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Keywords: MeSH
list: Clival Chordoma, Surgical pathway seeding, Nasal chordoma.
This is a 42-year old male patient who
suffered from complications of tumor seeding as a result of biopsy of a clival
chordoma. The patient was previously biopsied for this lesion via a sublabial
transnasal route at an outside hospital. Although initially pituitary tumor was
suspected, the pathology was consistent with chordoma, and the patient underwent
fractionated radiotherapy of 6400 cGy in 32 fractions. When he presented to
Mayo Clinic in Florida, he had a complete left sixth cranial nerve palsy with decreased
facial hair and low testosterone levels.
Pre-operative axial and sagittal T1-weighted MRI of
the brain with contrast showed a heterogeneously enhancing mass that appeared
to originate from the superior half of clivus, with rostral and dorsal invasion
of the sellar region and cavernous sinus bilaterally (Figure 1A). The brainstem
was compressed at the level of the pons caudally, and the optic chiasm and
pituitary infundibulum were displaced upward.
The lesion extended rostrally along the nasal septum, occluding parts of
the nasal cavity.
MRI findings were
suggestive of chordoma, Therefore, the patient underwent an image-guided
transnasal debulking of the clival chordoma and resection of the nasal septum
mass. The Stealth system was employed for image guidance to minimize operative
risk. The CUSA (Cavitron Ultrasonic Aspirator) was used to facilitate
tumor debulking, and the brainstem was decompressed. Due to prior surgery and radiotherapy, the
tumor was found to be tenacious, with excess scar tissue. Residual adherent tumor was left due to the
risk of basilar artery injury. There was no intraoperative evidence of CSF
leakage.
Post-procedural axial
T1-weighted MRI with gadolinium, and sagittal T1-weighted MRI without
gadolinium confirmed generous debulking of the tumor (Figure 1B). The pathology of the nasal septal mass was
consistent with chordoma (Figure 1C).
After one-month follow-up, the patient was neurologically stable with
persistent left CN VI palsy.
In conclusion, chordomas
are slow growing, yet aggressive and life-threatening tumors. Outcomes
for chordoma patients vary widely, and are highly dependent on the course of
treatment received and the individual tumor’s behavior. Clival chordoma
can be fibrous and adherent to surrounding structures. Therefore tumor removal
must be done cautiously to avoid damage to surrounding critical vascular and
neural structures, and to preserve neurological function. Modern treatment approaches can significantly
prolong patient survival, and proton beam therapy is widely used as an adjuvant
treatment. Neurosurgeons must be aware
of the serious complication of chordoma seeding along the biopsy pathway in
clival chordoma. (1-3)
1.
Gupta D, Rathore PK, Chauhan A, Khurana N. Unusual
Presentation of Chordoma in Nose. Indian J Otolaryngol Head Neck Surg.
2016;68(3):380-3.
2.
Iloreta AM, Nyquist GG, Friedel M, Farrell C, Rosen
MR, Evans JJ. Surgical pathway seeding of clivo-cervical chordomas. J Neurol
Surg Rep. 2014;75(2):e246-50.
3. Krengli M, Poletti A,
Ferrara E, Fossati P. Tumour seeding in the surgical pathway after resection of
skull base chordoma. Rep Pract Oncol Radiother. 2016;21(4):407-11.
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