Extraspinal ependymomas. Report of three cases

Area of Science:

• Neurosurgery
• Oncology
• Radiology

Background:

• Spinal ependymomas rarely originate from heterotopic ependymal cell clusters, leading to extraspinal locations.
• Understanding these rare tumors is crucial for accurate diagnosis and treatment.

Observation:

• Three cases of extraspinal ependymomas were presented with a literature review.
• Tumors occurred in the third decade, presenting as sacral soft tissue masses or pelvic masses.
• Posterior masses mimic pilonidal cysts; pelvic masses cause sphincter/sacral nerve root dysfunction.

Findings:

• Radiographic findings include sacral erosion on CT scans and extradural masses indenting the thecal sac on myelography.
• Cerebrospinal fluid protein levels remain normal.
• Extraspinal ependymomas exhibit characteristic clinical and radiographic features.

Implications:

• Complete tumor removal via a combined surgical approach is the preferred treatment.
• Radiation therapy is an alternative if complete resection is not possible.
• Systemic metastases are common, with an average postoperative survival of approximately 10 years.