Chordomas are tumors of notochordal origin that may affect the axial skeleton anywhere from the coccyx to the base of the skull, in either the midline or paramedian position. The cranial and caudal extremes of the spine are most often affected by a chordoma.
Chordomas are tumors originating from embryonic remnants of the primitive notochord which is a flexible rod-like structure present in the human embryo from which the spinal column develops. The notochord usually disappears almost entirely shortly after birth, the cells that give origin to chordoma are left over notochordal cells.
Chordomas of the skull base are particularly debilitating due to the aggressive involvement of local structures. Symptomology caused by these masses is generally varied. These may include headaches, double vision, or gait disturbance.
The impetus behind ordering diagnostic tests is usually the development of symptoms.
Imaging studies contribute to making the diagnosis of clival chordoma in a large number of cases, but not all. Neither computed tomography nor magnetic resonance has yet emerged however, as the definitive diagnostic test and true diagnosis of chordoma can only be made pathologically.
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 grossly identified tumor as possible. The role of adjuvant radiotherapy thereafter is still subject to debate.
Continued development of skull base microsurgery combined with other minimally invasive endoscopic approaches may allow more complete resection. These innovative approaches involving craniofacial manipulation may be associated with greater disease free survival.
Chordomas should be treated aggressively especially in younger since failure to do so will result in a locally aggressive behavior that can result in numerous cranial nerve deficits including blindness. When treated early and aggressively patients have and can be cured with follow-ups extending to 10 year disease free survival.
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