PMC5613465_01
Technetium bone, magnetic resonance imaging (MRI), and computed tomography (CT) scans. (a) Technetium bone scan was useful in localizing the lesion (arrow) and no metastatic lesion was found. (b, c) The MRI scan showed a lesion of increased signal intensity on T2-weighted images in the C5 region, and the tumor is protruding into the spinal canal from the left side of the C5 vertebral body. (d) The CT scan shows the osteolytic lesion in the C5 vertebral body.
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The MRI scan showed an area of increased signal intensity on T2-weighted images in the C5 region (Figure 1(b)). The neurological symptoms were ascribed to the compression of the spinal cord and nerve roots by an expanding mass partly protruding into the vertebral canal (Figures 1(c) and 1(d)). The metastatic lesion was found on neither the technetium bone scan (Figure 1(a)) nor the positron emission tomography.
Roentgenogram after surgery. (a) Lateral radiograph showing posterior cervical interbody fusion with the artificial vertebral body of C3 to T1 after the first surgery. (b, c) Sagittal (b) and axial MRI scan (c) at 6 years after the initial surgery with a third recurrence of the tumor. (d) Lateral radiograph of the cervical spine, and sagittal (e) and axial views (f) of the MRI image, showing no recurrence at 8 years 4 months after the first surgery.
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Complete intralesional resection of the tumor was performed by piecemeal, not en bloc resection, followed by spine column reconstruction by using a titanium mesh cage (Figure 2(a)). Regarding the postoperative course, the patient underwent three additional spinal surgeries, because local recurrences of the initial lesion were found at 9 months, 4 years and 2 months, and 6 years after the first surgery (Figures 2(b) and 2(c)). These recurring tumors were treated with en bloc resection through the anterior approach, followed by replacement of the titanium mesh cage (Figure 2(d)). At 8 years and 4 months after the first surgery, the patient was ambulatory without lower and upper limb paralysis and demonstrated no neurological symptoms without recurrence (Figures 2(e) and 2(f)).
Histological and immunohistochemical features of the tumor. (a) Hematoxylin and eosin stain showing pathognomonic physaliphorous cells with multivacuolated cytoplasm. The appearance of chordoma consists of cords and islands of tumor cells arrayed in a distinctly lobular fashion, with mucus-filled cytoplasm. (b, c, d) The tumor cells demonstrating positive immunostaining with AE1/AE3 (b), epithelial membrane antigen (EMA) (c), and KI-67 expression (d). Scale bar of all the panels: 100 mum.
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On pathological examination, the cytoplasm of the tumor cells was acidophil with considerable nuclear pleomorphism (Figure 3(a)). It was lobulated and composed of an abundant myxoid stroma, with distributed nests, sheets, and cords of univacuolated, multivacuolated (physaliphorous), and glandular cells that showed almost some degree of nuclear atypia (Figure 3(a)). Immunohistochemical analysis revealed that the tumor cells were positive for cytokeratin AE1/AE3 (Figure 3(b)). Epithelial membrane antigen (EMA: Figure 3(c)) and S-100 protein indicated a notochordal cell origin. The ratio of the MIB-1 index (Ki-67-positive cells) within the tumor was high (19.0%, Figure 3(d)).