Abstract
Sacrococcygeal myxopapillary ependymoma (MPE) is an uncommon type I glial tumor detected most frequently in the lumbosacral area of adolescents and children. It is usually presented as an intradural ependymal tumor that originates from the filum terminale and other locations within the ventricular system along the craniospinal axis. In rare cases, however, MPE may develop as a primary subcutaneous tumor in the sacrococcygeal area. Tumors can also appear as a dorsal sacrococcygeal growth or subcutaneous nodule. In this case report, we describe a rare case presenting as a subcutaneous sacrococcygeal mass in an elderly female that was subsequently resected and confirmed as subcutaneous MPE. The current standard treatment for MPE is maximal surgical resection with or without postoperative radiotherapy based on the locoregional extent and histological grading. However, there is limited evidence that radiotherapy for oligometastatic foci improves longevity or extends the time to recurrence. In addition to this case report, we provide a comprehensive review of similar cases and case series in the medical literature. Prospective studies evaluating the efficacy of resection and/or radiotherapy are required for improved management of extradural MPE.
Highlights
BackgroundEpendymomas are slowly growing glial cancers of the central nervous system (CNS) [1]
We describe a rare case presenting as a subcutaneous sacrococcygeal mass in an elderly female that was subsequently resected and confirmed as subcutaneous myxopapillary ependymoma (MPE)
Myxopapillary variants of ependymomas are rare. These myxopapillary ependymomas (MPEs) are well-circumscribed, slow-growing, grade II tumors that can appear throughout life but arise primarily in younger adults, with an average age of 36 years at the time of diagnosis [3]. They are distinguished from lower grade 2 ependymomas and malignant grade 3 ependymomas by differences in growth rate and site of origin, which in the case of MPE is most frequently in the caudal spinal cord close to the filum terminale, conus medullaris, and cauda equina
Summary
Ependymomas are slowly growing glial cancers of the central nervous system (CNS) [1]. These myxopapillary ependymomas (MPEs) are well-circumscribed, slow-growing, grade II tumors that can appear throughout life but arise primarily in younger adults, with an average age of 36 years at the time of diagnosis [3]. Extraspinal ependymomas arising in the sacrococcygeal region are observed, as either presacral or postsacral masses. While rare, these show a stronger propensity to spread systemically through lymphovascular dissemination [2,4,5]. Close monitoring has been maintained, but to date, there have been no signs of disease recurrence
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