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Chordomas are tumors of notochordal origin that may affect the axial skeleton anywhere from the coccyx to the base of the cranium (skull), in either the midline or paramedian position. The cranial and caudal extremes of the spine are most often affected.
Chordomas of the cranial base are particularly debilitating due to the aggressive involvement of local structures. Symptoms vary, but may include headaches, double vision or gait disturbance.
Diagnosis and Treatment
Imaging studies contribute to making the diagnosis of clival chordoma in a large number of cases, but not all.
There is no single surgical method for therapy or resection of these tumors. There are single-stage procedures that differ in the extent of the dissection and the area of optimum exposure. Surgical goals involve as complete a removal of the tumor as possible. The role of adjuvant radiotherapy thereafter is still subject to debate.
Continued development of cranial base microsurgery combined with other minimally invasive endoscopic approaches may allow more complete resection. These innovative approaches developed and used by neurosurgeons involving craniofacial manipulation may be associated with greater disease-free survival.
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