Prognosis

Worldwide, inequity in mortality from retinoblastoma persists. 3-year survival rates vary by country:[21]

  • 99.5% (95% confidence interval [CI], 98.8 to 100.0) for children from high-income countries,

  • 91.2% (95% CI, 89.5 to 93.0) for children from upper-middle-income countries,

  • 80.3% (95% CI, 78.3 to 82.3) for children from lower-middle-income countries,

  • 57.3% (95% CI, 52.1 to 63.0) for children from low-income countries.

Enucleation

  • Patient survival is 99%, but there is obviously no ocular salvage.[3]

Systemic intravenous chemotherapy plus focal therapy

  • In specialist centres, one study reported systemic intravenous chemotherapy plus focal therapy results in an ocular salvage rate of 100% of patients with Reese-Ellsworth Group I to IV disease and 83% of patients with Reese-Ellsworth Group V disease.[63] Patient survival typically approaches 95%.

  • One meta-analysis found an overall ocular salvage rate of 70% and a tumour recurrence rate of 15% with intravenous chemotherapy. Ocular salvage rates for International Classification of Retinoblastoma group B and C eyes were higher, at 83% to 89% respectively.[68]

Ophthalmic artery chemosurgery (also known as intra-arterial chemotherapy) plus focal therapy

  • In one meta-analysis, the overall ocular salvage rate was 76% and the tumour recurrence rate was 15%. Ocular salvage rates for International Classification of Retinoblastoma group B and C eyes were 92% and 96% respectively.[68]

Focal therapy alone

  • In properly selected patients (i.e., tumours 2 disc diameters or smaller) ocular salvage rates of 100% can be achieved by focal therapy alone.[3] When ocular salvage is successful there is no effect on patient survival.

External beam radiation

  • A standard lateral beam approach has an ocular salvage rate of 95% for Reese-Ellsworth Group I to III eyes, and an 83% 3-year ocular salvage rate in Reese-Ellsworth Group IV and V eyes.[105] For Reese-Ellsworth Group Vb tumours, it has a 81% 1-year ocular salvage rate and a 53% 10-year ocular salvage rate.[106]

  • This treatment causes increased patient mortality due to secondary cancers occurring at a rate of 0.5% to 1% per year.

Periocular chemotherapy

  • This technique has an ocular salvage rate of approximately 50%, but it is generally used in advanced, refractory cases only.[3] When successful in ocular salvage, there is no effect on patient survival.

Brachytherapy

  • When used after primary chemotherapy or external beam radiotherapy, brachytherapy has a 60% ocular salvage rate.[104] When successful in ocular salvage, there is no effect on patient survival.

Metastatic disease

  • Overall, most studies have demonstrated that patients with widespread metastatic disease to sites other than the central nervous system (CNS) fare better than patients with disease in the CNS.

  • Mortality rates for patients with optic nerve/choroidal invasion range from 0% to 81% for choroidal invasion; 0% to 37% for tumour anterior to (or up to) the lamina cribrosa; 13% to 69% for tumour beyond the lamina cribrosa but not reaching the surgical margin; and 50% to 81% for tumour at the surgical margin.[85] These mortality rates represent data from 23 different studies on patients with unilateral disease performed over many years with widely varying treatment protocols. As a result, making accurate comparisons among studies is challenging.

  • Orbital disease carries a high risk of mortality.

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