Second tumors in nonirradiated bilateral retinoblastoma

Abstract

We reviewed the records of 1,323 patients with retinoblastoma treated here between 1922 and 1978. Twenty-eight patients underwent bilateral enucleation alone and received no additional treatment for their advanced tumors. Of the 28 patients, 22 (92%) survived. Four patients were lost to follow-up, and two died of metastatic retinoblastoma. Of 22 survivors of bilateral retinoblastoma treated with enucleation alone, three developed second tumors at a mean of 15.3 years after bilateral enucleation. One patient developed a rhabdomyosarcoma in the left temple region, the second patient developed an osteogenic sarcoma of the right femur, and the third developed a malignant melanoma of the left thigh. All three patients died of their disease. The incidence of second tumors in patients who survived bilateral retinoblastoma treated without radiation was 14% (three patients), which is comparable to a series previously reported in which patients were treated with enucleation and irradiation.