A rare case of an intramedullary metastasis of a myxopapillary ependymoma

Abstract

Background: Myxopapillary ependimoma (MPE) is a benign slow-growing tumor, and it has been designated histologically as a Grade I neoplasm according to the 2016 World Health Organization classification. Despite the benign character, dissemination and metastasis have occasionally been reported. The retrograde dissemination to other levels of the neuraxis is extremely rare, being more frequent to the intracranial compartment. Case Description: We hereby present a case of medullary metastasis of cauda equina MPE, with a history of having undergone a subtotal resection and postoperative adjuvant radiotherapy. The patient presents complaints of night dorsal pain attributable to intradural metastasis twenty-one years after the first surgical intervention. Conclusion: The case reported highlights the importance of long follow-up in patients with MPE, since the possibility of secondary seeding to distant craniospinal sites or local spinal sites after surgery, and radiotherapy should be considered in metastatic disease.

Myxopapillary Ependymoma of the III Ventricle: Example of a Histological Variant of Ependymoma with Exceptional Localization

“…Thus, the negativity for epithelial markers (CAM5.2 and cytokeratins AE1-AE3) and the positivity for GFAP allowed us to exclude the possibility of a metastatic carcinoma. Similarly, the radiological examinations allowed us to rule out the possibility of dissemination of an intra-axial tumor through the cerebrospinal fluid [11] [12]. These previously described facts allow us to confirm the myxopapillary character of the tumor, and its intraventricular primary origin (third ventricle), which makes it different from other cerebral ependymal neoplasms recently described, which either presented different histological pattern or their location was different from the one observed in our case [13] [14] [15].…”

An Overview of Central Nervous System Tumours

“…Myxopapillary ependymomas (MPEs) are slow-growing ependymomas that are usually considered to be benign, low-grade tumours (grade I) [81]. MPEs are a variant type of ependymoma that occurs mainly in the filum terminale or conus medullaris [81]. Upon macroscopic analysis, cells are found to be encapsulated, lobulated, and sausage or oval shaped.…”