Orbital Nerve Seath Myxoma with Extraocular Muscle Involvement: A Rare Case

Abstract

A 66-year-old woman with breast cancer presented with a painless mass in the left orbit. MRI revealed a well-defined intraconal mass in the temporal quadrant of the orbit. Fifteen months later, a further MRI indicated the mass had grown, displacing the left optic nerve and making contact with the lateral rectus muscle, suggesting its possible intramuscular origin. Despite the clinical and radiological characteristics of the lesion and its slow growth, a PET/CT study was developed because of the history of malignant disease. No metabolic activity of the mass or malignant lesion in other locations was observed. After surgical excision, histopathological examination revealed an abundant myxoid matrix with few spindle-shaped cells and no signs of malignancy. The cells were immunopositive for CD34, positive for S-100 protein, and negative for EMA, actin, and CD57. A diagnosis was made of a nerve sheath myxoma. The orbital location of these tumors is extremely rare.

• Benign tumors
• immunohistochemistry
• nerve sheath myxoma
• neurothekeoma
• orbital neoplasms

Acknowledgements

The contents of this report were presented as a free paper at the XXII “Sociedad Española de Cirugía Plástica Ocular y Orbitaria” (SECPOO) Meeting, June 14-15th 2012, Badajoz, Spain.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.