Ependymomas arising outside of the central nervous system: A case series and literature review. (January 2018)

• Record Type: Journal Article
• Authors: Yust Katz, Shlomit; Cachia, David; Kamiya-Matsuoka, Carlos; Olar, Adriana; Theeler, Brett; Penas Prado, Marta; Gilbert, Mark R.; Armstrong, Terri
• Is Part Of: Journal of clinical neuroscience. Volume 47(2018)
• Page Start: 202
• Page End: 207
• Publication Date: 2018-01
• Subjects: Ependymoma -- Extra-CNS -- Metastases; Brain -- Surgery -- Periodicals; Neurosciences -- Periodicals; Nervous system -- Surgery -- Periodicals; Brain -- surgery -- Periodicals; Neurosurgical Procedures -- Periodicals; Neurosciences -- Periodicals; Electronic journals; 616.8
• DOI: 10.1016/j.jocn.2017.10.026
• Languages: English
• ISSNs: 0967-5868

Abstract

Highlights: Extra CNS ependymomas are rare tumors, here we present eight patients with extra CNS ependymoma. 5 cases originated in the sacrum or sacral area, and the rest in the breast, lung and the uterus. By histology, most cases were myxopapillary ependymomas (5 cases). Metastases occurred in 6 cases and 3 patients died due to progressive disease. Most cases required surgery, radiation and chemotherapy. Abstract: 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.