Childhood Cancer Types – Retinoblastoma

Hello everyone,

Childhood cancer is a subject extremely close to my heart as I lost my cousin to it. Today I thought I would focus on a childhood cancer type.

On average, the UK sees around forty cases of retinoblastoma in a year. Most of these cases are found in children under five years of age but it can affect children of any age.

Retinoblastoma is a tumour that occurs in the light sensitive lining of the eye called the retina. There are two types of retinoblastoma:

*A non heritable form where the cancer is unilateral (only affects one eye). The cause of this type is still unknown as yet.

*A heritable form where the cancer is bilateral (affecting both eyes). In some cases it can only affect one eye as well though. This type account for two in every five cases of retinoblastoma and is caused by a gene abnormality which has allowed the tumour to develop. This can be inherited form a parent or occur at an early stage of development whilst the child is still in the womb. This gene is known as Rb and people with this have an increased risk of developing another cancer type later in life. Genetic counselling is available for people who have retinoblastoma in their family. Not all children born to parents with this gene will be affected but should be checked for it anyway.

Some children with retinoblastoma will not have any symptoms and it is usually picked up by screening of children with family history of the condition. However, a symptom of retinoblastoma is a white pupil which does not reflect light. This can be detected in photographs using flash as the affected eye will look white in the photo. Some children with retinoblastoma have a squint whilst larger tumours can cause painful red eye.

Tests to diagnose retinoblastoma can be done under anesthetic and an ophthalmologist will examine the eye. Unlike most cancers, retinoblastoma can be diagnosed on sight, by their appearance. A biopsy is normally unnecessary. Examinations under anesthetic will be used to monitor treatment progress. Ultrasound scans, MRI, lumbar puncture and blood tests may be used to help diagnose the condition and stage the tumour.

There are two stages commonly used for retinoblastoma:

*intraocular retinoblastoma – cancer is in one or both eyes but has not begun to spread to other parts of the eye or surrounding tissue.

*extraocular retinoblastoma – the cancer has spread beyond the eye to surrounding tissue or to other parts of the body.

Treatment for retinoblastoma will depend on the size, position and stage of the cancer. Treatment is used to primarily remove the cancer but to also try and preserve sight in the affected eye, although some children sadly do lose some of their sight.

Smaller tumours can usually be treated with cryotherapy, laser therapy, plaque or thermotherapy.

Larger tumours are usually treated with chemotherapy, radiotherapy and/or surgery. If the tumour is really large and vision has been lost then an operation called enucleation may be used to remove the eye and replace it with a prosthesis.

My heart goes out to anyone watching their child suffer from retinoblastoma, what brave children they are.

xxxx