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October 1983

Postradiation Leiomyosarcoma of the Orbit Complicating Bilateral Retinoblastoma

Author Affiliations

From the Departments of Pathology (Drs Font and Jurco) and Ophthalmology (Dr Font) and the Methodist Hospital (Drs Font and Jurco), Baylor College of Medicine, Houston. Dr Brechner was in private practice in New Orleans, but is now practicing in Evergreen, Colo.

Arch Ophthalmol. 1983;101(10):1557-1561. doi:10.1001/archopht.1983.01040020559011

• A 31-year-old woman had bilateral retinoblastoma diagnosed in early childhood. The right eye was enucleated at the age of 1 year, and the left eye was treated with radiation therapy (a total dose of 16,000 rad). Twenty-three years later, in 1975, a subcutaneous mass was noted in the left periorbital region. A biopsy specimen of the mass was taken and a diagnosis of pleomorphic postradiation sarcoma was made. Electron microscopic studies of the periorbital mass confirmed the diagnosis of leiomyosarcoma. After additional radiation therapy, the residual mass was surgically excised. Five years later, a right renal mass, which histologically proved to be a renal cell carcinoma, was discovered. She was treated with nephrectomy, radiation, and chemotherapy. A recent follow-up examination disclosed that the patient is alive and apparently without any evidence of metastatic disease, 30 years after the diagnosis of bilateral retinoblastoma was made. The literature is reviewed regarding postradiation sarcomas and the occurrence of second malignant neoplasms in patients with retinoblastoma.