Intramedullary cervical chordoma

Abstract

This 52-year-old woman complained of lower trunk and limb numbness 2 months before admission. She presented with deteriorating leg weakness and urinary incontinence. Spastic quadriparesis, with impaired pinprick sensation below the T-2 dermatome, was demonstrated.Magnetic resonance (MR) imaging of the cervical spine with slight Gd enhancement revealed a diffuse intramedullary tumor extending from C-5 to T-3. Brain and lumbosacral MR images were negative. The tumor was radically resected via a posterior cervicothoracic laminectomy and midline myelotomy by the senior surgeon (C.E.G.) (Fig. 1). The gross appearance of the lesion was that of an intramedullary astrocytoma. At surgery, the tumor was easily dissected from the normal spinal cord. There was no distinct tumor capsule. The postoperative clinical course was uneventful, and the patient's neurological condition remarkably improved. She underwent rehabilitation for 3 months and has been followed for 8 months. She remains in excellent neurological condition without evidence of tumor recurrence. Histopathological examination showed a vascularized neoplasm composed of nonatypical vacuolated cells with clear cytoplasm and displaced deep-colored nuclei. Immunohistochemical analysis for S100 protein, vimentine, keratin AE1, and epithelial membrane antigen (EMA) was positive, whereas it was negative for glial fibrillary acidic protein (Fig. 2). The conclusion was that the tumor was a chordoma.Chordomas are tumors arising from the embryological remnants of the notochord. The presence of chordomas as intradural extramedullary lesions of the spine without osseous involvement is extremely rare. To the best of our knowledge, our case represents the first report of a purely intramedullary spinal chordoma. 1-4