Multifocal intradural extramedullary myxopapillary ependymoma of the spine

Abstract Background Myxopapillary ependymoma (MPE) is an uncommon spinal ependymal tumor that usually arises as a solitary intradural extramedullary (IDEM) lesion in the conus medullaris, cauda equina, or filum terminale. MPE is designated WHO grade 2 since the 2021. Synchronous multifocal spinal MPE at initial presentation is rare and creates uncertainty regarding the extent and timing of surgery. Case presentation A 34-year-old man with no significant medical history presented with sudden severe non-radicular low-back pain and no neurological deficit. Contrast-enhanced MRI of the brain and entire spine showed multiple enhancing IDEM lesions confined to the lumbosacral region (T12–L1, L2, L3–L4, L4–L5, and S1). The dominant right-sided T12–L1 lesion markedly compressed the conus medullaris/proximal cauda equina. Complete microsurgical resection of this lesion was performed, and histopathology confirmed MPE, WHO grade 2 (Ki-67 ≤ 2–3%). The patient declined radiation-oncology referral, and the remaining lesions were monitored with MRI and CSF cytology, which remained negative. Nine months later, he developed paresthesias and recurrent low-back pain, with enlargement of the L3–L4 and L4–L5 lesions. Both were completely resected in a second operation, while the stable L2 and S1 lesions were left in place. No new neurological deficit occurred after either operation. The patient subsequently underwent radiation-oncology evaluation and received radiotherapy, and the residual lesions were unchanged at 3 months. Conclusion Multifocal spinal MPE may require individualized, staged management. Prioritizing symptomatic or compressive lesions can achieve decompression and tissue diagnosis while limiting multilevel surgical morbidity. Residual disease warrants long-term imaging surveillance, CSF assessment, and evaluation for radiotherapy.