Surgical management of a rare myxopapillary ependymoma of the gluteal region: A case report

Abstract

Background: Ependymomas are rare tumors originating from neuroepithelial cells lining the wall of the ventricles or central canal of the spinal cord. While these tumors mainly occur within the central nervous system (CNS), there are occasional reports in children and young adult patients with a primary tumor occurrence outside of the CNS. Ependymomas of the sacrococcygeal region have been infrequently described in the literature with no standard of care established. We present a case report and review of the literature regarding this rare entity.

Case description: A 24-year-old woman presented with right gluteal pain worsened by sitting and a palpable soft tissue mass of the sacrococcygeal region. Magnetic resonance imaging revealed a 3.7 cm cystic mass centered in the right gluteal region. She underwent a biopsy at an outside institution, with histology revealing myxopapillary ependymoma. The patient was referred to our hospital and underwent an interdisciplinary neurosurgical and orthopedic oncology en bloc resection of the ependymoma, which intraoperatively appeared to originate from the coccygeal nerve.

Conclusion: In the present report, the authors demonstrate that a myxopapillary ependymoma may present as an isolated gluteal mass attached to the coccygeal nerve, without frank CNS involvement. Furthermore, an interdisciplinary approach to surgical resection of this lesion appears to represent an effective treatment modality.

Keywords: Extra central nervous system ependymoma; Extraneural ependymoma; Gluteal ependymoma; Rare ependymoma case; Rare myxopapillary ependymoma.

Figure 1:

Preoperative magnetic resonance imaging (MRI) imaging. MRI of pelvis demonstrating soft tissue mass centered within the right hemigluteal region. Sagittal T2-weighted image (a) and axial T1-weighted postcontrast image (b) is shown with arrowheads indicating location of the mass.

Figure 2:

Myxopapillary ependymoma. The tumor is composed of many papillary structures formed by vessels (arrow) encircled by basophilic myxoid material (asterisk) and collars of cuboidal tumor cells (arrowhead) (a) which demonstrate strong GFAP positivity (b).

Figure 3:

Postoperative magnetic resonance imaging (MRI) imaging. MRI of pelvis taken 6 months postoperative demonstrating no evidence of tumor recurrence or metastatic disease to the pelvis. Sagittal T1-weighted postcontrast image (a) and axial T1-weighted postcontrast image (b) shown above.