Chordomas are midline tumors of notochordal origin, occurring anywhere from the skull base to the coccyx. Although one-third of chordomas occur in the spheno-occipital region, to our knowledge only 1 case of jugular foramen chordoma with unusual extension into the neck has been reported in the literature to date. A 21-year-old woman presented with a 3-year history of a large neck mass and partly compensated lower cranial nerve symptoms of insidious onset. Imaging revealed a tumor involving the posterior cranial fossa and carotid space, with widening and erosion of the jugular foramen. Characteristic histopathologic findings and immunohistochemical staining confirmed the diagnosis. The tumor was removed by a combined retrosigmoid and lateral cervical approach. The patient was disease free 18 months after treatment.
Axial computed tomogram showing tumor encroaching in the right carotid space, with erosion of the transverse process and lamina of the axis.
T1-weighted magnetic resonance images. A, Coronal T1-weighted image showing lobulated tumor with widening of right jugular foramen and posterior fossa extension. B, Sagittal postcontrast fat suppression T1-weighted image showing variegated enhancement with nonenahancing areas of necrosis and anterior displacement of the internal carotid artery.
Microscopic features. A, Histologic features showing a moderately cellular tumor composed of clusters and cords of polygonal cells within a prominent myxoid matrix. The cells show minimally to mildly atypical nuclei and a moderate to abundant amount of cytoplasm that is frequently vacuolated (arrow) (physaliferous cells) (hematoxylin-eosin, original magnification ×200). B, Immunohistochemical staining shows diffuse nuclear expression of S-100 protein within the cords and clusters of cells, supporting the diagnosis of chordoma (original magnification ×40).
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