Jornal Internacional de Patologia Oftalmológica

Ocular Pathology: Essential for Defining and Refining the Treatment of Retinoblastoma

Matthew W. Wilson, Jena J. Steinle and Dianna A. Johnson

Retinoblastoma is the most common primary intraocular malignancy of childhood affecting approximately 5,000 to 8,000 children per year worldwide. Although regional variations in the incidence of retinoblastoma exist, it is estimated that 80% of children with retinoblastoma reside in developing countries. There is little question that the primary aim in the treatment of retinoblastoma should be preservation of life, with maintenance of the eye and vision as secondary and tertiary objectives. However, new treatment strategies targeting in particular, the secondary objective of saving the eye, are gaining wide acceptance in spite of scant evidence that preservation of vision is enhanced. Additionally, mounting evidence that life-threatening side effects and the potential for metastasis may in fact, compromise the primary aim of treatment. Ocular pathology plays a pivotal role in providing the careful assessment of outcomes necessary for defining and refining these new treatments.