The available data in the literature (177 cases), two current clinical patients, and cases which occurred in The Netherlands (13) were reviewed concerning the clinical presentation, pathological features, radiological data, and treatment options of chondrosarcoma of the cranial base.
The mean age of patients was 37 years, the male/female ratio 1: 1.1. The most frequent complaints were diplopia with oculomotor disorders (51%), headache (31%), and decreased hearing, dizziness, and tinnitus with statoacusticus dysfunction (21%). The mean duration of symptoms before diagnosis was 27 months.
The chondrosarcomas were located in the petrosal bone in 37% (47 cases), in the occipital bone and clivus in 23% (30 cases), in the sphenoïd bone in 20% (25 cases) and to a lesser extent in frontal, ethmoïdal, and parietal bones (14%). In 6% (eight cases) the primary location was in dural tissue. Radiological examinations showed bone destruction and variable calcification (CT), involvement of neuronal and vascular structures (MRI), and mostly hypovascularity on angiography. On histological examination 51% of tumours were classified as grade I, 11% grade II, 30% mesenchymal, and 8% myxoïd. The mesenchymal type was the most malignant as illustrated by a strong tendency to intradural and cerebral growth and possibly occurrence in younger age groups. The treatment of choice until recently was surgery because of the critical location and local aggressive nature. Regrowth of tumour after surgery occurred in 53% of the patients (average after 32 months). Charged particle irradiation gave a five year survival of 83-94% and a local control rate of 78%-91%. Both in surgery and radiotherapy there is treatment related morbidity and mortality that should be considered when offering these therapies.
Recent promising results imply that charged particle radiotherapy, in combination with surgery, may be the therapeutical choice of the future.

Keywords: chondrosarcoma; cranial base neoplasma