Spinal myxopapillary ependymoma outcome

Spinal myxopapillary ependymoma outcome

Telomerase reverse transcriptase gene promoter (TERTp) mutation has been identified in a subset of ependymomas with aggressive behavior 1).

Despite its benign biological nature, myxopapillary ependymoma (MPE) has a propensity to recur locally or distantly. Although variables influencing the prognosis, such as age, the extent of resection 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.

Treatment failure of MPE occurred in approximately one-third of patients. The observed recurrence pattern of primary spinal MPE was mainly local, but a substantial number of patients failed nonlocally. Younger patients and those not treated initially with adjuvant RT or not undergoing gross total resection were significantly more likely to present with tumor recurrence/progression 2).

The 5-year survival rate of spinal ependymomas ranges from 57–100% 3) 4) 5) and 10–33% of patients will experience local invasion of the tumour or recurrence 6) 7).

Metastasis is rare in MPE but there have been several reported cases 8) 9) 10) 11) 12) 13) 14) 15) 16) 17) 18) 19) 20).



Deniel A, Marguet F, Beaussire L, Tobenas-Dujardin AC, Peillon C, Gambirasio MA, Veresezan O, Magne N, Di Fiore F, Laquerrière A, Sarafan-Vasseur N, Fontanilles M. TERTp mutation detection in plasma by droplet-digital PCR in spinal myxopapillary ependymoma with lung metastases. World Neurosurg. 2019 Jul 19. pii: S1878-8750(19)32017-0. doi: 10.1016/j.wneu.2019.07.111. [Epub ahead of print] PubMed PMID: 31330336.

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. 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 Apr;17(4):588-95. doi: 10.1093/neuonc/nou293. Epub 2014 Oct 9. PubMed PMID: 25301811; PubMed Central PMCID: PMC4483075.

Volpp PB, Han K, Kagan AR, Tome M. Outcomes in treatment for intradural spinal cord ependymomas. Int J Radiation Oncology Biol Phys. 2007;69:1199–1204.
4) , 7)

Hanbali F, Fourney DR, Marmor E, et al. Spinal cord ependymoma: Radical surgical resection and outcome. Neurosurgery. 2002;51:1162–74.

Asazuma T, Toyama Y, Suzuki N, et al. Ependymomas of the spinal cord and cauda equine: An analysis of 26 cases and a review of the literature. Spinal Cord. 1999;37:753–59.

Bavbek M, Altinors MN, Caner HH, Bilezikci B, Agildere M. Lumbar myxopapillary ependymoma mimicking neurofibroma. Spinal Cord. 2001;39:449–452.

Friedman DP, Hollander MD. Neuroradiology case of the day. Radiographics. 1998;18:794–98. [PubMed] 17. Patterson RH, Jr, Campbell WG, Jr, Parsons H. Ependymoma of the cauda equina with multiple visceral metastases. J Neurosurg. 1961;18:145–150.

Agapitos E, Kavantzas N, Karaitianos J, Davaris P. Subcutaneous sacrococcygeal myxopapillary ependymoma: a case report. Archives d’anatomie et de cytologie pathologiques. 1995;43:157–159.

Al Moutaery K, Aabed MY, Ojeda VJ. Cerebral and spinal cord myxopapillary ependymomas: a case report. Pathology. 1996;28:373–376.

Helwig EB, Stern JB. Subcutaneous sacrococcygeal myxopapillary ependymoma: a clinicopathologic study of 32 cases. Am J Clin Pathol. 1994;81:156–161.

Ilhan I, Berberoglu S, Kutluay L, Maden HA. Subcutaneous sacrococcygeal myxopapillary ependymoma. Medical and Pediatric Oncology. 1998;30:81–84.

Kline MJ, Kays DW, Rojiani AM. Extradural myxopapillary ependymoma: report of two cases and review of the literature. Pediatric Pathology and Laboratory Medicine. 1996;6:813–822.

Kramer GW, Rutten E, Sloof J. Subcutaneous sacrococcygeal ependymoma with inguinal lymph node metastasis. J Neurosurg. 1988;68:474–477.

Pulitzer DR, Martin PC, Collins PC, et al. Subcutaneous sacrococcygeal (“myxopapillary”) ependymal rests. Am J Surgical Pathology. 1988;12:672–677.

Woesler B, Moskopp D, Kuchelmeister K, et al. Intracranial metastasis of a spinal myxopapillary ependymoma. A case report. Neurosurgery Review. 1998;21:62–65.

Graf M, Blaeker H, Otto HF. Extraneural metastasizing ependymoma of the spinal cord. Pathology Oncology Research. 1999;5:56–60.

Mavroudis C, Townsend JJ, Wilson CB. A metastasizing ependymoma of the cauda equine: case report. J Neurosurg. 1977;47:771–775.

Rubinstein LJ, Logan WJ. Extraneural metastases in ependymoma of the cauda equina. J Neurol Neurosurg Psychiatry. 1970;33:763–770.

Rickert CH, Kedziora O, Gullotta F. Ependymoma of the cauda equine. Acta Neurochir. 1999;141:781–2.

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