The sacrococcygeal chordoma is a rare tumor which is generally believed to arise from remnants of embryonic notochordal tissues. These may persist as the nucleus pulposus or in scattered nodular rests in some 2% of humans.1 A recent review of the literature revealed a total of 505 cases of chordoma.2 Chordomas show a predilection for the sacrum and coccyx, and about half are located in these areas. Although chordomas are malignant tumors in every sense and are locally invasive, proved metastases are very uncommon. Apparently, metastases from mucus-producing carcinomas of the rectum have in some cases been confused with metastatic chordoma. We are aware of only one case of presumable distant bony metastasis of a chordoma, but this was not proved by histologic examination.3
The primary therapeutic approach to sacrococcygeal chordomas is surgical. Block removal of the coccyx, most of the sacrum, and the tumor is recommended
McSWEENEY AJ, SHOLL PR. Metastatic Chordoma: Use of Mechlorethamine (Nitrogen Mustard) in Chordoma Therapy. AMA Arch Surg. 1959;79(1):152–155. doi:10.1001/archsurg.1959.04320070156027
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