Intradural extramedullary ependymoma: is there constantly a hormonal relationship?

BACKGROUND Ependymoma is a glial tumor that occurs in the central nervous system. The intradural extramedullary location of this neoplasm is very rare. The authors report a case of spinal intradural extramedullary ependymoma in a male and discuss its pathogenesis as well as its clinical, radiological, and therapeutical features. CASE DESCRIPTION A 31-year-old man was admitted at the author's institution. The patient has had 1-year history of cervical pain, progressive quadriplegia, and bladder disturbances. Magnetic resonance imaging revealed an enhanced cervical intradural extramedullary tumor extending from the bulbomedullary junction to the C3 level, with severe spinal cord compression. Emergency surgical resection was performed, and a total removal of the lesion was accomplished. One year and half later, a local recurrence associated to a small cerebellar lesion was noticed justifying a second spinal intervention. Both surgical interventions demonstrated an intradural extramedullary ependymoma without attachment to the spinal cord or to the dura mater. Adjuvant craniospinal radiotherapy was recommended to the patient. CONCLUSION The insufficiency of hormonal theory to explain solely the pathogenesis of this tumor might reveal other potential factors that have not been discussed in earlier literature.