Ependymomas arising outside of the central nervous system: A case series and literature review

Background Extracranial and extraspinal ependymomas are extremely rare tumors mostly published in the literature as case reports. Methods MD Anderson Cancer Center institutional database was screened for patients with extra CNS ependymomas over a 25 year period. Results Eight patients with extra CNS ependymoma were identified. Five cases originated in the sacrum or subcutaneous sacral area, and the other 3 cases originating in the breast, lung and adnexa of the uterus. By histology, most cases were myxopapillary ependymomas (5 cases), 2 cases were grade II ependymoma and 1 case was an anaplastic ependymoma. Metastases occurred in 6 cases and 3 patients died due to progressive disease. Most cases required surgery, radiation and chemotherapy. Conclusion Extra CNS ependymomas are very rare tumors that tend to metastasize, even though most cases are histologically classified as low grade ependymomas.