Chordomas of the skull base: Manifestations and management

Purpose of review: Chordomas are rare congenital tumors of the midline skull base. They are slow-growing and usually cause symptoms only when they compress a cranial nerve. Recent findings: The MRI will show the lesion with irregular uptake of gadolinium. The only way to define the diagnosis is with tissue, and there are two primary pathologic forms of the disease. Resection approaches vary with extent of disease, surgeon preference, and critical structures involved. Summary: There is building evidence that proton beam therapy improves survival and duration of a disease-free state.