Retinoblastoma

Retinoblastoma

Ophthalmology Services

What is Retinoblastoma?

Retinoblastoma is a rapidly developing tumour that arises from the immature cells of the retina, the light-detecting tissue of the eye. It is the most common tumour of the eye in children, occurring in around 1 in 14,000–18,000 live births. As a result, an estimated 8000 instances will arise worldwide. However, it is rare for adults to develop this cancer.

There are two types of retinoblastoma. One is hereditary and affects both eyes (occurs in 10% of cases) while the other is non-hereditary and affects just one eye. Although the illness is genetic, only 6% of newly diagnosed retinoblastoma patients have a positive family history of the disease. In all circumstances, genetic counselling is essential for children who have a germ-line mutation.

What is Retinoblastoma?

Retinoblastoma is a rapidly developing tumour that arises from the immature cells of the retina, the light-detecting tissue of the eye. It is the most common tumour of the eye in children, occurring in around 1 in 14,000–18,000 live births. As a result, an estimated 8000 instances will arise worldwide. However, it is rare for adults to develop this cancer.

There are two types of retinoblastoma. One is hereditary and affects both eyes (occurs in 10% of cases) while the other is non-hereditary and affects just one eye. Although the illness is genetic, only 6% of newly diagnosed retinoblastoma patients have a positive family history of the disease. In all circumstances, genetic counselling is essential for children who have a germ-line mutation.

Symptoms of Retinoblastoma

Since retinoblastoma primarily affects infants and young children, symptoms of the disease are uncommon. Among the signs you may see are:

  • A white hue appears in the centre circle of the eye. (e.g. when light is shone into the eye, such as when a flash photograph of the child is taken)
  • Eye swelling
  • Eyes that appear to be looking in different directions
  • Poor vision
  • Redness in the eyes

Causes of Retinoblastoma

Retinoblastoma develops when nerve cells in the retina start to mutate abnormally. These mutations cause cells to continue growing and multiplying even when healthy cells would die. This increasing clump of cells produces a tumour.

Retinoblastoma cells can spread further into the eye and surrounding structures. Retinoblastoma can also spread to other parts of the body, such as the brain and spine.

It is unknown what causes the genetic mutation that leads to retinoblastoma in the majority of cases. Nevertheless, there is a potential for children to inherit a genetic mutation from their parents.

Tests and Treatments

There are several tests that patients need to go through before a diagnosis can be made. Tests include:

  • Retinal examination – While the eyes are dilated, the doctor will examine the retina with an ophthalmoscope.
  • Optical Coherence Tomography (OCT) – This imaging test generates a cross-sectional, three-dimensional (3-D) image of the inside of the eyes.
  • Ultrasound – This non-invasive, painless examination utilises high-frequency sound waves to provide detailed images of the inside of the eyes and eye socket.
  • Fluorescein angiography – Your doctor may use this procedure to detect changes in the structure or functionality of the retinal blood vessels. To perform the test, the doctor injects dye into a vein in the arm or hand, then scans the retina as the dye highlights the blood vessels.
  • Electroretinogram – An electroretinography (ERG) test, also known as an electroretinogram, evaluates the electrical response of the light-sensitive cells in your eyes.
  • Bone scan – Bone scans can help determine whether retinoblastoma has progressed to other bones or the skull.
  • Lumbar puncture (spinal tap) – This test can detect cancer cells if retinoblastomas grow on the optic nerve and there is a probable tumour on the surface of the brain.
  • Bone marrow aspiration and biopsy – These tests are only utilised when we suspect that the retinoblastoma is spreading outside of the eye and into the blood and bone marrow.

The best treatments for retinoblastoma can be determined by the size and location of the tumour, if cancer has spread to areas other than the eye, your overall health, and your personal preferences. The purpose of treatment is to cure cancer. When feasible, doctors aim to choose treatments that do not require the removal of the eye or loss of vision. Treatments include:

  • Periocular chemotherapy – Higher doses of chemo can be applied directly to the eye for some advanced intraocular cancers without causing systemic side effects.
  • Intravitreal chemotherapy – Chemotherapy is injected into the vitreous humour, the jelly-like substance that surrounds the inside of the eye.
  • Radiation therapy – Intensity-modulated radiation therapy (IMRT) can better target tumours while sparing normal tissues nearby.
  • Laser therapy – Photocoagulation is the technical term for the process of directing a laser beam through the pupil and focusing it on the blood vessels that surround and supply the tumour.
  • Enucleation and eye prosthesis – Enucleation is the surgical removal of an eye and a portion of the optic nerve. When vision cannot be restored and a big tumour has progressed to the optic nerve, this procedure will be applied. It may be performed if the tumour is creating significant intraocular pressure. Following surgery, the patient can be equipped with an artificial eye that is the same size and colour as the other eye.
  • Intra-Arterial chemotherapy – This innovative method could be used in place of systemic chemotherapy. Chemotherapy is injected directly into the ophthalmic artery, the main blood vessel supplying the eye. Depending on the tumour’s shrinkage rate, the procedure can be repeated every few weeks.

At Subang Jaya Medical Centre, our expert team includes some of the best ophthalmologists in Malaysia, delivering state-of-the-art care using first-rate clinical practice and advanced medical diagnostics. Our team of highly experienced eye surgeons also offer a range of services, from surgery to fix cataracts to treatment of retinal disorders like retinal detachment and macular degeneration.

Meet our Specialist

Dr Ronald Arun Das

Designation
Consultant Ophthalmologist and Vitreo Retinal Surgeon
Specialty
Ophthalmology






Dr V. Ulagantheran Viswanathan

Designation
Consultant Ophthalmologist and Vitreo Retinal Surgeon
Specialty
Ophthalmology