Outcome of patients with a history of bilateral retinoblastoma treated for a second malignancy: the Memorial Sloan-Kettering experience.
BACKGROUND
Patients with bilateral retinoblastoma are well recognized to have a high risk of developing a second malignancy, but there are little published data regarding the outcome of these patients following treatment.


METHODS
We identified 15 patients with a history of bilateral retinoblastoma who received treatment at Memorial Sloan-Kettering Cancer Center for a newly diagnosed second malignancy. The median age of second tumor occurrence was 18 years (range 10-32 years). Three patients later had a third tumor (18 tumors total). Tumor sites included facial structures in 14 cases and extremities in 4. Histologies included osteosarcoma (5), leiomyosarcoma (5), high-grade spindle cell sarcoma (3), malignant fibrous histiocytoma (3), malignant mesenchymoma (1), and angiosarcoma (1).


RESULTS
Nine patients are alive: 7 disease free at a median of 29 months (range 6-214 months) and 2 with residual disease 59 and 148 months post-diagnosis of the second malignancy. Six patients have died at a median of 31 months (range 16-98 months) after diagnosis of the second malignancy.


CONCLUSIONS
Patients with a history of bilateral retinoblastoma who develop a second malignancy may enjoy extended periods of survival. Aggressive therapy appropriate to the tumor histology and site is indicated.
Dunkel
I J
IJ
Department of Pediatrics, Memorial Sloan-Kettering Cancer Center and New York Hospital, NY 10021, USA.
Gerald
W L
WL
Rosenfield
N S
NS
Strong
E W
EW
Abramson
D H
DH
Ghavimi
F
F
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D006801:Humans; D016609:Neoplasms, Second Primary / Q000628:therapy*; D019572:Retinal Neoplasms / Q000628:therapy*; D012175:Retinoblastoma / Q000628:therapy*; D016896:Treatment Outcome