ED-09TANCYTIC EPENDYMOMA: PRESENTATION OF A RARE CYSTIC DISEASE VARIANT AND REVIEW OF LITERATURE

BACKGROUND: Tanycytic ependymoma is a rare variant of ependymoma and treatment guidelines are not established. We conduct a systematic review of the literature to show management options for patients with tanycytic ependymoma. MATERIAL AND METHODS: We performed a Pubmed- and hand search to identify patients with pathologically confirmed tanycytic ependymoma. Symptoms, radiological and pathological findings as well as treatment modalities and outcomes were analyzed. RESULTS: 38 studies involving a total of 57 patients were retrieved. Most of the tanycytic ependymomas occurred in the spinal cord (45,6%), followed by lesions located in intracranial sites (40,4%) and only a few at the cervicomedullary junction (3,5%). There was no gender predelection (female to male ratio is calculated as about 1:1,3), with a mean age at diagnosis of approximately 34,9 ± 17,3 years. Surgery has been suggested offering a potential cure, but recurrence rates remain unknown. Radiation has been used after subtotal resection, but the effects of EBXRT have not been reported. The use of targeted chemotherapy has not been employed. We also present an illustrative case that was managed successfully with surgery without associated morbidity. CONCLUSION: This review on tanycytic ependymomas supports surgery as the initial treatment of choice for this rare tumor. Radiotherapy can be taken into consideration when total gross resection could not be achieved allowing prolonged progression free survival. Given the benign nature of this subtype of ependymoma, aggressive treatment modalities such as chemotherapy are usually not indicated.