Abstract
IntroductionEpendymomas are rare glial tumors of the brain representing less than 5% of brain tumors. However, spinal cord ependymomas in adults account for over 60% of all ependymomas including those arising from the filum terminale and only 40% are intracranial. Reports of the appearance of another neoplasia at a different location in patients with spinal ependymoma are scarce.MethodsWe searched PubMed for studies related to spinal cord ependymomas published over the last 30 years (from January 1984) and retrieved 1197.ResultsWe identified only two studies that met our criteria and we found an incidence of 9% of secondary neoplasias after treatment for spinal ependymoma. The neoplasms were diagnosed from 2 months to 20 years after patients underwent surgery for intraspinal ependymoma. These included pancreatic cancer, prostate cancer, Hodgkin lymphoma, intracranial meningioma, mucin-producing pulmonary adenocarcinoma, gastric cancer and astrocytoma.ConclusionsThe genetic abnormalities affecting patients with spinal ependymomas may indicate a predisposition to the development of secondary cancers or a general failure of the repairing mechanism in their DNA. The unaffected survival rates in those individuals permit for a long period the accumulation of different mutations on the genome and thus the appearance of a second cancer. However, more studies are needed, particularly in young patients with high survival rates.
Highlights
Ependymomas are rare glial tumors of the brain representing less than 5% of brain tumors [1,2,3]
According to the World Health Organization (WHO) classification, ependymomas are divided into three subtypes: subependymomas and myxopapillary ependymomas (MEPNs; WHO grade I), classic ependymomas (WHO grade II), and anaplastic ependymomas (WHO grade III)
Secondary cancer in this review study was found in nine patients out of 100 who underwent surgery for spinal ependymoma, suggesting that 9% of patients might develop a de novo tumor
Summary
Ependymomas are rare glial tumors of the brain representing less than 5% of brain tumors. Reports of the appearance of another neoplasia at a different location in patients with spinal ependymoma are scarce. Spinal ependymomas have a better prognosis [4] and gross total resection (GTR) is the gold standard in surgical treatment [5-9]. They must be considered a unique clinical entity requiring specific management. MEPNs represent 13% of all ependymomas and are mainly found in the cauda equina, with occasional extension into the conus medullaris [10]. The remaining spinal cord ependymomas generally consist of the classic ependymoma type, which are primarily located in the cervical and thoracic regions [11]
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