Pediatric spinal intradural extramedullary tumors

Abstract

Intradural spinal cord tumors are uncommon childhood tumors, and most series are comprised mainly of adult patients [1, 2]. Intradural extramedullary spinal tumors are estimated to represent less than one half of all intradural tumors in childhood [3-5]. Precise incidence figures are hard to come by for children. The main diagnostic considerations (Table 51.1) are schwan-noma, neurofibroma, and metastatic spread from an undiagnosed intracranial tumor or primary metastatic disease, particularly PNET. In children, low-grade tumors, such as low-grade astrocytomas and ganglio-gliomas, can also disseminate in CSF pathways, and spinal dissemination appearing as extramedullary disease can very rarely be the presentation of primary intracranial tumors. Meningiomas may also occur, but the incidence is exceedingly rare compared to adults. Developmental lesions, such as lipomas and epider-moid/dermoid tumors, are an important additional consideration as well, but clinical clues and distinctive features on MRI imaging usually allow for a preopera-tive diagnosis. Neurenteric cysts, also very rare lesions, may be intramedullary or extramedullary and can be diagnosed by identification of particular imaging features. In the cauda equina region, myxopapillary ependymomas and the rare paraganglioma are particular considerations, but these tumors are also much more common in adults. Most intradural extramedul-lary tumors are benign and resectable, so prognosis is often excellent (Fig. 51.1). © 2010 Springer-Verlag Berlin Heidelberg.