Multicentric extramedullary myxopapillary ependymomas: Two case reports and literature review

Ependymoma has been described typically as an intramedullary tumor derived from ependymal cells. Intradural extramedullary presentation is rarely described and almost always as a unique lesion. Myxopapillary ependymoma is a histological variant that distinguishes from the ordinary type of ependymoma because of its generally better prognosis. We present two cases of multicentric extramedullary myxopapillary ependymomas.

Case 1 was a 30-year-old man with progressive paresthesia and paresis in the lower limbs, urinary sphincter disturbances, gait instability, ataxia, and chronic low back pain with multiple intradural extramedullary lesions at C2-C3, D2-D4-D5, and D12-L1. Case 2 was a 32-year-old man, presented with low back pain and mild paresthesia in the right lower limb. Magnetic resonance imaging (MRI) showed multiple intradural extramedullary lesions with homogeneous enhancement after gadolinium injection at C7, D2, D4, D5, D8, D10, D11, L1, L3, L5, S1, and S2. Complete tumor resection of the approached tumors was archived in both cases. Histological studies confirm myxopapillary ependymomas. Patient's neurologic outcome was good and no residual tumor was present at MRI control at 10 years in case 1 and 12 months in case 2.

We report the first two cases of multicentric extramedullary myxopapillary ependymomas, this etiology must be taken into account in the differential diagnosis of intradural extramedullary tumors.