Multifocal Spinal Intradural Myxopapillary Ependymoma.

Abstract

Ependymal tumors constitute approximately 1.8% of all primary central nervous system (CNS) tumors. Myxopapillary ependymomas (MPEs), a subtype of ependymoma, account for nearly 15% of spinal ependymoma cases. Multifocal presentation of MPEs at the initial diagnosis in adults is exceedingly rare, with only 12 such cases reported in the literature between 2011 and 2024. Despite generally favorable prognoses, MPEs are associated with a high risk of recurrence, emphasizing the need for long-term surveillance. We report a case of a 21-year-old male presenting with lower back pain and right-sided foot drop. MRI revealed multiple intradural extramedullary lesions at D12-L3, L4-L5, and S1-S2 levels. Gross total resection (GTR) was performed, and histopathology confirmed the diagnosis of myxopapillary ependymoma. Postoperative recovery included significant neurological improvement. However, 6 months post-surgery, the patient developed a new lesion at the D3 level along with metastases at D12 and S2, prompting the initiation of whole-spine irradiation. Myxopapillary ependymomas are rare WHO grade II spinal tumors with a tendency for multifocality and recurrence. GTR remains the cornerstone of treatment for optimal outcomes. In cases where GTR is not feasible, adjuvant radiotherapy is recommended. Ongoing studies are warranted to better determine the indications for craniospinal irradiation versus localized radiotherapy in such cases. The online version contains supplementary material available at 10.1007/s43465-025-01467-6.