title: Retinoblastoma: An update.
abstract: Retinoblastoma is the most common ocular malignancy in children, and is initiated by mutation of the RB1 gene. The tumor may be unilateral or bilateral and can be inherited. Overall survival, eye salvage, and preservation of vision are largely dependent on the stage of disease at presentation. Despite a recently enhanced understanding of the etiology of retinoblastoma, the mortality associated with it remains high worldwide. This may relate to a continuing lack of awareness of the lesion by laypersons, and unavailability of modern treatment facilities. Adverse outcomes are also caused by the occurrence of secondary malignancies after treatment of retinoblastoma in childhood. Early diagnosis, multidisciplinary treatment, and genetic counseling are all priorities in the management of this tumor.
authors.affiliation: Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, Tamil Nadu, India.
authors.forename: Kushal S
authors.initials: KS
authors.lastname: Delhiwala
authors.affiliation: Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, Tamil Nadu, India.
authors.forename: Indu P
authors.initials: IP
authors.lastname: Vadakkal
authors.affiliation: National Reporting Centre for Ophthalmic Pathology, Centre For Sight, Hyderabad, India.
authors.forename: Kaustubh
authors.initials: K
authors.lastname: Mulay
authors.affiliation: Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai, Tamil Nadu, India; Ocular Oncology Services and Shri Bhagwan Mahavir Vitreoretinal Services, Sankara Nethralaya, Chennai 600006, Tamil Nadu, India. Electronic address: drkhetan@yahoo.com.
authors.forename: Vikas
authors.initials: V
authors.lastname: Khetan
authors.affiliation: Division of Surgical Pathology and Cytopathology University of Virginia Health System Charlottesville, VA, USA.
authors.forename: Mark R
authors.initials: MR
authors.lastname: Wick
mesh_terms: D003937:Diagnosis, Differential; D006801:Humans; D009367:Neoplasm Staging; D012160:Retina / Q000473:pathology*; D019572:Retinal Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D012175:Retinoblastoma / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D012307:Risk Factors
keywords: Childhood neoplasms; Heritable neoplasms; Ocular tumors; Retinoblastoma