Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma

Objective. This study aimed to investigate the incidence, timing, pattern, and distribution of, as well as survival as a result of, third, fourth, and fifth primary tumors in survivors of retinoblastoma. Design: This study was a retrospective case series of patients diagnosed wi th retinoblastoma and a second malignant neoplasm. Records were examined fo r demographic, prior treatment, and second tumor information, as well as an y evidence of the development of a third, fourth, or fifth nonocular tumor. When possible, telephone inquiries were conducted for follow-up. Participants: The study included 1506 patients followed in the Ophthalmic O ncology Center at New York-Presbyterian Hospital, New York Weill Cornell Me dical Center, 211 of whom developed a second tumor and had sufficient treat ment data to be useful for analysis. Main Outcome Measures: The development of third and additional nonocular tu mors and survival from these tumors were the primary outcome measures. Results. Of 211 second-tumor patients, 142 died before an additional malign ancy developed (median survival time, 1.8 +/-0.3 years) and in 28, third tu mors developed (5-year incidence rate, 11%; 10-year incidence rate, 22%; me dian time to third tumor development, 5.8 +/-8.3 years). The 5- and 10-year survival rates for this group were 41% and 30%, respectively (median survi val time, 4.1 +/-1.0 years). Of 28 patients in whom third tumors developed, 27 (96%) had received radiation therapy for their retinoblastoma. The most common sites for third tumors were soft tissues of the head (36% of all th ird tumors) and skin (36% of all third tumors). In six patients, a fourth t umor developed, and in two patients a fifth tumor developed. All fourth and fifth tumors were found in the soft tissues of the head, the skin, or the bones. Conclusions: Survivors of retinoblastoma in whom second malignant neoplasms develop are at a higher risk for the development of additional tumors than they were for the development of a second tumor. The locations and expecte d ages at which additional tumors develop are consistent with the patterns we have seen in second tumors. Ophthalmology 2001;108:1868-1876 (C) 2001 by the American Academy of Ophthalmology.