Retinoblastoma: 50 Years of Progress, With More to Be Done

American Academy of Ophthalmology (AAO) 2014

Roger F. Steinert, MD


November 14, 2014

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Hello. I am Dr Roger Steinert, chair of ophthalmology and director of the Gavin Herbert Eye Institute at the University of California, Irvine. I am reporting from the American Academy of Ophthalmology meeting in Chicago.

The highlight of the session on Sunday was the Jackson Memorial Lecture, given by Dr Hans Grossniklaus. His subject was retinoblastoma.

Dr Grossniklaus reminded us that it was 50 years ago that Dr Edwin Dunphy delivered the Jackson Memorial Lecture on retinoblastoma and talked about the four stages that defined retinoblastoma: prehistologic, histologic, enucleation, and treatment (radiation and chemotherapy).

In his lecture, Dr Grossniklaus added three more key steps that have resulted from modern technology. These are molecular biology, targeted treatment, and global health awareness.

He said that the retinoblastoma story continues to evolve. We are now doing a much better job with cytologic features, looking at genetic sequencing and doing clinical stages, chemotherapy reduction, and intra-arterial therapy. All of these have had a dramatic impact on survival rates in retinoblastoma.

A key step in treating retinoblastoma—and a key issue that remains and upon which he focused the most—is global health awareness. There is a lack of dissemination of knowledge and effective treatments.

The survival rate, according to Dr Grossniklaus, for retinoblastoma in the United States is 97%. In Japan, it is higher than 90%; but, in developing countries, it can be as low as 40%. Clearly, we have a lot of work to do in getting the correct treatment to those areas. There are about 1000 cases annually in China and India compared with about 100 cases in the United States.

This is a significant global health problem, and it tragically affects children with this unfortunate disease, which is essentially almost always curable with the proper modern treatment.

On behalf of Medscape, I'm Dr Roger Steinert. Thanks very much for listening.


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