Author: Dr. Ella Bowles

As a 35-year-old patient who was treated for retinoblastoma that many years ago, I am curious how retinoblastoma treatment has changed over the years? I had bilateral retinoblastoma, and one of my eyes was removed; in the other, I had xenon arc (an older type of laser) therapy and cryotherapy. At that time, external beam radiation and radioactive patches were also common retinoblastoma treatments. I know that these therapies have been fine-tuned since then, but I don’t know much more than that. To answer my questions, I sat down with Dr. Brenda Gallie to talk about how care has changed over the years, and what future directions may look like.

Me: How has the field of retinoblastoma changed over the last 30 or so years?

Dr. Gallie: The field of retinoblastoma has changed a lot. Now it is totally feasible for all 8000 children who have retinoblastoma in the world to live, and DePICT is one of the tools at the center of this possibility. Retinoblastoma could be a zero-death cancer in the next ten years. DePICT is a digital tool where all patient information, including all treatments, can be archived, and patients have the ability to access it. DePICT also provides tremendous opportunity for research, as it will preserve a lot of information that is lost in the current patient record system.

Major developments in retinoblastoma treatment include intra-arterial chemotherapy (chemotherapy delivered to the eye via ophthalmic artery) and intra-vitreal chemotherapy (injection of chemotherapy into the eye) in 2010 and 2011, as well as removal of the tumor and vitreous from inside the eye (2017). These new therapies are changing how eyes are treated, and the DePICT^RB  database will allow clinicians and researchers to assess if the treatments are better or worse, how they are better, and for which patients they are appropriate.

In addition, a new staging system for retinoblastoma (TNMH stage vs the Murphree A-E classification) was developed from outcome data on 1,728 eyes in 13 countries, to help all doctors ‘speak the same language’ about retinoblastoma and compare patients across centres.

A child now diagnosed with:

1. T3 eye: eye must come out

2. T1a eye: treat with laser, cryotherapy

3. T1b eye: treat with chemotherapy then laser, cryotherapy

4. T2a eye (retinal detachment with no seeding): treat with chemotherapy then laser, cryotherapy

5. T2b eye (any subretinal or vitreous seeding): treat first with chemotherapy, then injection of chemotherapy directly into the vitreous, and the new idea to surgically remove the tumor in some eyes when other treatments fail. We think that vitreous seeds may be easier to treat than subretinal seeds, and we will know once we have complete data in the DePICT^RB

Intra-arterial chemotherapy in particular has swept the world without evidence of outcomes. It definitely has an important role, and we look forward to full data on the outcomes of all children who receive intra-arterial chemotherapy, so we can know which children it serves well and which children do better with systemic chemotherapy.

Me: How much involvement do patients usually have in treatment decisions for their kids?

Dr. Gallie: Each treatment is a discussion, and every treatment is agreed to by the parents of patients. What clinicians need to do better is showing the parents the outcomes of each choice, for example how far to go to save a blind and dangerous eye.

Me: Where is the best place for patients and families to go to get information about treatment?

Dr. Gallie: World Eye Cancer Hope, which can be found here: https://wechope.org.

As a patient, I feel invested in the implementation of DePICT, Dr. Gallie’s passion project, perhaps for the same reasons as she is. I was treated 35 years ago, and since then I have moved many times, with my family, to go away to school, and now for work. Over all those moves, so much information about my treatment has been lost – well, it could be found, but it is locked in clinic basements in files, and nobody wants to go look for it. All I have is a series of letters. DePICT will allow for continuity of care, and for data about treatment and follow up to be retained so easily. As a patient, I also value the possibility that it will allow easy discussion among physicians about treatment in cases where collaboration on care would be beneficial. And lastly, as a patient, I think it would be beneficial for researchers to have comprehensive data for retrospective studies. For example, Dr. Gallie spoke of how intra-arterial chemotherapy has swept the world as a treatment without evidence that it is better. DePICT can provide the data to test that. Other examples of retrospective studies are: a lot is not yet understood about why second cancers arise, whether there may be particular RB1 mutations that predispose to second cancers, or what types of consequences arise from specific treatments. DePICT holds promise to facilitate all of this.