Chordoma
Chordomas, which originate from the notochord, are tumors that can occur along the axial skeleton, from the base of the skull to the coccyx, either in the midline or paramedian position. Typically, chordomas tend to affect the cranial and caudal ends of the spine.
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Chordomas arise from residual notochordal cells that originate from embryonic remnants of the primitive notochord, a flexible rod-like structure present during early human development from which the spinal column forms. Although the notochord typically diminishes considerably after birth, some notochordal cells remain and can give rise to chordoma tumors.
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The involvement of local structures in skull base chordomas can make them especially debilitating, with a wide range of symptoms possible, such as headaches, double vision, or difficulty walking. Typically, the onset of symptoms prompts the need for diagnostic tests to determine the underlying cause.
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While imaging studies play a crucial role in diagnosing clival chordoma in many cases, they are not always conclusive. Although both computed tomography and magnetic resonance imaging are commonly used, neither has been established as the definitive diagnostic tool. Ultimately, the definitive diagnosis of chordoma can only be confirmed through pathology.
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Various surgical methods are available for the treatment and removal of tumors, with single-stage procedures varying in dissection extent and optimal exposure area. The primary surgical objective is to remove as much of the grossly identified tumor as possible, although the effectiveness of adjuvant radiotherapy remains uncertain.
Advanced skull base microsurgery and minimally invasive endoscopic techniques may enable more thorough tumor removal.