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Orbital chondrosarcoma developing in a patient with Paget disease
C M Mooy1, N C Naus, A de Klein
1Department of Pathology, Erasmus University Rotterdam, The Netherlands. mooy@path.fgg.eur.nl
Purpose:
To describe the radiologic, histopathologic, and cytogenetic features of an orbital chondrosarcoma developing in a patient with Paget disease.
Methods:
A 64-year-old woman presented with rapidly progressive proptosis of her right eye. Computed tomographic scans, histopathologic examination, and cytogenetic analysis were performed.
Results:
Computed tomographic scans disclosed osseous changes of the temporal and frontal bones, with areas of high density consistent with Paget disease. A soft-tissue tumor in the right lateral orbital wall was consistent with Paget sarcoma. On histology, a chondrosarcoma was diagnosed, which was confirmed by fluorescent in situ hybridization.
Conclusion:
This is a unique case of orbital chondrosarcoma developing in a patient with Paget disease.