Primary Intramedullary Spinal Cord Astroblastoma with EWSR1/BEND2 Fusion: A Case Report and Literature Review

Abstract

with total surgical resection. CaseA 45-year-old female with no significant background medical history presented to the outpatients department of our institution with a six month history of progressive myelopathy. Her symptoms began with tingling in the fingers bilaterally, which slowly progressed to reduced dexterity and clumsiness. On examination, tone and power were intact in the muscle groups of all four limbs. She was hyper-reflexic throughout, with bilateral Hoffmann's sign, Babinski's sign, and clonus. Sensation was reduced in the fingers and the feet bilaterally and not in any particular dermatomal distribution, but proprioception was preserved. She had difficulty finger tapping. Her gait was normal.MRI imaging of her cervical spine (Figure 1) revealed an intramedullary lesion from C4 to C6, which was relatively well demarcated post-Gadolinium enhancement, with the radiological differential diagnosis including an ependymoma or astrocytoma. There was also significant associated oedema within the cervical spinal cord. Given the imaging findings and clinical progression, surgery was performed.A posterior approach was taken and C3 to C6 laminectomies performed. The spinal cord was visibly under pressure following durotomy. Intraoperative neurophysiological monitoring was employed and a midline myelotomy was performed with the assistance of dorsal column mapping. A soft pale grey and tan lesion was encountered which was relatively avascular.