (1989): 1156-1159.
18. Waldron JN., et al. “Spinal cord ependymomas: a retrospective analysis of 59 cases”. International Journal of Radiation Oncology, Biology, Physics2 (1993): 223-229.
19. Sonneland PR., et al. “Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases”. Cancer4 (1985): 883-893.
20. Celli P., et al. “Ependymoma of the filum terminale: treatment and prognostic factors in a series of 28 cases”. Acta Neurochirurgica 2-4 (1993): 99-103.
21. Ross DA., et al. “Myxopapillary ependymoma. Results of nucleolar organizing region staining”. Cancer 10 (1993): 3114-3118.
22. Schild SE., et al. “The results of radiotherapy for ependymomas: the Mayo Clinic experience”. International Journal of Radiation Oncology, Biology, Physics 5 (1998): 953-958.
23. Schweitzer JS and Batzdorf U. “Ependymoma of the cauda equina region: diagnosis, treatment, and outcome in 15 patients”. Neurosurgery2 (1992): 202-207.
24. Kramer GW., et al. “Subcutaneous sacrococcygeal ependymoma with inguinal lymph node metastasis”. Journal of Neurosurgery 3 (1988): 474-477.
25. Chamberlain MC. “Salvage chemotherapy for recurrent spinal cord ependymoma”. Cancer5 (2002): 997-1002.
26. Timmerman W and Bubrick MP. “Presacral and postsacral extraspinal ependymoma. Report of a case and review of the literature”. Diseases of the Colon and Rectum 2 (1984): 114-119.
27. Madden JR., et al. “Experience with tamoxifen/etoposide in the treatment of a child with myxopapillary ependymoma”. Medical and Pediatric Oncology 1 (2001): 67-69.
28. Fujiwara Y., et al. “Remarkable efficacy of temozolomide for relapsed spinal myxopapillary ependymoma with multiple recurrence and cerebrospinal dissemination: a case report and literature review”. European Spine Journal 3 (2018): 421-425.
29. Mork SJ and Loken AC. “Ependymoma: a follow-up study of 101 cases”. Cancer2 (1977): 907-915.
30. Bagley CA., et al. “Long term outcomes following surgical resection of myxopapillary ependymomas”. Neurosurgical Review 3 (2009): 321-334.