The nature of double concomitant myxopapillary ependymoma: report of a case

Abstract

Myxopapillary ependymomas are almost exclusively seen at the conus medullaris/filum terminale/cauda equina region, usually as solitary space-occupying lesions. The authors report the case of a 14-year-old boy with double concomitant myxopapillary ependymoma, proximal and caudal on the filum terminale in which a totally gross removal was achieved in two stages. This presentation is rare and, so far, we have known just three similar cases that were previously reported in children. The true nature of these lesions is controversial, and while some argue that they are related to metastatic seeding, others consider them independent lesions developing synchronously. A review on dissemination of spinal myxopapillary ependymomas was done.

Concomitant Double Tumors of Myxopapillary Ependymoma Presented at Cauda Equina-Filum Terminale in Adult Patient

“…MPEs are World Health Organization grade I (low-grade) ependymomas originating mostly from the filum terminale or the terminal ventricle 5 14 19) . For this reason MPEs are detected as solitary tumors more commonly at conus-cauda-filum levels 5 19 21) . Intracranial, cervical and thoracic region lesions are considered as CSF dissemination of MPEs, yet reverse situations have also been reported 21 25) .…”

“…For this reason MPEs are detected as solitary tumors more commonly at conus-cauda-filum levels 5 19 21) . Intracranial, cervical and thoracic region lesions are considered as CSF dissemination of MPEs, yet reverse situations have also been reported 21 25) . Tumor dissemination is basically caused by violation of tumor capsule of MPEs caused by piecemeal tumor removal, traumatic spinal tap, spinal trauma, spinal instrumentation, and tumor hemorrhage 4 11 15 18 20 21 23 27) .…”

“…MPE is composed of cellular areas harboring rosettes and pseudorosettes intermingled with papillary regions, which have vascular cores in mucoid matrix 14 19 21) . MPEs sometimes coexist with spinal dysraphisms 1 7 24 26) .…”

“…MPEs presenting as concomitant double tumors at conuscauda-filum level are very rare. This kind of presentation could not be directly considered as dissemination, since both tumors are in the site of classical origin of MPEs 21) . Ten cases have been presented up to date ( Table 1 ) 2 8 9 10 16 17 21) .…”

“…Concomitant double tumor presentation of MPE at conuscauda-filum level is very rare. To date, 10 cases have been presented 2 8 9 10 16 17 21) . Herein, we report a new case of MPE presented as double tumor in noncontiguous segments of lumbosacral spinal column.…”