RETINOBLASTOMA

What Is It❓

Retinoblastoma is often found in very young children, and it is a cancerous condition that originates in the eye.

Retinoblastoma occurs when nerve cells in the retina develop genetic mutations. These mutations cause the cells to continue growing and multiplying. This growing mass of cells forms a tumor. Retinoblastoma cells can invade further into the eye and nearby structures. Retinoblastoma can also spread to other areas of the body, including the brain and spine.

Causes
In the most cases, it's not super clear what causes the genetic mutations that cause retinoblastoma. However, it's possible for children to inherit a genetic mutation from their parents.

SYMPTOMS

• The pupil of the eye appears white instead of red when light shines into it.
• The eyes appear to be looking in different directions (crossed eyes).
• Pain or redness in the eye.

OTHER SYMPTOMS

• An enlarged or dilated pupil.
• Blurred vision or poor vision.
• Different colored irises.

Who❓
While retinoblastoma can be found in children as old as six years old, it is most commonly found in children two years of age and younger.

Diagnosis

Prompt and accurate diagnosis of retinoblastoma is important because if found early enough it is curable, but if left to develop it could spread to other parts of the body.

Eye Exam

One of the primary ways used to diagnose retinoblastoma is an eye exam. The ophthalmologist will see white spots in the eye that indicate the beginnings of tumors, and from there the doctor will use a special machine to examine the retina more closely to confirm that there are tumors. This equipment uses lights and magnifying lenses to look at the retina, and an accurate diagnosis can usually be made by doing this. In most cases the patient is unconscious during these retina examinations.

Severity
The next step of diagnosing the condition is to check on how far the condition has spread. Detailed scans such as a CT scan, and MRI, or an ultrasound are used to determine how far the cancerous cells have spread. In extreme cases a doctor may take bone marrow tests and order a spinal tap to see if the cancerous cells have reached the central nervous system, or to see if the cells have reached the bone marrow which could result in leukemia.

Biopsy

In some cases a doctor may order a biopsy to be taken of the infected area to determine if the tumor cells are benign or malignant. A biopsy is a diagnostic procedure where they would go into the retina and remove part of the infected area for testing. A biopsy isn't really common in diagnosing retinoblastoma because it could result in permanent damage to the eye, and it could also agitate cancerous cells which could cause the cells to spread.

~ What is the prognosis ~
Fortunately, most children with retinoblastoma survive and lead long lives. In the United States, nearly 98% of children survive, but not so in less advanced countries where about 50% of children die from tumor spread. Long term ocular and pediatric examinations are advised for the child.

~ Who is at risk for spread and death ~
The prognosis of a child with retinoblastoma depends on several factors. Children with retinoblastoma that has invaded into the optic nerve, choroid, sclera, orbit or anterior chamber require chemotherapy as they are at greatest risk for spread and death. Those without invasion do not require chemotherapy.

Photocoagulation
(Laser Therapy)

Laser therapy focuses a powerful beam of light onto the cancer and the surrounding tissue. This light destroys cancerous tumors inside of the eye.

Cryotherapy (Freezing Treatment)

Using very cold gas; the tumor is frozen and thawed several times. This destroys the tumor cells and leaves a flat scar with no signs of tumor.

Chemotherapy
is used in addition to laser therapy, cryotherapy or radiation therapies in order to:

🔻 Shrink a tumor before other therapies.
🔺 Help destroy cancer cells that remain after therapy.
🔻 Help destroy cancer if it spreads or recurs.

Enucleation

Enucleation is the surgical removal of the eyeball, leaving eye muscles and the contents of the eye socket intact. This is done when there is no other way to remove the cancer completely from the eye. Unfortunately, loss of vision for the eye is permanent because an eye cannot be transplanted.

External Beam Radiotherapy (XRT)

Also called radiation therapy or irradiation, uses an invisible form of high-energy to kill cancer cells or keep them from growing and dividing.

THE END