title: Retinoblastoma.
abstract: Retinoblastoma is the most common neoplasm of the eye in childhood, and represents 3% of all childhood malignancies. Retinoblastoma is a cancer of the very young; two-thirds are diagnosed before 2 years of age and 95% before 5 years. Retinoblastoma presents in 2 distinct clinical forms: (1) a bilateral or multifocal, heritable form (25% of all cases), characterized by the presence of germline mutations of the RB1 gene; and (2) a unilateral or unifocal form (75% of all cases), 90% of which are nonhereditary. The treatment of retinoblastoma is multidisciplinary and is designed primarily to save life and preserve vision.
authors.affiliation: Department of Pediatric Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, 450 Brookline Avenue, D3-133, Boston, MA 02215, USA. Electronic address: carlos_rodriguez-galindo@dfci.harvard.edu.
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
authors.affiliation: Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02215, USA.
authors.forename: Darren B
authors.initials: DB
authors.lastname: Orbach
authors.affiliation: Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02215, USA.
authors.forename: Deborah
authors.initials: D
authors.lastname: VanderVeen
mesh_terms: D016161:Genes, Retinoblastoma; D006801:Humans; D009154:Mutation; D009367:Neoplasm Staging; D019572:Retinal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D012175:Retinoblastoma / Q000235:genetics / Q000473:pathology* / Q000628:therapy
keywords: Chemotherapy; Germline mutation; Retinoblastoma; Second malignant neoplasms