A 60-year-old male presented with an increasingly painful and swollen phthisical eye. Enucleation revealed a large mass obliterating the eye with extension into the adjacent extraocular muscle. Histologic examination showed high-grade osteosarcoma. Systemic work-up showed no disease elsewhere, and a diagnosis of orbital extraskeletal osteosarcoma was rendered. Complete resection was not possible, and neoadjuvant radiation was given. The patient subsequently developed pulmonary metastasis and died of disease 5 months after initial diagnosis. Given the highly aggressive nature of this malignancy, raising awareness that extraskeletal osteosarcoma may arise in the background of phthisis bulbi will facilitate timely and accurate diagnosis and treatment.
Keywords: Enucleation; Intraocular neoplasms; Osteosarcoma; Phthisis bulbi.