Spinal Myxopapillary Ependymoma Epidemiology

Spinal Myxopapillary Ependymoma Epidemiology

The myxopapillary subtype of ependymomas (MPE) occurs mostly in the thoracolumbar region and is the most common form of ependymoma in the lumbar spine 1) 2) 3) 4).

In one study of 77 myxopapillary ependymomas 5). these tumors represented 27% of all spinal ependymomas and 90% of tumours in the conus medullaris 6) 7) 8) 9) 10) 11) 12).

Usually occurs in the adult population in the third and fourth decades of life and affect males more frequently than females 13) 14) 15).


Abdallah et al. retrospectively reviewed the medical records of 38 primary spinal myxopapillary ependymoma cases who underwent surgery at 2 neurosurgical centers spanning 16 years, from 2004 to 2019. All pediatric cases (patient age <18 years) who were diagnosed with MPE and re-presented with spinal seeding/drop metastases (SSM) were selected as the core sample for this study. Relevant literature was briefly reviewed.

Three pediatric MPE cases (2 females and 1 male) experienced SSM. The mean age at first presentation was 12.0 ± 1.0 years. The mean preoperative course was 2.9 ± 1.2 months. The predominant location was the lumbar spine in 2 tumors (both originated from filum terminale [FT]). Two tumors were located intradural intramedullary. Gross-total resection was achieved in 2 patients. No patient had neurofibromatosis type 2. No adjuvant treatment was given after the first surgery. The mean period between the first diagnosis and diagnosis of SSM was 44.0 ± 31.5 months. The location of SSM in all patients was the sacral spine (1 patient experienced distant metastasis in her brain besides her sacral metastasis). The mean follow-up was 68.3 ± 53.7 months.

They found a statistically significant relationship between SSM in pediatric MPEs and the intramedullary location, FT origin, and number of affected segments. Close clinical and radiological follow-up is essential for pediatric MPE patients. 16).

References

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Sonneland PR, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma: A clinicopathologic and immunohistochemical study of 77 cases. Cancer. 1985;56:883–93.
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Celli P, Cervoni L, Cantore G. Ependymoma of the filum terminale: Treatment and prognostic factors in a series of 2 cases. Acta Neurochir. 1993;124:99–103.
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Sonneland PR, Scheithauer BW, Onofrio BM: Myxopapillary ependymoma: A clinicopathologic and immunocytochemical study of 77 cases. Cancer 56:883–893, 1985
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Sakai Y, Matsuyama Y, Katayama Y, et al. Spinal myxopapillary ependymoma: Neurological deterioration in patients treated with surgery. Spine. 2009;34:1619–1624.
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Wippold FJ, Smirniotopoulos JG, Moran CJ, Suojanen JN, Vollmer DG. MR imaging of myxopapillary ependymoma: Findings and value to determine extent of tumour and its relation to intraspinal structures. Am J Radiol. 1995;165:1263–67.
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Bagley CA, Wilson S, Kothbauer KF, Bookland MJ, Epstein F, Jallo GI. Long term outcomes following surgical resection of myxopapillary ependymomas. Neurosurg Rev. 2009;32:321–334.
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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.
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Sun B, Wang C, Wang J, Liu A. MRI features of intramedullary spinal cord ependymomas. J Neuroimaging. 2003;13:346–351.
16)

Abdallah A. Spinal Seeding Metastasis of Myxopapillary Ependymoma: Report of Three Pediatric Patients and a Brief Literature Review [published online ahead of print, 2020 Aug 10]. Pediatr Neurosurg. 2020;1-14. doi:10.1159/000509061

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