Retinoblastoma: what is it, types, symptoms

THE retinoblastoma is a malignant tumor that affects children and affects the cells of the retina, where the photoreceptors that transform light into impulses that are interpreted by the brain are located. brain like pictures. Eye changes such as white pupillary reflex and strabismus may indicate the problem, so they should not be ignored.

It is important for parents or guardians to be aware of changes in eyes of their children and that they regularly take the children to the eye doctor. Early diagnosis is essential for successful treatment, which varies from patient to patient and may include radiotherapy and chemotherapy.

Know more: What is childhood cancer?

Summary of Retinoblastoma

  • Retinoblastoma is a cancer that affects the retina.

  • It usually occurs before the age of five.

  • It can affect just one eye or both.

  • White pupillary reflex (leukocoria) is considered the most frequent symptom of the disease.

  • Retinoblastoma can be treated. However, an early diagnosis is essential.

  • Treatment varies from one person to another and depends, among other factors, on the stage of the disease.

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What is retinoblastoma?

Retinoblastoma is a type of cancer that affects the retina and can affect one or both eyes. The retina is an essential part of the eye as it is where the photoreceptors are located. These are responsible for receiving light and transforming it into a nerve impulse, which is taken to the brain, where the images are interpreted. Retinoblastoma, if not discovered and treated early, can lead to vision loss and even death.

According to the Ministry of Health, retinoblastoma é the primary tumor nwoe common in children's eyes and usually occurs in early childhood or in infants and may be present from birth. This tumor occurs in two different ways.

→ Hereditary occurrence of retinoblastoma

In hereditary cases of retinoblastoma, the child has a mutation that affects a tumor suppressor gene known as RB1. Usually, the mutation in the gene is inherited from one of the parents. However, it can be a mutation that starts in the individual's body and is then transmitted to offspring.

→ Sporadic occurrence of retinoblastoma

When retinoblastoma is sporadic, the disease usually appears in children over the age of one year, whereas retinoblastoma resulting from a mutation in the RB1 gene develops before this period. Sporadic cases are the result of mutation of a cell, which starts to multiply in an uncontrolled way.

Important: Due to the genetic factor, it is important that families who have a history of this tumor inform the pediatrician and the ophthalmologist about it.

Types of Retinoblastoma

→ Unifocal or unifocal retinoblastoma

Unifocal or unifocal retinoblastoma is one of the types of retinoblastoma, characterized by affecting only one eye. It is often a sporadic form of the disease.

→ Bilateral or multifocal retinoblastoma

Bilateral or multifocal retinoblastoma is one of the clinical forms of the tumor, characterized by affecting both eyes and being almost always hereditary.

→ Trilateral retinoblastoma or PNET retinoblastoma (primitive neuroectodermal tumor)

This type of retinoblastoma only affects children with bilateral hereditary retinoblastoma. According to A. Ç. Camargo Cancer Center, trilateral retinoblastoma occurs when an associated tumor forms in the primitive nerve cells of the brain.

Also know: Lymphoma — a malignant neoplasm that affects the lymphatic system

Symptoms of Retinoblastoma

Retinoblastoma presents as main symptom is leukocoria or cat eye sign. It is a white reflex in the pupil, the result of the incidence of a light source on the tumor, which prevents the passage of light. This reflection can be noticed, for example, when taking a picture using the camera's flash.

In addition to leukocoria, a child with retinoblastoma may have other symptoms, such as:

  • squint;

  • eye pain;

  • low vision;

  • conjunctivitis;

  • redness;

  • exaggerated sensitivity to light (photophobia);

  • deformation of the eyeball.

Staging of Retinoblastoma

Retinoblastoma can remain confined to the eye (intraocular) or reach other parts of the body (extraocular). In general, the diagnosis is usually made while the tumor is still restricted to the eye, therefore, staging systems usually relate only to intraocular retinoblastoma.

We can divide these tumors into five groups according to the International Classification for Intraocular Retinoblastoma, which are groups A, B, C, D and E.

  • In group A are small tumors that are confined to the retina.

  • In group E, the last on the scale, there is an extensive tumor that has spread over a large part of the eye, making it difficult to preserve the structure.

Diagnosis of Retinoblastoma

Child having an examination in an ophthalmologist's office.
Just like adults, children should also make regular appointments with an ophthalmologist.

Early diagnosis of retinoblastoma is critical to successful treatment. For this, it is essential to carry out exams ophthalmic routine in the early years of the child and the careful analysis of his eyes.

Strabismus and the development of a white spot on the iris are warnings that cannot be ignored. The diagnosis of the tumor, however, is made by the doctor, with the aid of analyzes such as fundus examination and ultrasound. Normally, biopsies should not be performed.

See too: Tips for maintaining children's eye health

→ Eye test

It is essential to emphasize the importance oflittle eye test, or red reflex test (RRT), for diagnosing retinoblastoma and other eye problems, such as:

  • cataract;

  • glaucoma congenital.

According to the “Childhood Eye Health Care Guidelines: early detection and intervention for the prevention of visual impairments” of the Ministry of Health,

all newborns must undergo RRT before discharge from the maternity ward and at least two to three times a year in the first three years of life.

So, if the child has not been tested after birth, it is important to see a doctor. In addition, as highlighted in the guidelines, reassessment is essential, even when testing was done in the maternity ward.

Retinoblastoma treatment

Retinoblastoma is a type of cancer that can be cured. The treatment is based on the stage of development of the disease and the general health of the child, being, therefore, a individualized treatment. Some of the techniques that can be adopted are:

  • chemotherapy;

  • radiotherapy;

  • cryotherapy;

  • laser treatment;

  • surgical removal of the eyeball (in extreme situations).

By Vanessa Sardinha dos Santos
Biology teacher

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