ABSTRACT
Intracranial chondrosarcoma are rare tumours, accounting for approximately one in 1000 intracranial neoplasms. Although more common in the axial or appendicular skeleton, intracranial tumours present a challenging surgical and oncological problem. Chondrosarcoma have a predilection for the skull base and although commonly slow growing, Grade II and III lesions do occur. We present two illustrative patients from the Royal Melbourne Hospital, Australia, demonstrating dramatically differing presentation and clinical outcome and the diagnostic difficulties that may arise. A review of the literature regarding skull base chondrosarcoma is presented. We summarise the clinical, radiological and histological features. The evidence for surgical resection, radiotherapy and chemotherapy is presented and critically evaluated. Based on the available evidence, we advocate maximal safe resection, followed by radiotherapy for Grade II and III tumours. There is no current role for chemotherapy. Radical excision should not be attempted at the expense of neurological function.