Ectopic chordoma with orbital invasion.
OBJECTIVE
To report a rare ectopic chordoma within the orbital wall.


METHODS
Case report.


RESULTS
A 63-year-old woman developed swelling of the eyelid, tearing, blurred vision, and progressive proptosis RE of 1 month's duration. Neuroimaging studies revealed an osteolytic mass with epicenter at the sphenozygomatic suture that eroded intracranially, invaded into the orbit, and compressed orbital soft tissues. Surgical debulking was done followed by radiation treatment. The pathologic findings of physaliphorous epithelial cells with multiple vacuoles containing mucin, prominent nuclei, and positive immunohistochemical staining for S-100, Vimentin, epithelial membrane antigen, and pancytokeratin were diagnostic for chordoma.


CONCLUSIONS
Orbital wall ectopic localization of a chordoma distant from the clivus is a rare occurrence.
Department of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA. anouche@hotmail.com
A
A
Moshari
E E
EE
Bloom
I W
IW
McLean
N R
NR
Buckwalter
D014408:Biomarkers, Tumor / Q000032:analysis; D002817:Chordoma / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D005260:Female; D006801:Humans; D007633:Keratins / Q000032:analysis; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D018396:Mucin-1 / Q000032:analysis; D009361:Neoplasm Invasiveness; D009363:Neoplasm Proteins / Q000032:analysis; D009918:Orbital Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D009418:S100 Proteins / Q000032:analysis; D014057:Tomography, X-Ray Computed; D014746:Vimentin / Q000032:analysis
D014408:Biomarkers, Tumor; D018396:Mucin-1; D009363:Neoplasm Proteins; D009418:S100 Proteins; D014746:Vimentin; D007633:Keratins