Background: Patients with bilateral retinoblastoma are well recognized to have a high risk of developing a second malignancy, but there are little published data regarding the outcome of these patients following treatment.
Patients and methods: We identified 15 patients with a history of bilateral retinoblastoma who received treatment at Memorial Sloan-Kettering Cancer Center for a newly diagnosed second malignancy. The median age of second tumor occurrence was 18 years (range 10-32 years). Three patients later had a third tumor (18 tumors total). Tumor sites included facial structures in 14 cases and extremities in 4. Histologies included osteosarcoma (5), leiomyosarcoma (5), high-grade spindle cell sarcoma (3), malignant fibrous histiocytoma (3), malignant mesenchymoma (1), and angiosarcoma (1).
Results: Nine patients are alive: 7 disease free at a median of 29 months (range 6-214 months) and 2 with residual disease 59 and 148 months post-diagnosis of the second malignancy. Six patients have died at a median of 31 months (range 16-98 months) after diagnosis of the second malignancy.
Conclusions: Patients with a history of bilateral retinoblastoma who develop a second malignancy may enjoy extended periods of survival. Aggressive therapy appropriate to the tumor histology and site is indicated.