Chordoma: Surgery, Treatment & Symptoms


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Chordoma Surgery: Endoscopic treatment of tumors of notochordal origin - Skull Base Institute

Chordoma

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Chordoma: More Complete Resection of Chordomas with Endoscopic Techniques

At the Skull Base Institute, a plethora of transnasal, transoral and transfacial approaches have provided a vast experience in the treatment of chordomas. The individual approach, whether using staged endoscopic minimally invasive techniques or more traditional open microsurgical techniques is offered based on the specifics of each individual situation.

These results have been published in peer reviewed journals and book chapters. Although cure with a minimum 5 year disease free survival is our goal, patients treated elsewhere with either recurrent chordomas or chordomas initially treated with radiation only may still be candidates for surgical resection of their tumors. Although technically somewhat more challenging, specifically tumors that have been irradiated, these patients can still benefit from either an endoscopic minimally invasive transnasal, transoral or transcranial approach or a combination of an open transfacial approach with endoscopic visualization (i.e endoscope assisted).


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Overview
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.

Causes
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.

Symptoms
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.

Diagnosis
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.

Treatment
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.

Prognosis
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.

Articles:

Cha ST, Jarrahy R, Yong WH, Eby J, Shahinian HK: Rare Symptomatic Presentation of Eccordosis Physaliphora and Unique Endoscope-Assisted Surgical Management.

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