Skull base chordomas: a review of 38 patients, 1958-88.

The presentation and results of treatment are reviewed for 38 patients with skull base chordoma treated at the National Hospital for Neurology and Neurosurgery between 1958 and 1988. With few exceptions, previous studies have combined results for clival and sacral chordomas, or for chordomas and other similar tumours such as chondrosarcoma, and thus it is difficult to be specific about effects of therapy. This study included histological review using immunohistochemistry to confirm diagnosis. Analysis of the survival data for our patients suggests that there are two subgroups with distinct survival patterns: one group with high mortality within the first 5 years, and a second group with an indolent disease process and near normal life expectancy. The age of the patients at presentation ranged from 7 to 78 years, with a mean of 44.3 years. Male: female distribution was 6:5. The commonest presentation was with cranial nerve palsy (94%) or with headache (60%). The most frequently involved cranial nerve was the VIth (60%), followed by the IXth and Xth (40% each). Comparing our results with those of 50 years ago, there was little improvement in the outlook for these patients, despite improvements in surgical approaches and the use of radiotherapy. The promising results in skull base tumours using proton therapy must be treated with caution until definite criteria for diagnosis and outcome have been established. There is a case for a multicentre prospective study of this disease.