14 Mar 2024

Eye cancer, or retinoblastoma, can occur in various places in the eyeball, thus giving it a variety of technical names. Cancer of the eye itself is rather uncommon, but it is more common to experience cancer of the specific areas within the eye, like the retina or eyelid.

The following information is general information about retinoblastoma.

  • Genetic testing can be helpful to determine if a child has the heritable or non-heritable form of retinoblastoma.
  • Signs and symptoms of retinoblastoma include a “white” looking pupil and eyes that appear to be looking in different directions, like “cross-eyed.”
  • Those children with a family history of retinoblastoma should have their eyes examined regularly to check for signs of progression of new disease.
  • Retinoblastoma is a disease where malignant cells form in the tissue of the retina.
  • This disease can occur in heritable and non-heritable forms.
  • Any child who has heritable retinoblastoma has an increased risk of trilateral retinoblastoma and other cancers.
  • Some factors affect prognosis and the chance of recovery, along with treatment options.
  • Many tests that examine the retina are used to diagnose retinoblastoma.

Retinoblastoma may occur in one, or both, eyes. Another type of retinoblastoma is called, “cavitary,” as it creates small holes or cavities in the retina and eye which creates hollow spaces. This type of disease is extremely rare. While this disease can occur at any age, it most often affects children younger than two years old.

What Tests help Diagnosis Retinoblastoma

One or many of these tests may be needed to help diagnose retinoblastoma:

  • Health History and Physical Examination. To begin, your doctor will check your body and general health. Your doctor may also take note of your health habits, past illnesses, and treatments. They will also discuss your family history to note any links to retinoblastoma.
  • Eye examination with pupil dilation. A standard exam of your eye along with the addition of dilating your pupil will be done. The dilation of the pupil helps to open the pupil wider with medicated eye drops which help them to look deeper into your eye to your lens. Inside of your eye, they will examine the retina and optic nerve. For children, this process may need to be done under anesthesia.

There are many other types of eye exams that may be done when the pupil is dilated. Some are:

  • Fluorescein angiography: This is a procedure to look at the blood vessels and the flow of blood inside of the eye. An orange, fluorescent dye called fluorescein is injected into a blood vessel in the arm and goes straight into the blood stream. As the dye travels through the blood vessel in the eye, a special camera can then take pictures of the retina and choroid to find any abnormalities.
  • Ophthalmoscopy: An exam of the inside of the back of the eye to check the retina and optic nerve using a small magnifying lens and a light.
  • MRI imaging: This is a procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside of the body, such as the eye. The technical name is nuclear magnetic resonance imaging.
  • Ultrasound exam of the eye: This procedure involves high-energy sound waves that are bounced off the internal tissues of the eye, making echoes that are readable by specialists. Eye drops are used to numb the eye and a small probe is placed gently on the surface of the eye. These echoes help your doctor determine the distance between your cornea and retina.
  • RB1 gene test: This is a laboratory test where a sample of blood tissue is tested for a change in the RB1 genes.

This disease can typically be diagnosed without any biopsy, although in some cases may be necessary.

The Stages of Retinoblastoma

After retinoblastoma has been diagnosed there are tests done to find out if any cancer cells have spread within the eye or to other parts of the body. The International Retinoblastoma Staging System, or IRSS, is typically used to determine the stage. The stages are as follows:

  • Stage 0
  • Stage 1
  • Stage II
  • Stage III
  • Stage IV

There are three ways that this cancer can spread within the body, from where it began, to other locations. Treatment will depend on whether it is intraocular, in the eye, or extraocular- outside of the eye. Extraocular retinoblastoma is often metastatic. These tests help to determine the stage of the cancer, if the cancer has spread and help to find the exact location.

  • Bone Scan. A procedure to check if there are any dividing or cancer cells within the bone. A small amount of radioactive material is injected into a vein and travels through the blood stream. The material then collects in the bone, where any areas of cancer show up brighter in the picture because they soak in that radioactive container.
  • Bone marrow aspiration and biopsy. The removal of bone marrow and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist then studies the samples under a microscope to look for signs of cancer. This test is typically done when the doctor believes the cancer has metastasized out of the eye.
  • Lumbar puncture. This procedure collects cerebrospinal fluid, or CSF fluid, from the spinal column. This is done by inserting a needle into the spinal cord and removing a sample of the fluid. This sample is then checked under a microscope for signs that the cancer has spread around the body.

Things that can Affect Prognosis or Recovery

The prognosis and treatment of retinoblastoma depends on these things:

  • If the cancer is new or newly diagnosed, or if it has returned.
  • If a second cancer has formed.
  • The age of the child, or person with retinoblastoma.
  • The likelihood that vision can be saved in one or both eyes.
  • Whether the tumor has spread to the area around the eye, to the brain, or to any other part of the body.
  • The size, stage and location of any tumors.
  • If the cancer is in one or both eyes.

If you suspect you may have retinoblastoma, do not hesitate to meet with your doctor or Optometrist to have an evaluation. The sooner this disease is controlled and treated, the better chances you may have of retaining your vision.

Citations:

Centers for Disease Control and Prevention

National Institutes of Health and Human Services

MedLinePlus.gov