Chondrosarcoma Involving the Ciliary Body and Choroid

A 56-year-old man with a history of hereditary multiple exostoses and secondary low-grade chondrosarcomas was diagnosed with a pigmented mass in his left eye involving the inferonasal ciliary body and peripheral choroid following a superotemporal visual field defect. The intraocular mass continued to grow following brachytherapy, prompting enucleation that revealed a low-grade chondrosarcoma (CHS), similar in appearance to his prior skeletal neoplasms. There are 3 reports of CHS metastatic to the eye, and all 3 individuals had metastases to the lungs and other body sites before or at the time of ocular metastasis. Our patient had no pulmonary or other metastases identified radiographically at the time his intraocular tumor was diagnosed, presenting a conundrum: was the intraocular tumor a metastasis or did it arise de novo in a patient with hereditary multiple exostoses? The intraocular CHS in our patient resembles that reported rarely in other mammals, suggesting that it was a primary neoplasm arising from induced chondrogenic differentiation of uveal mesenchymal stromal cells in a patient with hereditary multiple exostoses and a predilection for developing low-grade chondrosarcomas.