Retinoblastoma
Original Editor - User Name
Top Contributors - Kapil Narale and Kim Jackson
Introduction[edit | edit source]
Retinoblastoma is the most common ocular cancer that occurs in the pediatric population. It is lethal if it is left untreated. [1]
Epidemiology[edit | edit source]
In high-income countries, retinoblastoma is known to be a curable disease, with close to 100% chance of survival. However, in middle- and low-income countries, the prognosis is extremely low. In addition, more than 80% of cases occur in these countries. [1]
It is estimated that the majority of retinoblastoma cases occur in Asia (53%), then Africa (29%), Latin America (8%), North America (3%), and Europe (6%). With this information it is seen that global retinoblastoma patient survival is <30%. It is seen that the survival rate is 40% (23‑70%) in low‑income countries, while it is 79% (54‑93%) in upper‑middle‑income countries. [1]
Assessment[edit | edit source]
Prior to examining the patient, a thorough assessment must be completed. If there is any family history of the condition, a germline mutation may be present, and the child may need to undergo systemic chemotherapy to prevent pineoblastoma, even with a unilateral presentation of the disease.
A=n in-depth evaluation is warranted prior to determining the degree of the necessary treatment, and minimise and prevent from any secondary complications.
The treatment decision for retinoblastoma is dependent on the International Classification of Retinoblastoma (ICRB) classification (similar to the ICF), the presence of extraocular clinical factors, germline test results, family psychosocial status, and available medical facility resources. If there is presence of a germline disease, genetic testing should be conducted in all cases of retinoblastoma, for the patient and the rest of their family.
All patients should go through a baseline high‑resolution simple and contrast‑enhanced magnetic resonance imaging (MRI) of the brain and orbits. Special attention should be given for pineoblastoma or any features of disruption to the optic nerve.
As a part of the assessment, complete blood count, urine sample, and a general physical examination are performed by the pediatric oncologist. Subsequent to the general physical examination, a detailed examination using anesthesia is conducted, where ICRB staging is confirmed, and the first treatment can be administered.
The response to the first treatment can guide long‑term
outcomes. Hence, this might be the most important decision
made by the ocular oncologist, with the goal of delivering
a potent therapy with the needed strength while avoiding
unnecessary toxicity. A simplified consensus of three
retinoblastoma centers on treatment protocol based on ICRB
staging and laterality is presented in Table 2. The specific
treatment modalities are discussed in detail below.
Treatment[edit | edit source]
Enucleation has been the preferred mode of treatment, for advanced retinoblastoma, in low-middle-income countries. however, over the last 30 years, the globe-savaging techniques have been used. [1]
Although the management of retinoblastoma may differ from place to place, and from clinic to clinic, the overall structure of treatment involves a similar framework. [1]
Resources[edit | edit source]
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