Retinoblastoma: symptoms, causes, treatment, etc. & bull; hello healthy

Definition

What is retinoblastoma?

Retinoblastoma is a type of eye cancer that attacks the retina. The retina is a network of nerves located at the very back of the eye.

The retina is made of special nerve cells that are sensitive to light. These cells are connected to the brain by the optic nerve. Light patterns or objects that are captured by the retina will be transmitted by the optic nerve to a part of the brain called the visual cortex, so that we can see.

Retinoblastoma occurs when nerve cells in the retina mutate and form a tumor. These cells can also spread to other parts of the body, including the brain and spine.

Generally, this disease can affect one or both parts of the eye. The most common signs and symptoms are white pupils, also known as leukocoria.

Retinoblastoma cure rate is greater if it can be detected early or early.

How often do retinoblastomas occur?

Retinoblastoma is the most common disease in children. As many as 90% of cases of this disease are diagnosed in children under 4 years of age.

As many as 1 in 3 children who suffer from this disease experience it from birth (congenital). In addition, as many as 3 out of 4 children with this type of eye cancer only had a tumor in 1 eye, and the rest had 2 cancer-affected eyeballs.

The number of incidence of this disease in male and female patients is the same. This disease rarely occurs in children over 6 years of age. The incidence of this disease in adults is also very small.

Retinoblastoma can be treated by recognizing the existing risk factors. To find out more information about this disease, you can discuss with your doctor.

Signs & symptoms

What are the signs and symptoms of retinoblastoma?

The most commonly seen signs and symptoms of retinoblastoma are white pupils or leukocoria. This condition occurs in about 60% of children who suffer from this disease.

These white pupils are usually seen when the eyes are exposed to light or flash camera. However, sometimes the presence of leukocoria can be a sign of another eye problem. This condition requires further examination by an ophthalmologist.

Apart from leukocoria, another sign of this type of eye cancer is a condition called "lazy eye", also known as strabismus. This condition causes the eye muscles to weaken, so that the eyes look crossed or focused in different directions.

Other signs and symptoms of this disease include:

• Vision problems
• Sore eyes
• Redness on the whites of the eyes
• Bleeding at the front of the eye
• Swelling of the eye
• The pupil does not shrink when exposed to bright light
• The iris of the two eyes is a different color

There may be some signs and symptoms not listed above. If you have concerns about a particular symptom, consult your doctor immediately.

When should I see a doctor?

If you have any of the signs or symptoms listed above or have any other questions, please consult your doctor. If your family has a history of this disease, make sure you get checked out for early detection and treatment.

Each sufferer's body shows signs and symptoms that vary. To get the most appropriate treatment and according to your condition, immediately visit the doctor or the nearest health service center.

Cause

What causes retinoblastoma?

Basically, the main cause of retinoblastoma is a genetic change or mutation that occurs in the eye cells.

Since we are born, the cells in the eye replicate and develop into new cells that fill the retina. At one point in time, these cells will stop growing and become mature retinal cells.

However, in some cases, these cells continue to grow and develop uncontrollably, causing the appearance of tumor tissue and cancer cells.

These changes in the cells in the retina are caused by gene mutations. Several types of genes that play a role in cell growth and replication are oncogens. Meanwhile, genes in charge of controlling cells and regulating when cells have to die are tumor suppressor genes or antioncogens.

In the case of this disease, the error occurs in the tumor suppressor gene RB1 or retinoblastoma-1. Mutations can occur because of two things, namely passed on from parent to child, or acquired several years after the child was born.

1. Hereditary or bilateral retinoblastoma

This type occurs in 1 in 3 children who suffer from this disease. This condition is caused by a mutation germline on one of the RB1 genes.

These mutations appear in the early stages of child development, even when they are still in the fetus in the womb As many as 9 out of 10 children are born with the mutation germline RB1 will suffer from this disease, and generally occurs in both eyes.

2. Non-hereditary or sporadic retinoblastoma

In non-hereditary or sporadic types, cancer develops in 2 out of 3 sufferers. The mutation of the RB1 gene does not occur since the child is born, but appears at one particular time in his life (acquired).

Patients with this type of cancer do not have the potential to pass on the gene mutation to their children later. However, for both the inherited and non-derived RB1 mutations, it is still not known what the exact cause is.

Risk factors

What factors increase my risk of developing retinoblastoma?

Retinoblastoma is a disease that can affect anyone. However, there are several factors that can increase a person's risk of developing this disease.

Having one or all of the risk factors does not mean you have this disease. There are also several cases that occur even without any risk factors.

The following are risk factors that trigger this disease:

1. Age

Many people with this disease are diagnosed when they are less than 3 or 4 years old. Generally, congenital or hereditary retinoblastoma is diagnosed in the first year after birth.

Meanwhile, types of cancer that are not inherited are usually diagnosed at the age of 1-2 years. This disease is very rarely found in children over 6 years of age and in adults.

2. Descendants of the family

The risk of a child suffering from this disease will be higher if you have parents with hereditary retinoblastoma. This condition often affects both eyes, or is bilateral.

3. Other risk factors

Some other risk factors that may predispose a person to this disease are:

• Lack of consumption of fruits and vegetables during pregnancy
• Exposure to chemicals such as gasoline or exhaust fumes during pregnancy

The absence of risk factors does not mean you cannot develop retinoblastoma. These factors are for reference only, consult your doctor for more information.

Complications

What are the complications caused by retinoblastoma?

Children who receive treatment may develop the disease again after they are cured. Therefore, the doctor will always schedule a follow-up examination or follow-up to check whether the cancer cells in the eye come back.

In addition, children who have this disease from both parents have the potential to suffer from other types of cancer. This condition may occur several years after treatment.

Therefore, it is important for children who have had this disease to routinely perform cancer tests or examinations in other parts of the body.

Stadium

What are the levels or stages of retinoblastoma?

There are several levels or stages in this disease. The staging is based on how far the cancer cells or tumors have spread after the surgical procedure. This division is in accordance with the standard The International Retinoblastoma Staging System (IRSS).

1. Stage 0

At this level, the tumor or cancer cells are only present in the eye. Treatment can still be done without surgical eye removal procedures.

2. Stage 1

The tumor is only in the eye. After the surgical removal of the eye, there are no more cancer cells left.

3. Stage 2

The tumor is only in the eye. After the surgical removal of the eye, there are still cancer cells that can only be seen with a microscope.

4. Stage 3

This stage can be divided into stages 3a and 3b.

• Stage 3a

At this stage, cancer cells have spread from the eye to the tissue around the eye bag.

• Stage 3b

Cancer cells have spread from the eye to the lymph glands around the ear or neck.

5. Stage 4

This stage is also divided into two, namely stages 4a and 4b.

• Stage 4a

At this stage, cancer cells have spread to the bloodstream, but have not yet reached the brain and spinal cord. The tumor may have spread to other parts of the body, such as the bones or liver.

• Stage 4b

At this stage, cancer cells have spread to the brain, spinal cord, and other parts of the body.

Diagnosis & treatment

The information provided is not a substitute for medical advice. ALWAYS consult your doctor.

What tests are done to diagnose retinoblastoma?

When you see signs and symptoms of retinoblastoma in your child, immediately consult the nearest doctor.

First of all, the doctor will perform a thorough physical examination. The doctor will also ask you about risk factors such as a family history of illness.

After that, several tests will be carried out to detect and diagnose this disease:

1. Eye test with dilated pupils

This test is done by placing special drops so that the pupils can widen. With this test, the doctor can see the inside of the eye, including the retina and optic nerve. Depending on the child's age, this test is usually done under an anesthetic.

Some of the types of tests that are performed are:

• Ophthalmoscopy
• Biomicroscopy slit-lamp
• Fluorescein angiography

2. RB1 genetic test

The doctor will take a sample of the child's blood or tissue and examine it in the laboratory. The aim is to determine whether there is a change or mutation in the RB1 gene.

3. Eye ultrasound

This procedure is done by emitting high-power sound waves to the tissues in the eye. From the emission of this wave, a detailed image of the inside of the eye will be generated.

4. MRI

This test uses magnetic and radio waves to produce clear images of the inside of the eye.

5. CT scan

Similar to ultrasound and MRI, this test aims to obtain photos or images of the eye area. Images are taken with an X-ray machine from various angles.

Generally, the diagnosis of retinoblastoma does not require a biopsy procedure, considering the risk is quite high and can actually spread cancer cells to other parts of the body. Meanwhile, all the procedures mentioned above have proven to be accurate in detecting this disease.

If the doctor has confirmed that your child has retinoblastoma, there are several additional tests that will be done to determine the stage of the cancer, such as scan bone and spinal cord biopsy.

What are your treatment options for retinoblastoma?

Treatment options depend on the size of the tumor, its location, whether the cancer has affected one or both eyes, how impaired the vision is, and how far the disease has spread beyond the eye.

The goals of retinoblastoma treatment are to get rid of cancer cells, save the affected eye, improve vision, and reduce the risk of side effects that may occur after treatment.

There are several types of treatment available:

1. Cryotherapy

This therapy is done by using a device to freeze and destroy abnormal tissues of the body. This procedure is also often referred to as cryosurgery .

2. Thermotherapy

In this type of medicine, a machine with a laser light is used on the pupil of the eye. The goal is to deliver heat to destroy cancer cells in the eye.

3. Chemotherapy

Chemotherapy is a procedure that uses drugs to kill cancer cells. This drug is given by injection or orally.

There are two types of chemotherapy, namely systemic chemotherapy and regional chemotherapy. Systemic type is done by injecting or drinking drugs, which then flow through the bloodstream. In the regional type, the drug is directly injected into the part of the body that is affected by cancer cells.

4.Radiation therapy (radiotherapy)

This therapy is carried out using high-power radiation to kill cancer cells. There are two types of radiotherapy, namely internal (injecting radiation fluid into the body) and external (using a radiation-emitting machine from outside the body).

5. Operation (enucleation)

Surgery is performed to remove the eye and part of the optic nerve. Enucleation is the path chosen if the above treatments do not work. Patients whose eyes are removed are usually given false eyes.

After surgery, the patient must undergo further tests for 2 years or more, to prevent the possibility of cancer cells reappearing.

Home remedies

What are some lifestyle changes and home remedies that can be done to treat retinoblastoma?

The following lifestyle changes and home remedies can help you to cope with this disease:

• Remember that the highest incidence of this disease is classified as a hereditary disease. Family members should be examined to find out if there is a risk of developing retinoblastoma.
• Perform routine checks to find out the progress of the disease and your health condition.
• Follow the doctor's instructions.

If you have any questions, consult your doctor to understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.