Every year, nearly 1,500 children in India develop retinoblastoma, an eye cancer that begins in the retina and affects young children. Speaking with Kavita Bajeli-Datt, Dr Manas Kalra, senior pediatric oncologist at Sir Ganga Ram Hospital & Dr Sima Das, director of oculoplasty and ocular oncology at Dr Shroff’s Charity Eye Hospital, said diagnosis should not be delayed as this disease is highly curable under multidisciplinary care.
What is retinoblastoma?
Retinoblastoma is a type of childhood cancer seen in children under 4-5 years of age, arising from the retina. This can run in families (genetic disorders) without any genetic background. If a close family member is affected with retinoblastoma, there is a 50% chance of getting affected by this cancer. In India, approximately 1,500 children develop this cancer every year.
How can parents find out if their child is affected by retinoblastoma?
Parents should report to an eye specialist/eye cancer specialist/paediatrician/ pediatric oncologist if they notice their child’s eye has a whitish glow in the middle of the eyeball (pupil), sometimes called a cat’s eye reflex. They may notice this in their child’s photograph. Parents may also see a recent onset squint, decreased vision, or bulge in the eyeball. Any such finding should prompt a thorough and immediate check-up. If we identify this cancer in a child, their siblings should also be evaluated for this condition. Late presentation of this cancer is very common in developing countries. In the West, these children are diagnosed much earlier, and their outcomes are much superior.
In India, children are presented very late to the specialist when the disease spreads outside the eye and to other body parts. Much needs to be done to increase awareness among paediatricians, general physicians and ophthalmologists to pick this disease early and refer to appropriate centres.
How is this condition treated?
This cancer is highly curable. It is treated under the multidisciplinary care of an eye cancer specialist, a pediatric oncologist, a radiation oncologist and a geneticist. An ocular oncologist is the leading facilitator in treating a child with retinoblastoma. It is usually treated with laser and chemotherapy in the early stages. The child will require frequent examination of their retinas under short general anaesthesia. The treatment is gentle and well-tolerated by most children. If the diagnosis is delayed, then the consequences can be devastating. Saving the eye and saving the life may become complicated if the child presents late to the treating team. If the child has no helpful vision by the time they consult a doctor, eye removal is done. A cosmetic prosthesis can then be put in.
Most people are scared of chemotherapy. They want to avoid chemotherapy for children. Is chemotherapy very toxic?
Chemotherapy does have side effects. But most of these side effects are transient. Nausea, vomiting, hair loss, drop in blood counts, changes in nails, skin, etc, are some common side effects. Chemotherapy helps kill cancer cells and prevents the spread of cancer in the body. Local therapies for eye and chemotherapy play an integral role in managing these in children. The risk of untreated or partially treated cancer is much more than the side effects of chemotherapy.
What are the new therapies in this field?
Newer treatment modalities like specialized chemotherapy delivery like intra arterial chemotherapy and plaque brachytherapy can also help treat and save some children’s eyes with advanced cancers.
Do these children require long-term follow-up after completion of therapy?
Yes, these kids need to be followed up for a long time after completion of therapy. These children are at risk of relapse, which means cancer recurrence. They also need to be followed up as this disease may develop in the other eye or the eyes of their siblings. These children will need follow-up to rehabilitate them in society, which is now becoming more accessible with advancements in treatment and the availability of suitable cosmetic prostheses. They also need follow-up for screening them for late effects of chemotherapy. Children with genetic variants of retinoblastoma are also at risk of other types of cancers, and early detection helps to improve cure rates.