We present a case of cranial chordoma that recurred 8 years after radical removal of a nasopharyngeal tumor. This tumor consisted of three parts: typical physaliphorous cells, a fibrosarcomatous component and a chondrosarcomatous component. Chest X-ray showed multiple lung metastases 3 months after the partial removal of the recurrent tumor. Radiotherapy and combination chemotherapy (ADM, MMC, HCFU and OK-432) were not beneficial. The patient finally died of progressive metastatic disease. At autopsy, 9 years after diagnosis, only the fibrosarcomatous component was found in the pulmonary and vertebral metastases, and typical chordoma tissue was found in the cranial recurrent tumor. Intracranial invasion of the recurrent tumor was not observed.
