Elsevier

Ophthalmology

Volume 108, Issue 10, October 2001, Pages 1868-1876
Ophthalmology

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

https://doi.org/10.1016/S0161-6420(01)00713-8Get rights and content

Abstract

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 with retinoblastoma and a second malignant neoplasm. Records were examined for demographic, prior treatment, and second tumor information, as well as any 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 Oncology Center at New York-Presbyterian Hospital, New York Weill Cornell Medical Center, 211 of whom developed a second tumor and had sufficient treatment data to be useful for analysis.

Main Outcome Measures

The development of third and additional nonocular tumors and survival from these tumors were the primary outcome measures.

Results

Of 211 second-tumor patients, 142 died before an additional malignancy developed (median survival time, 1.8 ± 0.3 years) and in 28, third tumors developed (5-year incidence rate, 11%; 10-year incidence rate, 22%; median 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 survival 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 third tumors) and skin (36% of all third tumors). In six patients, a fourth tumor 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 expected ages at which additional tumors develop are consistent with the patterns we have seen in second tumors.

Section snippets

Materials and methods

This study is based on 1506 patients with retinoblastoma identified from medical records on file at the Ophthalmic Oncology Center at New York-Presbyterian Hospital, New York Weill Cornell Medical Center. A previous report contains a description of the methods used to locate survivors and document deaths.19 Briefly, information on date of birth, retinoblastoma laterality, date of diagnosis, treatment history for retinoblastoma, and second neoplasm diagnosis date and treatment history was

Results

Relevant population data for the 211 patients with second nonocular tumors are presented in Table 1. The second tumors in this study were diagnosed between March 1925 and December 1992. The patients’ age at diagnosis of second malignant neoplasm ranged from 1.3 years to 65.0 years, with a median age of 16.4 years. In four cases, the second tumor was detected at autopsy. The follow-up period from the date of retinoblastoma diagnosis ranged from 2.0 years to 74.3 years, with a mean follow-up for

Incidence and timing

Survivors of germinal retinoblastoma are at increased risk for the development of a variety of additional nonocular neoplasms, including osteogenic sarcomas of the skull and long bones, soft tissue sarcomas, cutaneous melanomas, pineoblastomas, breast carcinomas, and Hodgkin’s disease.21 Cumulative incidence reports vary, but we now believe that a rate of 1% per year of life (e.g., a 20% chance of a second tumor developing by age 20) is a reasonable estimate for purposes of patient counseling.26

Conclusions

Based on this study, we can now inform physicians, patients, and families of children with bilateral retinoblastoma that they are at life long risk for the development of additional nonocular cancers. If they survive treatment for retinoblastoma, they are at risk for second nonocular cancer. If they survive that second cancer, they are at risk for the development of a third, fourth, and even fifth cancer. Ultimately, most bilateral retinoblastoma patients will have multiple cancers that will

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