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Outcome of patients with a history of bilateral retinoblastoma treated for a second malignancy: The Memorial Sloan-Kettering Experience

✍ Scribed by Dunkel, Ira J.; Gerald, William L.; Rosenfield, Nancy S.; Strong, Elliot W.; Abramson, David H.; Ghavimi, Fereshteh


Publisher
John Wiley and Sons
Year
1998
Tongue
English
Weight
32 KB
Volume
30
Category
Article
ISSN
0098-1532

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✦ Synopsis


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.

Patients and 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.