The retina is the coating of the back of the eye made of specialized cells that process light and colour for us to see. Uncontrolled growth of retinal cells due to a change in a gene, known as the retinoblastoma (RB) gene, causes retinoblastoma (pronounced ret’-n-oh-bla-stoh’-muh).
Retinoblastoma is a rare form of cancer, affecting 1 in 15,000 to 1 in 30,000 live births. Retinoblastoma is considered a childhood cancer since 95% of cases occur in children less than 5 years of age.
There seems to be an equal chance of the condition occurring in either eye, in either boys or girls, and regardless of race. About 75% of cases occur in one eye, and about 25% of cases occur in both eyes.
The body’s cells reproduce themselves throughout a lifetime – tissues wear out and cells are replaced in a controlled manner. Retinoblastoma, like all cancers, occurs when that control is lost and cells begin to divide at an unusually high rate. Researchers are still working on the exact reasons for the gene mutation in retinoblastoma. However, they have discovered familial and non-familial causes for the disease.
Retinoblastomas can be classified into 2 categories: one is caused by inheriting a defective gene (familial) and the other is caused by a random change in the RB gene during cell replication (sporadic). The main cause of retinoblastoma is through the sporadic form. About 90% of children with retinoblastoma have no previous family history of the disease. Meanwhile, children who have a parent with the disease only account for less than 10% of all diagnosed cases. Retinoblastoma that occurs in only one eye is usually not inherited. Retinoblastoma that occurs in both eyes is almost always inherited.
Although researchers are not completely sure of what causes the retinoblastoma gene to change and lead to retinoblastoma, they are aware of the risk factors for the condition.
Family history is the main risk factor for retinoblastoma. Children who inherit the altered gene have a 90% risk of developing retinoblastoma.
Smaller studies suggest that a parent infected with human papillomavirus (HPV) or a parent’s type of job or work exposure may increase the risk of their offspring developing retinoblastoma, but these two observations are controversial within the medical community.
Several treatment options exist for retinoblastoma, however, treatment depends on the severity of the condition and whether or not the cancer has spread outside the eye. If the doctor believes the patient can keep any part of their vision, laser therapy, or cryotherapy (freezing cancer cells) may be used.
Of the non-surgical options available, radiation therapy is one of the last resorts employed because of the potential side effects of damage to healthy tissue and the possibility of triggering a new non-ocular tumour (not in the eye) in the case of familial retinoblastoma. Chemotherapy is sometimes used to shrink the tumour before other treatment options are used.
Enucleation, also known as removal of the eye, is done only if a child’s vision cannot be saved or if there is a high risk that the cancer will spread.
While there are no preventative measures for retinoblastoma, eye examinations should be considered. Children with a family history of retinoblastoma should be closely followed by an eye specialist shortly after birth and then every 4 months until they reach 4 years of age. As well, siblings of a child with retinoblastoma should have their eyes routinely checked by an eye specialist.
All material copyright MediResource Inc. 1996 – 2025. Terms and conditions of use. The contents herein are for informational purposes only. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Source: www.medbroadcast.com/condition/getcondition/Retinoblastoma
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