Chondrosarcoma Involving the Ciliary Body and Choroid.
Alan D Proia, Arpita S Maniar, Brian E Brigman et al.
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Case in brief
A 56-year-old man with hereditary multiple exostoses and prior secondary low-grade chondrosarcomas developed a superotemporal visual field defect. Examination identified a pigmented mass involving the inferonasal ciliary body and peripheral choroid. The mass continued to enlarge after brachytherapy, leading to enucleation; histology showed low-grade chondrosarcoma resembling his previous skeletal tumors. Imaging found no pulmonary or other metastases when the ocular tumor was diagnosed, leaving its origin uncertain. The authors considered it likely a primary intraocular neoplasm arising in the setting of hereditary multiple exostoses and a predisposition to low-grade chondrosarcoma.
What made this case unusual
The intraocular chondrosarcoma was diagnosed without radiographically identified metastases elsewhere, unlike the three previously reported cases of ocular chondrosarcoma metastasis, in which lung and other-site metastases were present before or at ocular diagnosis. This left primary origin versus metastasis unresolved.
Diagnostic / clinical pearl
In a patient with hereditary multiple exostoses and prior chondrosarcomas, an intraocular chondrosarcoma without detectable systemic metastases may raise the possibility of a primary uveal tumor, although the origin can remain uncertain.