PMC4891574_01
Extraskeletal chordoma of the cervical spine.. A. Sagittal T2-weighted MRI in the midline shows a lobulated mass along the posterior aspect of C1-C4 with high signal. The mass does not appear to invade the vertebral bodies. The cervical cord is severely compromised. There is also incidental degenerative disc disease with loss of height at C3-C4, and mild retrolisthesis of C3 over C4.. B. Sagittal T1-weighted post-gadolinium MRI shows mild enhancement within the mass.. C. Axial short tau inversion recovery (STIR) MRI following gadolinium injection shows the large epidural mass occupying the right side of the spinal canal, compressing the cervical cord to the left. The lesion extends into the right neural foramen.[Powerpoint Slide]
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The lesion was predominantly extraskeletal and extradural, and extended along the posterior aspect of the vertebral bodies from C2 to C5 with at least 60% spinal canal compromise and severe cord compression (Fig. 1).
Extraskeletal chordoma of the cervical spine. Photomicrograph (200x, hematoxylin and eosin) - Some areas of the neoplasm revealed sheets of epithelioid cells admixed with pools of mucin. Some of the cells contain vacuolated cytoplasm. [Powerpoint Slide]
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At pathology, the lesion infiltrated bone and soft tissue and was composed of islands of epithelioid cells embedded in a prominent myxoid matrix (Fig. 2A). The lesional cells contained abundant eosinophilic cytoplasm, round to oval shaped nuclei, fine chromatin, and small, inconspicuous nucleoli (Fig. 2B). Focally, the cytoplasm of the cells was extremely vacuolated and there were occasional multivacuolated physaliferous cells (large vacuolated, glycogen or mucin-containing cells) (Fig. 2C & 2D). Immunohistochemical studies revealed the lesion to be positive for cytokeratins and S-100 protein (Fig 2E).