CRANIOCERVICAL CHORDOMAS

In a 20 year period seven cases of craniocervical chordoma were seen and treated. Since it is not possible to completely resect these lesions, the aim of treatment has been to debulk the tumor as much as feasible and employ postoperative irradiation. Palliation is the most that can be hoped for, and minimal success in delaying the natural history of the tumor has been achieved. Surgery for relief of symptoms secondary to bulky recurrence is indicated for palliation, as is a repeat course of x-ray therapy. Isolated cases of long-term useful survival have followed doses in the range of 10,000 rads or more [19,20]. This therapy is usually given in smaller increments of an initial dose when there is evidence of recurrent tumor or pain [2]. However, whenever the dosage to the spinal cord or the base of the brain exceeds 4,000 rads, there is a concern about possible permanent radiation damage. These patients are difficult to care for late in the course of their disease when multiple cranial nerve paralysis, long tract signs, and intractable pain become progressively severe. Chordomas usually remain localized; however, about 10 per cent metastasize distally. One case is presented of a large retropharyngeal mass with destruction of the second cervical vertebra that recurred locally in 3 months despite usual treatment; distal bony metastases were found 3 years after initial treatment. The histology of the lesion, its recurrence, and distal bony metastases were essentially indistinguishable from the other lesions that remained localized. Short periods of palliation from severe local pain and exquisite tenderness have been achieved by local resection of the involved bones. © 1979.