Survivors of Retinoblastoma Need Monitoring for Second Cancers

Although survival rates are excellent for children diagnosed with retinoblastoma, new research has shown that survivors are at increased risk of developing medical problems as adults, including second cancers, especially when the disease has affected both eyes.

Danielle Novetsky Friedman, MD

Although survival rates are excellent for children diagnosed with retinoblastoma, new research has shown that survivors are at increased risk of developing medical problems as adults, including second cancers, especially when the disease has affected both eyes.

Previous reports on treatment-related complications in survivors of retinoblastoma—the most common eye tumor of young children—have been limited by small numbers of participants or have focused exclusively on specific problems, such as eye/vision problems or new cancers.

To address this knowledge gap, Danielle Novetsky Friedman, MD, a pediatrician specializing in retinoblastoma at Memorial Sloan Kettering Cancer Center in New York City and colleagues assessed the long-term medical problems of 470 adult retinoblastoma survivors from the Childhood Cancer Survivor Study who were diagnosed between 1932 and 1994. Survivors were compared with 2377 unaffected individuals of similar age, sex, and race.

The investigators found that adult retinoblastoma survivors were at increased risk of developing various medical problems, including second cancers, when compared with unaffected individuals. This increased risk was largely driven by those with retinoblastoma in both eyes, a group that is known to harbor a genetic risk for developing new cancers.

“Novel data presented in this report will guide practice for oncologists, ophthalmologists, survivorship practitioners, and primary care providers involved in the long-term care of survivors of retinoblastoma by helping them identify those at highest risk for medical problems,” said Novetsky Friedman.

“Appropriate lifelong risk-based screening of this population will allow for timely treatment of any medical problems that may arise.”

When the researchers excluded vision problems and new cancers, those with a history of disease in one eye had no increased risk of chronic medical problems when compared with unaffected individuals of similar age, sex, and race. Additionally, the vast majority of retinoblastoma survivors rated their general health as “excellent to good,” indicating that this population continues to maintain comparatively normal health many years after completing therapy.

A related study, also based on an analysis of results from the Childhood Cancer Survivor Study, Peter de Blank, MD, and colleagues at Case Western Reserve University School of Medicine and the UH Rainbow Babies & Children’s Hospital in Cleveland, examined the long-term effects of blindness in children with brain tumors.

The study included 1233 survivors of childhood brain tumors, 22.5% of whom had visual impairment. Vision loss in childhood did not affect psychological outcomes when patients reached adulthood, but blindness in both eyes was associated with an increased risk of being unmarried, living with a caregiver, and being unemployed. More limited vision loss—such as in a single eye—was not clearly associated with negative outcomes.

In about one-third of cases, current chemotherapy treatments improve vision in children with brain tumors involving visual pathways, according to de Blank. Another third will have their vision stabilized, but another third will have their vision deteriorate despite intense treatment.

“We hope that this study will help inform therapeutic decision-making as patients consider the risks and benefits of therapy,” he said. “And for the third of patients whose vision will continue to deteriorate, we hope that this study provides some evidence that for many survivors, the long-term effects of vision impairment do not have to impact their long-term psychological or socioeconomic outcomes.”

The findings, along with a companion editorial, were published in the journal Cancer.1-3

1. Friedman DN, Chou JF, Oeffinger KC, et al. Chronic medical conditions in adult survivors of retinoblastoma: results of the Retinoblastoma Survivor Study [published online ahead of print January 11, 2016]. Cancer.

2. de Blank PM, Fisher MJ, Lu L, et al. Impact of vision loss among survivors of childhood central nervous system astroglial tumors Study [published online ahead of print January 11, 2016]. Cancer.

3. Armenian SH, Bhatia S. Looking back to inform the future: lessons learned from survivors of childhood cancer [published online ahead of print January 14, 2016]. Cancer.