Lumbosacral ependymomas: a review of the management of intradural and extradural tumors

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>The goal of this study was to review the management of intra- and extradural ependymomas. Spinal ependymomas most commonly occur as intramedullary tumors throughout the spinal axis. In the lumbosacral region, ependymomas are most commonly associated with the conus medullaris and cauda equina, but can also occur extradurally in the sacrum, presacral tissues, or subcutaneous tissues over the sacrum. These two tumor locations produce different management concerns. Intradural ependymomas, especially those in the lumbosacral region, are now recognized for their potential to spread throughout the central nervous system (CNS), whereas extradural tumors elicit more concern for their association with extraneural metastases.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>The authors have reviewed the literature regarding both of these distinct tumors and have summarized recommendations for the management of intra- and extradural lumbosacral ependymomas. For both tumors, it appears that gross-total resection is the treatment of choice when feasible. The role of radiation therapy has not been adequately studied for either tumor location, but most clinicians use this modality in patients with subtotal resection of intradural ependymomas, local recurrence, or CNS dissemination. Data supporting the use of radiation therapy for extradural ependymomas are lacking. There does not appear to be a significant role for chemotherapy in either tumor location.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Despite the risk for local recurrence and CNS dissemination, the prognosis for intradural lumbosacral ependymomas is good, with a greater than 90% 10-year patient survival in most series. The prognosis for extradural ependymomas does not appear to be as good. Much depends on extradural tumor location, however; the outlook is better for dorsal sacral tumors than presacral tumors.</jats:p></jats:sec>

A primary malignant ependymoma of the abdominal cavity: a case report and review of the literature

“…Primary extraneural ependymomas may originate from remnant embryonic neuroectodermal cells due to incomplete regression [5]. Accordingly, most extraneural ependymomas were found in or near structures of the neural axis, i.e., mediastinal, coccygeal locations or in the filum terminale, where ependymal rests can be found after birth [5]. Other discussed mechanisms include upfront unidirectional teratomas [6], remnants of neural tissue following the involution of the other two germ-layer components of a teratoma [6] or the incomplete closure of the neural arch [7], which, similar to the findings in the ovary, would allow for the development of a neometaplasia of heterotopic Muellerian duct-derived tissue [8] or a heterotopic monodermal tumor [9] outside the central nervous system.…”

“…Primary extraneural ependymomas are very rare, and to date, only isolated cases have been documented [3,4]. Primary extraneural ependymomas may originate from remnant embryonic neuroectodermal cells due to incomplete regression [5]. Accordingly, most extraneural ependymomas were found in or near structures of the neural axis, i.e., mediastinal, coccygeal locations or in the filum terminale, where ependymal rests can be found after birth [5].…”

Mutation analysis of the motor neuron and pancreas homeobox 1 (MNX1, former HLXB9) gene in Swedish patients with Currarino syndrome

“…All patients with anal stenosis, with or without funnel anus, have an MRI to exclude presacral tumors. Family members of index cases should also be considered for evaluation [21].…”

“…At least in adults, the prognosis of extradural tumors appears to be less favorable than that of intradural lesions. Furthermore, the outcome of presacral tumors is worse than that of dorsal sacral tumors [21].…”

Functional Reconstruction of the Pelvic Ring With Simultaneous Bilateral Free Fibular Flaps Following Total Sacral Resection

“…Chemotherapy, however, has no role in the therapy for this tumor. 10 Tumors of the lumbosacral area require resection of vital skeletal portions that are key to structural integrity for weight bearing. It requires both an anterior (intraperitoneal) and posterior approach.…”