Myxopapillary ependymoma (WHO Grade 2)

Synonyms
myxopapillary ependymoma
ICD-10
D43.2
ICD-11
XH15U1

A myxopapillary ependymoma is a tumor that occurs almost exclusively in the spinal cord. It is a subtype of spinal Ependymomas that occurs primarily in the Filum terminale and Conus medullaris. Rarely, tumor cells may disseminate into the Cerebrospinal fluid during surgery, which can lead to intracerebral myxopapillary ependymomas[^3].

WHO classification

Since the 2021 WHO classification, myxopapillary ependymomas have been classified as WHO Grade 2[^2]. In previous WHO classifications, myxopapillary ependymomas were classified as WHO Grade 1.

Epidemiology

The mean age at initial diagnosis of a myxopapillary ependymoma is approximately 32 years[^1]. A myxopapillary ependymoma can occur in both children and adults; however, the course in the pediatric population is significantly more aggressive, with a higher recurrence rate and a higher dissemination rate along the neuraxis[^1].

Imaging

The gold standard for the diagnostic imaging of myxopapillary ependymoma is MRI imaging.

Myxopapillary ependymoma on MRI
Sagittal MRI images of a myxopapillary ependymoma. Left: T1 without contrast, center: T1 with contrast, right: T2.

Treatment

A definitive diagnosis can only be established by histopathological examination and not by imaging. Surgical treatment depends on the patients’ symptoms and complaints. To date, no benefit has been demonstrated for additional postoperative radiotherapy or chemotherapy[^1].

Prognosis

In a group of 52 patients with myxopapillary ependoma, the survival rate after 11.5 years of follow-up was 94 %[^1].

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