Retinoblastoma in Asia

Retinoblastoma is an intraocular pediatric cancer that begins in the retina. Asia, the largest continent by area and population, bears most of this burden. Differences in geography, economy, literacy, government programs, infrastructure, and availability of skilled medical personnel can affect the prognosis and outcome of retinoblastoma in different Asian nations. Yet, there is little information regarding the matter. 

Dr. Swathi Kaliki, head of the Ophthalmic, Plastic, and Facial Aesthetic Surgery Department and the Operation Eyesight Universal Institute for Eye Cancer at LVPEI, talks about her latest research paper, which describes the clinical presentation and treatment outcomes of 2112 retinoblastoma patients across 33 Asian countries. 

As a clinician, what are your research interests?
My primary field of expertise is ocular oncology, specifically retinoblastoma. Research is the most effective strategy to deepen our understanding of the disease and improve our current approach, practice patterns, and outcomes for retinoblastoma. 

Does what you see in the clinic impact your choice of research?
Yes, the cases I see influence my research choices, which in turn impacts future cases. For instance, we used to push for chemotherapy treatment in situations of intracranial extension of retinoblastoma, which is when the tumor has progressed into the brain. However, our research data showed that, despite receiving treatment, none of the patients survived. Instead of helping, chemotherapy only led to side effects. Based on that research, we no longer administer chemotherapy in these end-stage cases. Instead, we provide palliative care to ensure the patients' comfort in their final days. 

Can you tell us a little about the Global Retinoblastoma Study Group? What is your role in the group?
The global retinoblastoma study group was formed in 2017. It started off as a collaboration with African physicians who came to LVPEI. Initially, the group consisted of India, the UK, and Africa. With time, the association became an international endeavor that included most retinoblastoma centers worldwide, and it is still expanding today.
When the study group was formed, we selected chief coordinators for different regions of the world. I serve as the chief coordinator for Asia. 

What are some research questions that the group wants to answer?
There are several research questions that the group aims to explore or has explored, the first of which was to assess the presenting patterns for retinoblastoma in different countries. As we collected more data, we also looked at outcomes, referral patterns, gender bias, etc. However, we often group the data together for each continent. So, we wanted to see if there was enough disparity in retinoblastoma outcomes within each continent, especially in a diverse continent like Asia. 

There is a significant difference between East Asia and South Asia when comparing the lag time between the detection of retinoblastoma symptoms and the patient's presentation at a clinic. What are the implications of a short lag time in South Asian countries?
Lag time is the difference between when parents identify retinoblastoma symptoms in their child and when they bring the child to a clinic. However, when the disease symptoms are detected, that is more important. The lag time in East Asia is longer because parents there have the awareness to identify the disease symptoms earlier, and so the region has better outcomes for retinoblastoma. Compare that to South Asia, where parents identify the symptoms too late, and you get a higher mortality rate. 

While 60% of cases in East Asia received an early diagnosis (T1–T2 stages), over half (54%) of retinoblastoma patients in South Asia were discovered at a later stage (T3–T4). Similarly, South Asia has a fourfold increased chance of death as the outcome of retinoblastoma. How can we increase the early diagnosis of retinoblastoma in India?
Awareness is of key importance. Parents need to be aware of retinoblastoma symptoms, recognize them if present in their child, and seek professional medical help at the earliest. Many parents do not even realize that a child can have cancer. So, we must put in the effort to improve awareness of retinoblastoma among the people. Moreover, if a comprehensive eye examination becomes a routine part of every pediatric visit, that could make a significant difference in early detection of retinoblastoma. 

Can genetic screening for RB1 (a tumor suppressor gene) mutations help reduce the incidence of retinoblastoma? What are the factors that keep this method from being widely used in India?
Indeed, it can be quite beneficial, particularly if there is a known family history of retinoblastoma. With genetic testing a couple can know if their future children have a risk of retinoblastoma and plan accordingly.
Genetic testing has been available since the 1970s. Unfortunately, very few centers in Asia have access to genetic testing, even today. The cost and expertise required to perform the test are the two major barriers that limit access. Asia is still at a stage where retinoblastoma treatment is the focus rather than its prevention. 

How can the findings of this study influence India, especially LVPEI’s strategy for improving retinoblastoma outcomes? 
South Asia falls somewhere in the middle when it comes to the best and worst outcomes for retinoblastoma. The main strategy for improving outcomes would be improving awareness among parents and pediatricians, so that it lowers the age of diagnosis. When it comes to treatment, LVPEI is well equipped to deal with the disease. We also have access to RB1 genetic testing. But unless the patient is brought to us early, even state-of-the-art treatment cannot save them. Fortunately, LVPEI has a wide network of secondary and vision centers. With their help it is possible to spread this awareness deep into urban and rural communities. 

What is the future direction of your research?
The current paper is based on data from 2017. Six years (2023) have passed since then, so the next phase would be to see if anything has changed regarding availability of medical resources, clinical presentation of retinoblastoma, and other parameters, especially in regions with poor treatment outcomes. Thus, starting in 2024, we will begin collecting new data. The upcoming project will be a global study, which will include many retinoblastoma centers from Asia. 

Dr. Swathi Kaliki spoke to Sayantan Mitra, Science Writer, LVPEI. Read more about her research here. 

Kaliki S, Vempuluru VS, Mohamed A, Al-Jadiry MF, Bowman R, Chawla B, Hamid SA, Ji X, Kapelushnik N, Kebudi R, Sthapit PR, Rojanaporn D, Sitorus RS, Yousef YA, Fabian ID; Global Retinoblastoma Study Group. Retinoblastoma in Asia: Clinical Presentation and Treatment Outcomes in 2112 Patients from 33 Countries. Ophthalmology. 2023 Oct 13:S0161-6420(23)00757-1  

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