4.4 Review

Retinoblastoma

Journal

CURRENT OPINION IN OPHTHALMOLOGY
Volume 24, Issue 6, Pages 581-588

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/ICU.0000000000000002

Keywords

ocular oncology; pediatric tumors; retinoblastoma; retinoblastoma gene

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Purpose of reviewRetinoblastoma is the most common malignant intraocular tumor of childhood. Treatment and diagnostic modalities associated to this condition are changing rapidly as our understanding of this condition crystallizes. The purpose of this review is to provide an update of the current understanding of retinoblastoma.Recent findingsKnowledge on tumorigenesis and genomic expression has expanded tremendously with the development of a mouse model for retinoblastoma. Tumor hypoxia has been identified as a significant step in the tumor progression and a novel target for future treatments. Current globe-sparing therapies, including periocular carboplatin, selective ophthalmic artery chemoreduction, intravitreal melphalan, and focal consolidation are being used and investigated actively. Diagnosis and the management of retinoblastoma is also undergoing major advances including wide-field photography, autofluorescence, and high-resolution optical coherence tomography.SummaryProgressive advances in the understanding of retinoblastoma pathogenesis continue to lead treatment strategies. Improvements in the diagnosis and management of retinoblastoma are improving morbidity and mortality associated to this condition in the developed nations. However, it is of outmost importance to flatten the international boundaries to offer prompt care to retinoblastoma children in underdeveloped communities.

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