Clivus Chordoma: Case Report and Current Considerations on Treatment Strategies

Chordomas are the rare malignant bone lesions derived from the embryonic notochord. They are slow-growing tumors with a locally aggressive behavior. The clival chordomas are extradural, exophytic, and lytic lesions centered on the clivus, and are managed differently from those arising elsewhere because of the emphasis on preserving the neurological function. The gold standard for therapy is the complete resection followed by radiation therapy for a better local control of the tumor. This case report concerns a 20-year old girl with an incidental diagnosis of the clival chordoma, which was first treated via an endoscopic anterior approach to remove the exophytic portion of the tumor. The adjuvant radiation therapy was not feasible because of the macroscopic intradural residual tumor being at the level of jugular foramen. The girl was referred to our institute for the removal of the intradural residual tumor via a petro-occipital trans-sigmoid (POTS) approach followed by adjuvant proton beam therapy. The choice of the surgical approach depends on the size of the tumor, its location, and its anatomical distribution, but should also be balanced against the morbidity, considering the patient's age, and the feasibility of postoperative rehabilitation.