Third (fourth and fifth) nonocular tumors in survivors of retinoblastoma
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
References (29)
- et al.
Second nonocular tumors in survivors of heritable retinoblastoma and prior radiation therapy
Am J Ophthalmol
(1998) - et al.
Second cancers in retinoblastoma
Lancet
(1973) - et al.
Second tumors in nonirradiated bilateral retinoblastoma
Am J Ophthalmol
(1979) - et al.
Second nonocular tumors in retinoblastoma survivors. Are they radiation-induced?
Ophthalmology
(1984) - et al.
Incidence of second neoplasms in patients with bilateral retinoblastoma
Ophthalmology
(1988) - et al.
Second nonocular tumors in survivors of bilateral retinoblastoma. A possible age effect on radiation-related risk
Ophthalmology
(1998) - et al.
A phase I/II study of subconjunctival carboplatin for intraocular retinoblastoma
Ophthalmology
(1999) Retinoblastoma 1990diagnosis, treatment, and implications
Pediatr Ann
(1990)- et al.
Second primary neoplasms in patients with retinoblastoma
Br J Cancer
(1986) Retinoblastoma associated with other primary malignant tumours
Trans Ophthalmol Soc U K
(1974)