Two-Stage Surgical Management of a Giant Pediatric Chordoma of the Craniocervical Junction (C0-C3): A Case Report

Background The surgical management of skull base and craniocervical junction (CCJ) chordomas remains a formidable challenge in modern neurosurgery. Various anterior and posterior approaches are currently employed for the resection of these tumors. Case Presentation: We present a rare clinical case of a three-year-old patient with a three-month history of headaches, progressive weakness in all extremities, and gait disturbance caused by giant chordoma extending from the clivus to the C1–C3 vertebrae. The surgical strategy involved a two-stage procedure: initial posterior craniocervical stabilization followed by tumor resection via a transoral approach. Results Subtotal resection of the tumor was achieved. Postoperatively, the patient's presenting tetraparesis demonstrated near-complete regression. The patient was discharged on the 14th postoperative day. Conclusion This case illustrates that advancements in neurosurgical techniques and accumulated clinical experience now enable the surgical treatment of extremely complex pathologies of the craniocervical junction, even in very young pediatric patients, and can ensure a high quality of life. This report details the surgical strategy and discusses the rationale for a staged approach in the management of giant pediatric chordomas.