title: Outcome of patients with a history of bilateral retinoblastoma treated for a second malignancy: the Memorial Sloan-Kettering experience.
abstract: 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.
authors.affiliation: Department of Pediatrics, Memorial Sloan-Kettering Cancer Center and New York Hospital, NY 10021, USA.
authors.forename: I J
authors.initials: IJ
authors.lastname: Dunkel
authors.forename: W L
authors.initials: WL
authors.lastname: Gerald
authors.forename: N S
authors.initials: NS
authors.lastname: Rosenfield
authors.forename: E W
authors.initials: EW
authors.lastname: Strong
authors.forename: D H
authors.initials: DH
authors.lastname: Abramson
authors.forename: F
authors.initials: F
authors.lastname: Ghavimi
mesh_terms: 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