Clinical characteristics and long-term surgical outcome of spinal myxopapillary ependymoma: a French cohort of 101 patients

Ependymoma: Evaluation and Management Updates

“…Surgical resection is the mainstay of treatment for MPE, and the extent of resection is the most important prognostic factor [ 94 , 96 , 97 , 98 •]. However, due to a close adhesion to the nerve roots and production of a myxoid matrix, a safe GTR of MPE may be challenging: in fact, the rate of GTR in the literature ranges from 53 to 75% [ 98 •, 99 •].…”

“…Surgical resection is the mainstay of treatment for MPE, and the extent of resection is the most important prognostic factor [ 94 , 96 , 97 , 98 •]. However, due to a close adhesion to the nerve roots and production of a myxoid matrix, a safe GTR of MPE may be challenging: in fact, the rate of GTR in the literature ranges from 53 to 75% [ 98 •, 99 •]. A systematic review of the literature [ 100 ] found that the recurrence rate after GTR (15.5%) was significantly lower than that after STR (32.6%), while being higher in pediatric patients as compared to adults (40.5% vs 23.4%).…”

Updates in the classification of ependymal neoplasms: The 2021 WHO Classification and beyond

“…Myxopapillary ependymoma are morphologically defined tumors that predominantly arise in the most caudal part of the spinal cord. As for other spinal ependymomas, the majority of patients with MPE are adults, with a median age of 39 years [33, 34]. By far, the most common localizations of MPE are the conus medullaris and the filum terminale, but rare cases with occurrence in the brain and in the upper spinal cord or even outside the CNS have been described [6, 7] (Figure 2).…”

“…However, in around 20 % of MPE, relapses occur, either locally or at a distant site. Recurrence is associated with several clinical parameters, such as subtotal resection, tumor size, and multifocal lesions [34]. MPE might also present as disseminated disease with distant spinal or intracranial metastases, both in pediatric and adult patients [34,35].…”

“…Recurrence is associated with several clinical parameters, such as subtotal resection, tumor size, and multifocal lesions [34]. MPE might also present as disseminated disease with distant spinal or intracranial metastases, both in pediatric and adult patients [34,35]. Acknowledging this partially aggressive clinical behavior with recurrences and dissemination, the grading of MPE was changed from WHO grade I to CNS WHO grade 2 in the current version of the WHO classification.…”