Non-ocular Tumors and Other Long-term Complications

In the United States and other developed nations, more patients with retinoblastoma will die from non-ocular second malignant neoplasms than from primary intraocular disease. There is an increased lifetime risk of second malignant neoplasms in survivors of heritable retinoblastoma compared with their nonheritable counterparts. External beam radiation increases the risk of second malignant neoplasms and should be avoided when possible. All patients at risk must be educated concerning the potential signs and symptoms of subsequent malignancies and counseled to avoid exposure to environmental risk factors. The prognosis for patients with second malignant neoplasms is guarded and underscores the importance of counseling patients with the heritable form of the disease.