Uterine Cervix Metastasis of Myxopapillary Ependymoma Originated from the Spinal Cord

Abstract

Background: Myxopapillary ependymomas are well differentiated low-grade tumors which have been documented to local or distant metastasis. In the literature, this is a unique case of myxopapillary ependymoma with metastasis to the uterine cervix. Here, we present a rare case of extra neural metastasis of spinal ependymoma that developed over a long period.

Case report: A 34-year-old woman was referred to our hospital for pelvic mass. A mass (110×100 mm) localized between the sacrococcygeal region and the uterus was detected by magnetic resonance imaging. In 2004, she had been operated upon for myxopapillary ependymoma seated in the sacrococcygeal region for the first time. She underwent tumor resection eight times due to the recurrence of spinal tumor in the same region in nine years. Under the diagnosis of uterine neoplasm, we carried out radical hysterectomy, omentectomy and pelvic lymphadenectomy as the surgical procedure. The pathological findings were reported as myxopapillary ependymoma. Immunohistochemically, the myxopapillary ependymal cells showed strong positivity for glial fibrillary acidic protein, whereas they were negative for low molecular weight cytokeratin. The Ki-67 labeling index was about 2-3%. The patient had an uneventful postoperative period. She has remained free of symptoms in the year since surgery.

Conclusion: Extra-spinal myxopapillary ependymoma is very rare, but it must be considered in the differential diagnosis of pelvic mass lesions.

Keywords: Ependymoma; spinal cord tumor; uterine cervix.

FIG. 1.

a, b. The histopathological images belonging to a previous sacral tumor. The myxoid area positioned between small vessels, around which small tumor cells aggregates are shown (H&E×4) (a). The tumor cells are positive for glial fibrillary acidic protein (GFAP×20) (b). MRI images for the case (c).

FIG. 2.

a–d. The sagittal T2-weighted image of the pelvis shows a mass near the coccyx (a), the coronal T2-weighted image reveals a multi-lobulated mass in the pelvic area (b). The axial contrast enhanced image shows a huge mass extending to pelvic area from the sacral region (c). The sagittal T2-weighted image demonstrated marked tumoral involvement of sacrococcygeal region (d).

FIG. 3.

a, b. Photograph of the sectioned gross specimen showing a dark red to brown lobulated tumor seated in the uterine cervix with uterus and adnexa (a). A micrograph of current MPE. Ependymomas are composed of cells with regular, round to oval nuclei with a variably dense fibrillary background (×200) (b).