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Presented by Edward McCarthy, M.D. and prepared by Lynette S. Nichols, M.D.
Case 3: A 60 year old man had a 8 month history of constipation and fullness in the rectal area.
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1. Question
Week 100: Case 3
A 60 year old man had a 8 month history of constipation and fullness in the rectal area. An MRI showed a 7 cm mass at the tip of the coccyx./images/Lyn’s/Mc-7-22-02.3.a.jpg
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/images/Lyn’s/Mc-7-22-02.3.e1.jpgCorrect
Answer: Chordoma
Histology: The lesion consists of cords or nests of clear, bubbly, cells in a bluish background matrix. Occasionally, the matrix may be abundant and resemble cartilage. The cells are positive for cytokeratin and S-100 protein.
Discussion: Chordoma is an epithelial neoplasm which probably arises from notochordal rests. Most cases occur at either end of the spinal canal in the sacral area or at the base of skull. Chordomas are low grade malignant tumors which are extremely difficult to surgically eradicate. The most important histologic feature of chordoma is the arrangement of the cells in nest and cords, and the positivity for cytokeratin and S-100 protein.
The lesion is not a metastatic carcinoma because of the abundant chondroid matrix. Like chordomas, metastatic carcinomas are keratin positive. However, S-100 protein is almost never present in metastatic carcinomas.
Chondrosarcomas may occur in the spine in this location. However, chondrosarcomas are not positive for cytokeratins. Therefore, lesions in the spine which have abundant chondroid matrix should be stained with keratin. Chondrosarcomas have S-100 positivity. However, unlike chordomas, the cells are arranged individually throughout the matrix rather than in clusters as in chordomas.
Incorrect
Answer: Chordoma
Histology: The lesion consists of cords or nests of clear, bubbly, cells in a bluish background matrix. Occasionally, the matrix may be abundant and resemble cartilage. The cells are positive for cytokeratin and S-100 protein.
Discussion: Chordoma is an epithelial neoplasm which probably arises from notochordal rests. Most cases occur at either end of the spinal canal in the sacral area or at the base of skull. Chordomas are low grade malignant tumors which are extremely difficult to surgically eradicate. The most important histologic feature of chordoma is the arrangement of the cells in nest and cords, and the positivity for cytokeratin and S-100 protein.
The lesion is not a metastatic carcinoma because of the abundant chondroid matrix. Like chordomas, metastatic carcinomas are keratin positive. However, S-100 protein is almost never present in metastatic carcinomas.
Chondrosarcomas may occur in the spine in this location. However, chondrosarcomas are not positive for cytokeratins. Therefore, lesions in the spine which have abundant chondroid matrix should be stained with keratin. Chondrosarcomas have S-100 positivity. However, unlike chordomas, the cells are arranged individually throughout the matrix rather than in clusters as in chordomas.
