Retinoblastoma

Retinoblastoma

Retinoblastoma is a common eye cancer in children. This cancer occurs when cells in the retina of the eye grow quickly and uncontrollably, then damage the surrounding tissue. One sign of retinoblastoma is eyes that look like “cat eyes” when exposed to light.

The retina is located at the back of the eyeball. The retina consists of a network of nerves whose function is to capture light from outside which will then be interpreted by the brain as an image so that a person can see.

Retinoblastoma is a  type of eye cancer  that often affects children. This condition can interfere with retinal function. In its advanced stages, retinoblastoma can damage eye tissue and cause blindness.

Causes of Retinoblastoma

Retinoblastoma is caused by changes or mutations in the RB1 gene. This mutation causes retinal cells to grow rapidly, uncontrollably, and damage the surrounding tissue. Although it rarely happens, these eye cancer cells   can also spread (metastasize) to other organs.

The cause of the genetic mutation in retinoblastoma is uncertain. However, approximately 25% of retinoblastoma cases are inherited in an autosomal dominant pattern. In this pattern, the affected gene is inherited by one parent.

In other cases, retinoblastoma occurs randomly (sporadic) and is not inherited from parents.

Symptoms of Retinoblastoma

One of the early and characteristic signs of retinoblastoma is the appearance of a "cat's eye". This sign is actually leukocoria, which is a white patch that appears when the eye is exposed to light. Leukocoria is an abnormal condition, because the eyes should emit a reddish color when exposed to light.

Leukocoria in retinoblastoma will generally be followed by other symptoms and signs, such as:

  • Crossed eyes ( strabismus )
  • Red eye
  • Eyes ache
  • Swollen eyes
  • Enlargement of one or both eyeballs
  • Change in the color of the iris of the eye
  • Impaired vision

When to see a doctor

Check with your doctor  if your child has the above symptoms. Early detection and treatment are expected to prevent cancer development and reduce the risk of complications.

If your child is diagnosed with retinoblastoma, follow the treatment and recommendations from the doctor. Patients with retinoblastoma need to undergo periodic examinations. This aims to determine the progress of therapy and the condition of the child.

Diagnosis of Retinoblastoma

The doctor will conduct questions and answers about the child's symptoms and medical history. After that, the doctor will do an eye examination. The doctor will also use the help of an ophthalmoscope to see the deeper layers of the eye.

To confirm the diagnosis, the doctor will carry out supporting examinations in the form of:

  • Scanning with  ultrasound  OCT ( optical coherence tomography ), MRI of the eye, or  CT scan  of the eye and bone, to determine the location of the cancer and its spread
  • Genetic test, to find out whether retinoblastoma is inherited from parents or not

Retinoblastoma stage

Based on its size and level of spread, retinoblastoma is divided into several stages, namely:

  • Stage 0
    Cancer only grows in the eye and has not spread.
  • Stage 1 The
    cancer is growing in the eye and there has been small spread to the optic nerve.
  • Stage 2
    Cancer is in the eye and has spread to the optic nerve and the white part of the eye (sclera).
  • Stage 3
    Cancer has spread to the tissues around the eye socket, eye bones, and lymph nodes around the eye.
  • Stage 4
    Cancer has spread to organs that are far from the eyes, such as the bones, liver, brain, or spinal cord.

Retinoblastoma Treatment

Treatment for retinoblastoma aims to prevent the development of the cancer and further damage to the eye. The treatment method used by doctors depends on the size, location and spread, as well as the severity of the cancer.

The sooner cancer is detected and treated, the better the chances of successful treatment. Some of the treatment options that can be done are:

Chemotherapy

Chemotherapy  aims to kill cancer cells using special drugs. Chemotherapy drugs can be given in drinking form, and by injection into the eye or vein. Types of drugs used include:

  • Cisplatin
  • Carboplatin
  • Etopodise
  • Topotecan
  • Doxorubicin
  • Cyclophosphamide
  • Vincristin

Laser therapy ( laser photocoagulation )

Laser therapy  is used to destroy blood vessels that supply nutrients to tumors so that they can kill cancer cells.

cryotherapy

Cryotherapy  uses liquid nitrogen to freeze cancer cells before they are removed. This therapy is considered effective in dealing with cancer cells that are still small. Cryotherapy can be done several times until the cancer cells are completely lost.

Radiotherapy

Radiotherapy  is a cancer treatment using high radiation beams. Radiotherapy can be used to treat cancer that is difficult to treat, reduce the size of the cancer before surgery, or kill cancer cells that have spread to other parts of the body.

There are two types of radiation therapy that can be done, namely:

  • External radiation therapy, by focusing radiation beams from outside the body
  • Internal radiation therapy, using a radioactive substance that is inserted into the body to stop the growth of cancer cells

Operation

The aim of the operation is to lift the eyeball so that it can prevent the cancer from spreading to other parts of the body. This method will be used if the tumor is very large and difficult to treat with other methods.

The operation is performed by first removing the cancer-affected eyeball (enucleation). After that, an artificial eyeball (implant) will be installed and connected to the eye muscles.

As the healing process progresses, the eye muscle tissue will adapt to the artificial eyeball. Later, the artificial eyeball can move like a real eye even though it cannot see.

Retinoblastoma complications

If not treated immediately, retinoblasta can cause complications in the form of:

  • Spread of cancer to other tissues and organs ( metastasis )
  • Retinal detachment
  • Bleeding in the eyeball
  • Glaucoma
  • Inflammation of the eyeball and surrounding tissue ( orbital cellulitis )
  • Pineoblastoma
  • Phthisis bulbi
  • Blindness

Prevention of Retinoblastoma

Retinoblastoma cannot be prevented. However, routine eye examinations can be performed, especially in children who have family members with a history of retinoblastoma.

In patients who have a family history of retinoblastoma planning a pregnancy, the doctor will recommend doing a genetic test first.

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