The 2-stage surgical treatment of a giant chordoma of the clivus growing into the oropharynx and posterior cranial fossa
Chordomas are relatively rare dysontogenetic tumors developing from the remnants of the spinal chord. Intracranial chordomas consist of 85% of all chordomas. Slowly progressive growth with destructive osteolysis in adjacent bones and low metastatic potential are specific for chordomas. Ninety six surgeries were performed in 71 patients with chordomas at the Burdenko Institute of Neurosurgery from 1987 to 1996. This article presents a clinical case of large clival chordoma with extra-intracranial growth in a 58 year-old female patient. The clinical picture is characterized by lesions in the caudal cranial nerves, predominantly on the right side, as well as cerebellar and brainstem signs and symptoms. Two-stage partial removal of malignant clival chordoma was performed via transoral and retrosigmoid approaches. The interval between the stages was 6 months. In the authors' opinion, multistage surgery is preferred in the treatment of giant clival chordomas. Partial removal should be started from the portions of a tumor producing the most prominent neurological signs. This method allows the patient's status to be temporarily improved.