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.


METHODS
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.


METHODS
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.


METHODS
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.
Abramson
D H
DH
Department of Ophthalmology, New York-Presbyterian Hospital, New York Weill Cornell Medical Center, New York, New York, USA.
Melson
M R
MR
Dunkel
I J
IJ
Frank
C M
CM
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D008875:Middle Aged; D009381:Neoplasms, Radiation-Induced / Q000209:etiology* / Q000401:mortality; D016609:Neoplasms, Second Primary / Q000209:etiology* / Q000401:mortality / Q000473:pathology; D019572:Retinal Neoplasms / Q000401:mortality / Q000532:radiotherapy*; D012175:Retinoblastoma / Q000401:mortality / Q000532:radiotherapy*; D012189:Retrospective Studies; D012983:Soft Tissue Neoplasms / Q000209:etiology* / Q000401:mortality / Q000473:pathology; D015996:Survival Rate; D017741:Survivors; D013997:Time Factors