A total of 684 chordoma cases including 134 Japanese cases has been reviewed in the present study. Most of chordomas occur in the thirties and forties. In Japanese cases the average age for sellar chondromas was 51.8 years, which was above the average age of 42 years for this series.
There were 76 (57%) head and neck chordomas among the 134 Japanese cases.
Talking cervical chordomas, the first and second cervical vertebrae and their surrounding tissues were more involved than other regions.
Among the cranial chordomas clival chordomas (30 cases) predominated followed by sellar (10 cases), nasopharyngeal (4 cases) and parasellar chordoma (1 case).
In counties other than Japan there is a predominance of males, but in Japan 53 cases were males and 69 females: a female predominance of 1.3 to 1.
There were three major initial manifestations of clival chordomas, they were diplopia initially followed by visual disturbance and then headache in Japanese cases.
The initial symptoms of sellar chordomas were visual disturbance (7/12) and diplopia (1/6), accompanied by exophthalmos, headache and nasal obstruction.
The initial symptom of cervical chordomas were nasal obstruction, deviation of the tongue and maldeglutition. Headache is the cardinal symptom of cranial chordomas.
There are such cases as subdural or subarachnoid haemorrhage due to damage to the basilar artery, resulting in death.
At the first examination the inspection and palpation of chordomas appearing in the pharynx is extremely important for early diagnosis.
To differentiate chordoma from chondrosarcoma and chondoid sarcoma is considerably difficult.
Phosphotangstic acid and reticulum stain are useful to differentiate chordoma from chondro-sarcoma.
Treatment consists of surgical therapy, chemotherapy and radiotherapy independently or in combination. Chordoma is resistant to the radiotherapy. In Japan 17 clival chordoma cases were autopsied, and eight cases (47%) had metastases.
In 23 clival chordoma cases, 10 cases (43%) died within one year.
The average survival rate of clival chordomas was one year and one month.
In 10 sellar chordoma cases no death was reported less than one year. In cranial chordoma cases, sellar chordomas had a better prognosis than clival tumors.
View full abstract