title: Diagnosis and management of retinoblastoma.
abstract: BACKGROUND
  Retinoblastoma is a highly malignant tumor of the eye that manifests most often in the first 3 years of life.
  
  
  METHODS
  Published articles were reviewed to evaluate the clinical features and current methods of diagnosis and to assess the trends in management.
  
  
  RESULTS
  This malignancy leads to metastatic disease and death in 50% of children worldwide but in less than 5% of children in the United States and other developed nations with advanced medical care. Over the past decade, there has been a trend away from enucleation and external beam radiotherapy and toward chemoreduction followed by focal therapies. This is largely due to more effective chemotherapeutic regimens, improved focal treatment modalities, and the desire to avoid loss of the globe and/or exposure to radiotherapy. Chemoreduction and focal therapies are most successful for eyes with minimal to moderate retinoblastoma, with enucleation needed in less than 15% of cases. Eyes with very advanced retinoblastoma require enucleation in approximately 50% of cases.
  
  
  CONCLUSIONS
  Progress in the clinical recognition and management of retinoblastoma has led to high survival rates. Improved methods of treatment using chemoreduction and focal treatments without the need for external beam radiotherapy allow preservation of the eye in some cases, often with visual function.
authors.affiliation: Ocular Oncology Service, Wills Eye Hospital, Thomas JeffersonUniversity, Philadelphia, PA 19107, USA. carol.shields@shieldsoncology.com
authors.forename: Carol L
authors.initials: CL
authors.lastname: Shields
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Shields
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D002675:Child, Preschool; D002882:Chromosomes, Human, Pair 13; D003131:Combined Modality Therapy; D017679:Cryotherapy / Q000379:methods; D003937:Diagnosis, Differential; D005128:Eye Diseases / Q000175:diagnosis; D015353:Eye Enucleation / Q000379:methods; D005134:Eye Neoplasms / Q000556:secondary; D017353:Gene Deletion; D006801:Humans; D006979:Hyperthermia, Induced / Q000379:methods; D007223:Infant; D007231:Infant, Newborn; D012175:Retinoblastoma / Q000145:classification / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents