Outcome of 132 Operations in 97 Patients With Chordomas of the Craniocervical Junction and Upper Cervical Spine

OBJECTIVE: To study the outcomes of surgery for chordomas of the craniocervical junction and upper cervical spine as well as complication rates, survival, and associated adverse factors. METHODS: Retrospective review of patients (1982-2007) at 2 European centers who underwent transoral, transfacial, transmandibular, and anterior cervical approaches for excision of chordomas of the craniocervical junction and cervical spine. The X-2 test and Fisher exact test were used to determine significant adverse factors (P < .05), and log-rank survival analysis was used to compare outcome in different groups. RESULTS: One hundred thirty-two operations were performed in 97 patients. The most common operations were transoral surgeries and maxillotomies. After surgery, neck pain was the same or better in 98.1% of patients. Of the 18.6% of patients who presented with myelopathy, 27.8% improved, 44.4% remained unchanged, and 27.8% deteriorated. Major complication rates were velopharyngeal incompetence, 3.1%; vertebral artery stroke, 1%; wound infection, 3.1%; dysphagia, 3.1%; failure of fixation, 2.1%; sepsis, 3.1%; meningitis, 3.1%; and cerebrospinal fluid leakage, 6.2%. Five- and 10-year overall survivals were 55% and 36%, respectively. Patients who presented to our units for revision surgery, after prior attempts at resection elsewhere, were associated with a worse survival than patients who underwent de novo surgery. CONCLUSION: We present, to our knowledge, the largest published series of chordomas at the craniocervical junction. Complication rates for these major operations can be minimized at specialist centers, with careful patient selection and counseling. As complete or as radical an operation as possible should be performed at first presentation; the best chance for the patient is the first chance.