PMC3317097_01
(a) and (b) CT shows a large heterogeneously contrast-enhanced intrasellar mass (3.6 cm in the largest diameter), with suprasellar extension, invasion of the sphenoid sinus, and erosion of the sellar floor.
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fig1
The computed tomography (CT) scan revealed a large heterogeneous contrast-enhanced intrasellar mass (largest diameter: 3.6 cm), with suprasellar extension, invasion of the sphenoid sinus, and erosion of the sellar floor (Figures 1(a) and 1(b)).
(a) Photomicrograph demonstrating the typical chordoma cells with large, pleomorphic nuclei and vacuolated cytoplasm (hematoxylin and eosin stain) forming sheets or lobular structures that are embedded in a mucoid stroma. Characteristic of chordomas chondroids are the formation of lobules of neoplastic tissue separated by fibrous stroma and areas of chondroid tissue (*), and vacuolated cell and lymphocytic infiltration. The tumor displays remarkable morphological variation, mainly based on the amount of interstitial matrix and vacuolated cells; these physaliphorous cells (arrow) with multivacuolated cytoplasm and sometimes pleomorphic nuclei are surrounded by mucinous extracellular matrix, some with chondroid aspect. Physaliphorous comes from the Greek word physalis (bubble). (b) PAS stain: the cytoplasm of many tumor cells is strongly PAS positive (arrows), highlighting them against mucoid matrix. The vacuoles, however, are usually negative. To define the nature of PAS positive material (glycogen), the technique was repeated in cuts, one of which was previously handled by diastase. (c) Neoplastic physaliphorous cells with arrows. (d) AE1/AE3: antibodies against keratins clearly show the ratio of neoplastic cells (dark brown) and interstitial tissue (in blue). The lobules of tumor tissue stand out, surrounded by strands of fibrous tissue.
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fig2
On light microscopic examination, the tumor was composed typically of a lobular pattern (Figures 2(d) and 3(b)). It histologically showed a solid pattern or cordonal of anastomosing cell cords, with neoplastic cells embedded in a myxoid intercellular matrix with chondroid differentiation (Figure 2(a)). PAS+, which is digested with diastase (Figures 2(b) and 2(c)). Physaliphorous cells contain multiple vacuoles surrounding the nucleus located in the center of the cell. Immunohistochemically, the neoplastic cells of chordoma were positive for cytokeratin AE1/AE3 (Figure 2(d)).
(a) EMA: epithelial membrane antigen positive cells of chordoma in papillary structure. Cytoplasmic pattern with enhanced cell membrane (insert). (b) S-100: strong nuclear and cytoplasmic positivity. (c) VIM. Cytoplasmic positivity. (d) KI67: positivity in a few scattered nuclei of neoplastic cells (less than 5%, low proliferation).
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fig3
The lobes are separated by a framework of fibrous septa of variable thickness, which in sections appear as septa that run by the thin-walled vessels giving aspect of papillary structures (Figure 3(a)). They were characteristically positive chordoma cells for EMA (Figure 3(a)) and the protein S-100 (Figure 3(b)) and VIMENTIN (Figure 3(c)). The proliferation index was low as evidenced by weak nuclear positivity for Ki67 (Figure 3(d)). 