Abstract

Retinoblastoma is a rare but significant cause of childhood eye cancer world-wide. The prognosis depends upon early diagnosis and treatment but also upon accurate classification of the tumours. Unilateral incidence is normally non-hereditary compared with bilateral incidence where secondary tumours are more common. Survivorship is much better for unilateral compared with bilateral and trilateral retinoblastoma. Early signs are important to detect and photography can assist in identifying no return of “red-eye” during flash photography and yellow appearance of the tumour. Treatment options are discussed together with new psycho-oncology approaches that address potential trauma in the survivor as well as in the family of the survivor.

Highlights

  • The human eye is a complex organ that allows immense detail to be assimilated and, with both eyes, enables judgement of depth via stereoscopic vision

  • Rb presents from birth to 5 years of age, typically being diagnosed in children before the age of 3 years of age [7, 8]

  • The standard treatment options for when the cancer has spread within the eye, known as orbital and loco-regional metastasis, is chemotherapy and radiation therapy; with the cure rate standing at 60 to 85 per cent [30]

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Summary

Introduction

The human eye is a complex organ that allows immense detail to be assimilated and, with both eyes, enables judgement of depth via stereoscopic vision. Enucleation: As a result of earlier tumour detection as well as improved use of more conservative eye-sparing treatments such as focal therapy, there has been a significant decrease in the use of enucleation in patients with Rb over the last 40 years [3].

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