Multifocal intradural extramedullary anaplastic ependymoma of the spine

Ependymomas are neuroectodermal tumours arising from the ependymal lining of the ventricles and central canal of the spinal cord. Intradural extramedullary (IDEM) ependymomas which are multifocal, and/or anaplastic (WHO grade III) at presentation are exceedingly rare. We present the second case of multifocal anaplastic IDEM ependymoma in the literature. A 47-year old female presented with left gluteal and thigh pain radiating to the groin associated with paraesthesiae. She had a normal neurological examination. Magnetic resonance imaging of the lumbar spine and subsequent magnetic resonance imaging (MRI) of the remaining neuroaxis demonstrated >10 lesions throughout cervical, thoracic and lumbosacral levels. There were no intracranial lesions. The patient initially underwent surgery for removal of three symptomatic lesions at S2. She recovered well. One year later she had further surgery for three progressing lesions at T5. Four of six lesions were WHO grade III. Two smaller nodules at T5 were WHO grade II. The patient had mild sensory disturbance over the right side of the trunk which resolved postoperatively. There were no long-term sequelae. The patient subsequently underwent full craniospinal irradiation using proton beam therapy. Due to their rarity, there are no guidelines for the management of multifocal IDEM ependymoma. The only previously published case of multifocal anaplastic IDEM ependymoma by Schuurmans et al. involved surgical resection and 20 cycles of whole-spine radiotherapy. Schuurmans patient unfortunately died two years post-diagnosis with progressive cranial metastases and post-radiation myelopathy. In our case, all remaining lesions are stable and she is neurologically intact at 48-month follow up.