Paragangliomas are exceptional in the orbit. Among the 47 cases reported in the world literature more than 1/3 has been classified as alveolar soft part sarcoma. Diagnosis of paraganglioma was confirmed on the basis of pathology reports in only 10. We observed a case in a 46-year-old woman who presented with a tumour of the orbit. The histology examination and the ultrastructure determined by electron microscopy confirmed the diagnosis of paraganglioma. Characteristic histologic features are discussed together with a review of the literature.
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