Investigation of ectopic recurrent skull base and cervical chordomas: The Institut Curie's proton therapy center experience

Background Cervical and skull base chordomas may relapse locally, marginally, or in ectopic sites (ie, surgical pathway, lymph nodes, prevertebral space, subdural space, or distant organs). Methods Among 371 patients treated between 1995 and 2010, we matched the initial dosimetry with the 3D imaging of recurrence and selected the patients with isolated ectopic recurrence. Results During our follow-up, we identified 13 patients who developed ectopic relapses (n = 18) in the form of lung metastasis (n = 2), axial dissemination (n = 6), nodal recurrence (n = 2), subcutaneous metastasis (n = 3), and/or seeding along the surgical pathway (n = 5). Despite treatment of these 13 patients with radiation, surgical interventions, and/or chemotherapy, we could only salvage 5 patients with recurrence in surgical pathway, whereas the remaining 8 patients succumbed to a poor prognosis. Conclusion Our study emphasizes an urgent need for prediction and early diagnosis of ectopic relapses in patients with cervical and skull base chordoma to improve accuracy of their aggressive treatments.