Primary intradural extramedullary anaplastic ependymoma of the cauda equina: a rare and challenging case
Ivo Kehayov1, Atanas Davarski1, Daniel Markov2
1Department of Neurosurgery, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria.
Abstract:
Intradural extramedullary anaplastic ependymomas in the cauda equina are extremely rare. The latent period from the beginning of symptoms until diagnosis is notably shorter compared to other ependymoma subtypes. In some cases, severe neurological deficit may develop rapidly due to intratumoral hemorrhage. We present the case of a 36-year-old male who experienced sudden numbness in his legs, followed by an inability to move his feet. In the following days, independent walking became impossible, and he stopped urinating. At the time of admission, bilateral hypesthesia was observed across the area of L4, L5, and sacral nerve root distribution, along with clinical signs indicative of cauda equina syndrome. Enhanced magnetic resonance imaging showed the presence of an intradural extramedullary mass occupying the spinal canal in the L3–S1 segment. Intraoperatively, an intradural tumor was identified between the roots of the cauda equina, with signs of intratumoral hemorrhage. The tumor was dissected free from the uninvolved surrounding nerve roots using a microsurgical technique. Following the sectioning of the filum terminale at the tumor attachment site, total tumor removal was achieved. In the postoperative period, a reduction of preoperative symptoms was noted. The patient underwent postoperative radiotherapy. The two-year follow-up demonstrated mild hypesthesia in the L5 and S1 dermatomes, reduction of the weakened plantar and dorsiflexion of the feet, and persistent bladder control impairment. Postoperative MRI revealed no evidence of recurrence. Anaplastic ependymomas require adjuvant radiation therapy, even after total resection and systemic neuroimaging screening for recurrence or metastases. Early detection and operative treatment aiming at gross-total removal provide patients with an improved quality of life and prolonged survival.

