Craniovertebral junction chordomas: Case series and strategies to overcome the surgical challenge

Introduction: Chordomas are rare and malignant primary bone tumors. Different strategies have been proposed for chordomas involving the craniovertebral junction (CVJ) compared to other locations. The impossibility to achieve en bloc excision, the impact on stability and the need for proper reconstruction make their surgical management challenging. Objective: The objective is to discuss surgical strategies in CVJ chordomas operated in a single-center during a 7 years' experience (2013-2019). Methods: Adult patients with CVJ chordoma were retrospectively analyzed. The clinical, radiological, pathological, and surgical data were discussed. Results: A total number of 8 patients was included (among a total number of 32 patients suffering from skull base chordoma). Seven patients underwent endoscopic endonasal approach (EEA), and posterior instrumentation was needed in three cases. Three explicative cases were reported: EEA for midline tumor involving lower clivus and upper cervical spine (case 1), EEA and complemental posterior approach for occurred occipitocervical instability (case 2), C2 chordoma which required aggressive bone removal and consequent implant positioning, focusing on surgical planning (timing and type of surgical stages, materials and customization of fixation system) (case 3). Conclusion: EEA could represent a safe route to avoid injuries to neurovascular structure in clival locations, while a combined approach could be considered when tumor spreads laterally. Tumor involvement or surgical procedures could give raise to CVJ instability with the need of complementary posterior instrumentation. Thus, a tailored preoperative planning should play a key role, especially when aggressive bone removal and implant positioning are needed.