title: Orbital sarcomas in retinoblastoma patients: recommendations for screening and treatment guidelines.
abstract: OBJECTIVE
  Retinoblastoma is the most common primary ocular malignancy in children. Although currently retinoblastoma has an excellent survival rate in developed countries, hereditary retinoblastoma survivors as well as those with a history of radiation therapy as children are at an increased risk for second primary tumors (SPTs), and specifically, for orbital sarcomas. Despite the known increased risk for SPTs in retinoblastoma survivors and the associated morbidity and mortality, no screening or treatment guidelines exist.
  
  
  RESULTS
  Understanding of retinoblastoma tumorigenesis and genomic expression has expanded significantly, and treatment has evolved with a shift away from radiotherapy. Until the last two decades, however, radiation was the therapy of choice for patients with bilateral disease. Because both hereditary retinoblastoma and radiation are independent risk factors for the development of SPTs such as sarcomas and these SPTs are often fatal, appropriate surveillance for retinoblastoma survivors is crucial.
  
  
  CONCLUSIONS
  As a result of the excellent survival rates for retinoblastoma patients, it is important to: recognize the risk of sarcoma, particularly in patients with hereditary retinoblastoma and/or prior radiation therapy; establish a screening protocol, such as the one proposed, to maximize early detection; and discuss and develop treatment guidelines for high-risk patients. Future directions of research for these patients will involve the development of molecularly targeted agents and the use of proton radiotherapy.
authors.affiliation: aThe University of Iowa Hospitals and Clinics, Department of Ophthalmology and Visual Sciences, Iowa City, Iowa bSkull Base Division, Neuro-Ophthalmology Service Wills Eye Hospital, Pennsylvania, USA.
authors.forename: Meredith S
authors.initials: MS
authors.lastname: Baker
authors.forename: Lindsay K
authors.initials: LK
authors.lastname: McConnell
authors.forename: Teri T
authors.initials: TT
authors.lastname: Kleinberg
authors.forename: Erin M
authors.initials: EM
authors.lastname: Shriver
authors.forename: Jurij R
authors.initials: JR
authors.lastname: Bilyk
authors.forename: Richard C
authors.initials: RC
authors.lastname: Allen
mesh_terms: D003941:Diagnostic Techniques, Ophthalmological / Q000592:standards; D006801:Humans; D016609:Neoplasms, Second Primary; D009918:Orbital Neoplasms / Q000175:diagnosis* / Q000628:therapy; D017410:Practice Guidelines as Topic; D019572:Retinal Neoplasms / Q000401:mortality / Q000473:pathology*; D012175:Retinoblastoma / Q000401:mortality / Q000473:pathology*; D012307:Risk Factors; D012509:Sarcoma / Q000175:diagnosis* / Q000628:therapy; D015996:Survival Rate