Chordomas of the Clivus and Craniocervical Junction: Results and Follow-Up after Surgical Resection

Chordomas are rare tumors derived from the notochord remnants. According to their localization and invasiveness, their surgical treatment is still a challenge. The purpose of our study was to evaluate the outcome of 18 patients with large clival chordomas treated surgically in M. Sklodowska Memorial Cancer Center between 1999 and 2006. All the patients had a pathologically confirmed diagnosis of chordoma. For the present analysis the cases of large tumors (maximum diameter > 50 mm) located at the clivus and/or craniocervical junction were included. Different skull base approaches (fronto-orbitozygomatic, transmaxillary, suboccipital-transcondylar, trans-sphenoidal, and the transbasal) were used for the tumor removal. The follow-up period ranged from 3 to 82 months (median, 40 mos). Gross total resection was accomplished in 5 patients (27.5%). Subtotal removal was achieved in 8 patients (45%), and partial removal in 5 patients (27.5%). Adjuvant therapy consisted of postoperative conventional radiation therapy given to 14 patients and proton-photon beam therapy given to 3 patients (22%). There was one death related to surgical treatment. The most common surgical complications were cranial nerve deficits and cerebrospinal fluid leak. At the present follow-up review (median, 40 mos) 13 patients (77%) were still alive, and 4 patients (23%) had died of chordoma recurrence. The analysis of factors influencing the prognosis indicate that the radical resection with the use of skull base approaches is the principal factor favoring better outcome.