Retinoblastoma - Symptoms, causes and treatment

Retinoblastoma is eye cancer in children. This eye cancer occurs when the retinal cells of the eye grow quickly, uncontrollably, and damage the surrounding tissue. One sign of retinoblastoma is that the eyes look like "cat's eyes" when exposed to light.

The retina is located on the back wall of the eyeball. The retina consists of a network of nerves that function to transmit light to the brain, so that a person can see. Retinoblastoma will cause disruption of retinal function. In advanced stages, this condition will damage the eye tissue and cause blindness. Retinoblastoma is one type of cancer that often attacks children.

Causes of Retinoblastoma

Retinoblastoma is caused by a change or mutation in the RB1 gene. Changes in this gene cause retinal cells to grow quickly, uncontrollably, and damage surrounding tissue. Although rare, eye cancer cells can also spread (metastasize) to other organs.

The exact cause of genetic mutations in retinoblastoma is not known. Approximately 25% of cases of retinoblastoma are inherited in an autosomal dominant pattern, ie the gene that has the disorder is inherited by one parent. The rest occur sporadically and randomly, not inherited from parents.

Symptoms of Retinoblastoma

One of the early and characteristic symptoms of retinoblastoma is the appearance of "cat's eye". This appearance is actually leukocoria, which is a picture of white patches that appear when the eyes are exposed to light. Leukocoria is an abnormal picture, 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
  • Swollen eyes, and the size of one or both eyeballs increases
  • Eyes hurt
  • Changes in the color of the iris in the eye
  • Visual disturbance

When to go to the doctor

Check with the doctor if your child has the above symptoms. Early detection and treatment is expected to prevent the development of cancer and complications that can occur.

If your child is diagnosed with retinoblastoma, follow the treatment and advice given by the doctor. Patients with retinoblastoma will undergo periodic examinations. It aims to determine the progress of therapy and the condition of the child.

Retinoblastoma diagnosis

The doctor will ask questions about the complaints and symptoms experienced by the child, as well as the child's medical history. After that, the doctor will perform 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 perform supporting examinations in the form of:

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

Retinoblastoma Treatment

Treatment for retinoblastoma is aimed at preventing the development of cancer and further damage to the eye. Treatment of retinoblastoma depends on its size, location and spread, and the severity of the cancer.

The sooner it is detected and treated, it is hoped that the treatment results will be better. Some of the treatment options that can be done to treat retinoblastoma are:

Chemotherapy

Chemotherapy aims to kill cancer cells using special drugs. Chemotherapy drugs can be given by injection directly into the eye, through a vein, or by mouth.

The types of drugs used include:

  • cisplatin
  • Carboplatin
  • Etopodise
  • Fluorouracil
  • Doxorubicin
  • Cyclophosphamide
  • Vincent

Laser therapy (laser photocoagulation)

Laser therapy is used to destroy the blood vessels that supply nutrients to the tumor so that it can kill cancer cells.

Cryotherapy

Cryotherapy uses liquid nitrogen to freeze cancer cells before they are removed. Cryotherapy can be done several times until the cancer cells are completely gone.

Radiotherapy

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

There are 2 types of radiation therapy that can be done, namely:

  • External radiation therapy, by focusing radiation rays 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

Surgery is performed to remove the eyeball, which can help 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 carried out in several stages, starting from removing the cancerous eyeball (enucleation). After that, an artificial eyeball (implant) will be placed and attached to the eye muscles.

The eye muscle tissue will adapt to the artificial eyeball as the healing process progresses, so that later the artificial eyeball can move like a real eye even though it can't see.

Complications of Retinoblastoma

If not treated immediately, retinoblastoma can cause complications such as:

  • 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)
  • Phthisis bulbi
  • Blind

Prevention Rethinoblastoma

Retinoblastoma cannot be prevented. The best way that can be done is to always have regular eye exams, especially in children who have family members with a history of retinoblastoma.

For those of you who are planning a pregnancy but have a family history of retinoblastoma, it never hurts to do a genetic test.