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Original Investigation | Epidemiology

Retinoblastoma Incidence Patterns in the US Surveillance, Epidemiology, and End Results Program

Jeannette R. Wong, MPH1; Margaret A. Tucker, MD2; Ruth A. Kleinerman, MPH1; Susan S. Devesa, PhD3
[+] Author Affiliations
1Radiation Epidemiology Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, Maryland
2Human Genetics Program, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, Maryland
3Biostatistics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, Maryland
JAMA Ophthalmol. 2014;132(4):478-483. doi:10.1001/jamaophthalmol.2013.8001.
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Importance  Several studies have found no temporal or demographic differences in the incidence of retinoblastoma except for age at diagnosis, whereas other studies have reported variations in incidence by sex and race/ethnicity.

Objective  To examine updated US retinoblastoma incidence patterns by sex, age at diagnosis, laterality, race/ethnicity, and year of diagnosis.

Design, Setting, and Participants  The Surveillance, Epidemiology, and End Results (SEER) databases were examined for retinoblastoma incidence patterns by demographic and tumor characteristics. We studied 721 children in SEER 18 registries, 659 in SEER 13 registries, and 675 in SEER 9 registries.

Main Outcomes and Measures  Incidence rates, incidence rate ratios (IRRs), and annual percent changes in rates.

Results  During 2000-2009 in SEER 18, there was a significant excess of total retinoblastoma among boys compared with girls (IRR, 1.18; 95% CI, 1.02 to 1.36), in contrast to earlier reports of a female predominance. Bilateral retinoblastoma among white Hispanic boys was significantly elevated relative to white non-Hispanic boys (IRR, 1.81; 95% CI, 1.22 to 2.79) and white Hispanic girls (IRR, 1.75; 95% CI, 1.11 to 2.91) because of less rapid decreases in bilateral rates since the 1990s among white Hispanic boys than among the other groups. Retinoblastoma rates among white non-Hispanics decreased significantly since 1992 among those younger than 1 year and since 1998 among those with bilateral disease.

Conclusions and Relevance  Although changes in the availability of prenatal screening practices for retinoblastoma may have contributed to these incidence patterns, further research is necessary to determine their actual effect on the changing incidence of retinoblastoma in the US population. In addition, consistent with other cancers, an excess of retinoblastoma diagnosed in boys suggests a potential effect of sex on cancer origin.

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Figure 1.
Trends in Retinoblastoma Incidence Rates by Race/Ethnicity

Individual data points are the incidence rates by year, shaded according to the number of cases diagnosed; slopes of the lines portray the annual percent change. A, White non-Hispanics (data are for whites only for Surveillance, Epidemiology, and End Results [SEER] 9 because Hispanic ethnicity is not available before 1992); B, white Hispanics; C, blacks; and D, Asian/Pacific Islanders (data on Asian/Pacific Islander race are not available as a separate group in SEER 9).

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Figure 2.
Trends in Retinoblastoma Incidence Rates Among White Non-Hispanics

Individual data points are the incidence rates by year, shaded according to the number of cases diagnosed; slopes of the lines portray the annual percent change. Data are presented for whites only for SEER 9 because Hispanic ethnicity information is not available before 1992. A, Diagnosed at younger than 1 year; B, diagnosed at 1 to 4 years old; C, unilateral disease; and D, bilateral disease.

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