New Progress in Pathological Classification and Treatment of Intracranial Chordoma

Chordoma is a rare malignant bone tumor with an annual incidence of approximately 0.08 per 100,000 individuals according to the Surveillance, Epidemiology, and End Results (SEER) database, and intracranial chordoma accounts for approximately one-third of all chordoma cases. Its rarity has limited understanding of the disease, particularly of uncommon pathological subtypes, and no universal consensus has been established regarding pathological classification and optimal treatment strategies. Intracranial chordoma is also characterized by difficulty in achieving gross total resection, resistance to conventional radiotherapy and chemotherapy, and a high recurrence rate, which complicate clinical management. This narrative review synthesizes clinical studies and translational research addressing the pathological classification and treatment of intracranial chordoma, with emphasis on surgical approaches, radiotherapy, and targeted therapies. More detailed characterization of pathological subtypes, together with developments in neuroendoscopic surgery, proton beam therapy, carbon ion radiotherapy, and molecularly targeted treatment, has expanded the available approaches to disease classification and management. Evaluation of these developments provides an updated overview of current strategies and remaining challenges in the diagnosis and treatment of intracranial chordoma.