Original Articles
Second nonocular tumors in survivors of heritable retinoblastoma and prior radiation therapy

https://doi.org/10.1016/S0002-9394(98)00146-9Get rights and content

Abstract

PURPOSE: The principal objectives of this study were to estimate the incidence of second tumors among children treated for heritable retinoblastoma during a 50-year period and to investigate the relationship between these tumors and previous radiation therapy.

METHODS: The records of all retinoblastoma patients examined at the Mayo Clinic from 1941 through 1990 were retrospectively reviewed. The therapeutic modality used to manage the tumor, the occurrence of any second malignancy, and current follow-up on all patients were evaluated.

RESULTS: Eighty-two (46%) of 180 children with retinoblastoma had bilateral tumors (76 patients) or unilateral disease and a positive family history (six patients) and were followed for an average of 21.8 years (range, 1 month to 53 years). The Kaplan-Meier estimates of second nonocular tumors among the 82 patients with heritable retinoblastoma were 12% at 10 years, 16% at 25 years, and 30% at 40 years. Although 14 of the 15 patients who developed second malignancies had received radiation therapy, only four of the malignancies occurred within the field of irradiation.

CONCLUSIONS: The relatively low incidence of second tumors among long-term survivors of heritable retinoblastoma in this series of patients occurred predominantly outside the field of irradiation. The variable incidence of second nonocular malignancies in previous reports may reflect variations in radiation technique and dosage.

Section snippets

Methods

The medical records of all retinoblastoma patients examined at the Mayo Clinic between January 1, 1941, and December 31, 1990, were retrospectively reviewed. The following epidemiologic characteristics were obtained on all patients: gender, laterality, family history, age and Reese-Ellsworth classification at diagnosis, and therapeutic modality. An attempt was made to quantitate the radiation dosage received in patients who underwent either radium application, brachytherapy, or external beam

Results

In this retrospective study that spanned 50 years at one institution, 180 patients (84 males and 96 females) with retinoblastoma were identified. One hundred four (58%) of the 180 children had unilateral retinoblastoma, and 76 (42%) had bilateral disease. The mean age at diagnosis for patients with unilateral disease was 24 months, whereas bilateral involvement was diagnosed at a mean age of 15 months. A family history of retinoblastoma was contributory in six (6%) unilaterally affected

Discussion

Children who live in medically advanced nations and who harbor the hereditary form of retinoblastoma are more likely to die of a second nonocular tumor than from disseminated retinoblastoma. Although the occurrence of these second tumors is well recognized, there is some controversy regarding their incidence. Early reports attributed their development to radiation alone. Sagerman and associates26 reported that the incidence of radiation-induced neoplasia correlates with the dose and estimated

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    This study was supported in part by research Grant EY08039 from the National Eye Institute, Bethesda, Maryland, and a grant from Research to Prevent Blindness, Inc, New York, New York.

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