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

Med Pediatr Oncol. 1998 Jan;30(1):59-62. doi: 10.1002/(sici)1096-911x(199801)30:1<59::aid-mpo14>3.0.co;2-3.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Child
  • Combined Modality Therapy
  • Disease-Free Survival
  • Humans
  • Neoplasms, Second Primary / therapy*
  • Retinal Neoplasms / therapy*
  • Retinoblastoma / therapy*
  • Treatment Outcome