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Ectopic chordoma with orbital invasion.

A Moshari1, E E Bloom, I W McLean

  • 1Department of Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA. anouche@hotmail.com

American Journal of Ophthalmology
|March 10, 2001
PubMed
Summary

A rare ectopic chordoma, a type of bone cancer, was found in the orbital wall of a 63-year-old woman. This case highlights unusual locations for this rare tumor.

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Area of Science:

  • Ophthalmology
  • Oncology
  • Pathology

Background:

  • Chordomas are rare malignant bone tumors typically arising from the skull base or spine.
  • Ectopic chordomas, particularly in the orbit, are exceptionally uncommon.

Observation:

  • A 63-year-old female presented with a month of progressive right eye proptosis, eyelid swelling, tearing, and blurred vision.
  • Neuroimaging revealed an osteolytic mass at the sphenozygomatic suture with intracranial extension, orbital invasion, and soft tissue compression.

Findings:

  • Pathological examination showed physaliphorous epithelial cells with characteristic features of chordoma.
  • Immunohistochemical staining was positive for S-100, Vimentin, epithelial membrane antigen, and pancytokeratin, confirming the diagnosis.

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Implications:

  • This case underscores the importance of considering rare tumor locations, such as the orbital wall, in the differential diagnosis of orbital masses.
  • Early diagnosis and appropriate management, including surgical debulking and radiation, are crucial for managing orbital chordomas.