Retinoblastoma Diagnosis and Treatment

What is Retinoblastoma?

Retinoblastoma is a rare malignant tumor of the retina. It occurs in both hereditary and non-hereditary forms and is usually seen in children under 5 years of age.

Retinoblastoma: How to Recognize It?

What are the signs of retinoblastoma?

The diagnosis of retinoblastoma is clinical. The most characteristic symptom is a whitish reflex in the pupils of the affected eye in children. This is often detected in flash photographs, where the pupils appear white instead of the normal orange-red glow. This is called leukocoria or cat's eye pupil.

Other symptoms include strabismus, or crossed eyes. Parents may notice this by covering each eye in turn while the child looks at an object.

Some children experience eye redness and pain. This pseudo-inflammation is seen in about one-tenth of patients. Other less frequent symptoms include: swelling of the eyeball; discoloration of the iris in the affected eye; differences in the size, shape, and activity of the affected pupil; and symptoms due to metastases such as bone pain. Visual acuity may be affected, or the child may experience double vision.

Confirmation of Retinoblastoma Diagnosis

Indirect ophthalmoscopy is necessary to confirm the diagnosis. The examination is performed with dilated pupils, and anesthesia is required for very young children and infants. The interior of the eyeball, including the entire retina and optic nerve head, must be carefully visualized.

A slit-lamp examination (biomicroscopy) is also performed using a powerful focused light beam and a microscope.

Other eye tests may be requested, such as:

• Fluorescein angiography: A dye (fluorescein) is injected into the eye's blood vessels to identify blocked, dilated, or leaking vessels on an X-ray film;
• Ocular ultrasonography: Ultrasound waves create an image of intraocular tissues;
• Magnetic resonance imaging (MRI): Provides a detailed image of soft tissues, including tumor spread to neighboring tissues;
• Computed tomography (CT): Uses X-rays from different angles to create a composite image. It's often avoided in young children, especially with a family history of retinoblastoma or other tumors, due to radiation exposure risks;
• A complete physical examination is performed to detect related or unrelated diseases. A bone scan may be necessary to check for bone spread;

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Retinoblastoma Treatment

Treatment depends on:

• the tumor's size;
• the tumor's location;
• the extent of intraocular and extraocular involvement;
• family history of retinoblastoma;
• the potential for vision preservation;

Retinoblastoma treatment requires a team of healthcare providers experienced in childhood cancer. The goals are to prevent death from cancer, save the child's eye, and minimize side effects.

Treatment Approaches

Cryotherapy

Cryotherapy uses a cryoprobe to freeze and destroy tumors up to 5 mm in diameter and 3 mm in thickness. This can be repeated at 3-4 week intervals.

Laser Photocoagulation

Laser photocoagulation uses laser light energy to heat and destroy tumor blood supply, rendering them non-viable or making them more sensitive to other ablation techniques.

Thermotherapy

Microwaves are used to heat and destroy tumor cells.  

Radiation Therapy and Brachytherapy

Radiation therapy includes external beam irradiation and brachytherapy. External beam radiation therapy is more precise, sparing surrounding tissues and preserving vision. Modalities include intensity-modulated radiation therapy (IMRT), stereotactic external beam radiation therapy (gamma knife), and proton beam radiation therapy.

Brachytherapy uses plaque radiotherapy, where radioactive metals (e.g., I-125) are placed on a plaque sutured to the sclera. The plaque remains in place for 3-7 days, depending on tumor size and extent.

Side Effects of Retinoblastoma Treatment

Late effects include:

• Radiation effects on facial growth (midfacial hypoplasia), hearing, and vision;
• Cognitive and mood effects;
• Second cancers (lung, bladder, osteosarcoma, sarcoma, melanoma) are common in hereditary retinoblastoma;

Prognosis

Nine out of ten children with retinoblastoma are cured, with no recurrence within five years of treatment. Lifelong follow-up is mandatory due to the high risk of second tumors and tumor development in the other eye (usually within the first three years). Eye exams every 2-4 months for at least 28 months are recommended. The risk of trilateral retinoblastoma is high in hereditary forms, requiring MRI scans every six months until the child is at least five years old.

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