What is Retinoblastoma and its stages?

Retinoblastoma is a kind of pediatric cancer affecting the eye’s retina. The retina is found at the back of the eye and helps in visualization. It has layers of cells that detect light and transmit data to your brain. Retinoblastoma almost always occurs in children under 5 years of age. A combination of therapies is required for the successful treatment of Retinoblastoma, and if detected and treated timely, the child can lead a tumor-free life. Various therapies include Cryotherapy, chemotherapy, radiation, and surgery if required. 

Retinoblastoma is the most common intraocular malignancy, which affects 1 in 15000 to 18000 live births. It has an equal impact on both boys and girls. The condition usually affects only one eye, and about 25 to 30 % of children have both eyes involved. The average age of detection is around 18 months.

Stages Of Retinoblastoma 

Staging is a method used by cancer specialists to classify any form of cancer so that proper and standard guidelines can be followed for treatment and hence prognosis can be explained. The majority of retinoblastoma tumours are discovered before they spread to the body. The majority of the children present with symptoms of either white reflex ( commonly called cat’s eye reflex) or squint. In such tumours, early detection can help in not only salvaging the eye but sometimes vision too.

What causes Retinoblastoma?

Retinoblastoma is caused by a genetic mutation (a change in the child’s genes). Mutation in RB1 causes Retinoblastoma. This mutation causes cells in the eye to increase uncontrolled, resulting in tumor formation. In only 6% of the children, the disease is transmitted from the family. In the rest 94% of newly diagnosed patients, it is sporadic, meaning it is not tranmitted from either of the parents, and the mutation has happened spontaneously. Nonheritable tumours primarily affect one eye and can be cured entirely if diagnosed on time.

How To Diagnose Retinoblastoma?

The most common symptom which parents note in their child is the white reflex which is also popularly called the cat’s eye reflex is noted by the parents. Other common symptoms include a squint in the eye followed by redness, pain, poor vision, etc. A specialist eye examination is required to identify Retinoblastoma. The child is examined under anesthesia ( in which the child will be sedated so that the proper size and location of the tumor is determined), and the treatment guideline is made accordingly. The doctor will advise further investigation, like imaging which includes an eye ultrasound and, if required, an MRI scan of both the eye and brain to further evaluate the spread of the disease. After that tumor is grouped under the international retinoblastoma classification, and a further course of treatment is decided. 

Retinoblastoma Treatment 

Retinoblastoma Treatment depends on the stage of the disease. Your child’s medical team will assess if the cancer is limited to the eye (intraocular Retinoblastoma) or whether it has spread to other places of the body (extraocular Retinoblastoma). Treatments for Retinoblastoma include: 

Chemotherapy involves the administration of medicines through blood vessels which prevent the further proliferation of cancer cells. This can take a few months for the course to get completed. 
 
Cryotherapy (cryoablation): Cryotherapy is a localized treatment used when the tumor is limited inside the eye. 
 
Photocoagulation and thermotherapy are examples of laser techniques used in localized tumors when limited intraocularly. 
 
Radiation therapy kills cancer cells and prevents them from replicating. These days, oncologists use plaque radiotherapy, which only gives radiation to a particular area and prevents side effects on other body parts.  

Intraarterial chemotherapy: This is a recently added therapy for treating large tumors that earlier oncologists used to remove the affected eyeball. Here localized chemo drugs are given to the affected part, which not only helps save the eye but also prevents the whole body from the side effects of chemo drugs. 

Large tumours may still necessitate the removal of the entire eyeball and a portion of the optic nerve beneath the eyeball. This procedure is known as enucleation. 
 
Overall, a cancer diagnosis is a life-changing event. CFS healthcare providers provide treatments that enhance the outlook for patients suffering from this type of cancer.

Why CFS?

We have a team of dedicated and experienced ophthalmologists at CFS that will provide you with the best treatment and experience possible. We have been in the eyecare industry for a long time and have successfully treated our patients’ diverse eye ailments. Keep in touch with CFS to know more about it.

Article: What is Retinoblastoma and its stages?
Author: CFS Editorial Team   |   July 21 2022 | UPDATED 12:37 IST
*The views expressed here are solely those of the author in his private capacity and do not in any way represent the views of Centre for Sight.