Background: Vitreoretinal lymphoma (VRL) is an unusual presentation of primary central nervous system lymphoma and a subcategory of intraocular lymphoma (IOL). The etiology and epidemiology of this rare malignancy is unknown.

Methods: We diagnosed 10 subjects with VRL in our clinical practices in New York City (NYC) during 2010-2013. Upon systematic collection of demographic and exposure history, 6 patients reported residence in regions proximal to the Chernobyl nuclear disaster (4 Ukraine, 1 Poland, 1 Moldova). We studied clinical variables including presentation, diagnostic parameters, therapies and survival of this case series. To provide context, we examined population-based incidence data from the Surveillance, Epidemiology and End Results (SEER) program to determine age-adjusted incidence rates of IOL (there is no unique code for VRL) per 100,000 person-years in the United States (US) during 1992-2013. New York State (NYS) rates are not reported to SEER and were examined separately using NYS Cancer Registry (NYSCR). Incidence trends using 10-year intervals (1992-2002 and 2003-2013) were also analyzed.

Results: All six subjects (5 females) were diagnosed with diffuse large B-cell lymphoma with bilateral presentation. Diagnosis in all cases was made by immunohistochemical analysis on tumor specimen obtained by vitreous fine needle aspiration, vitreous biopsy or stereotactic brain biopsy. Median age at diagnosis was 76 years (range, 62-84 years). Median interval from Chernobyl accident (1986) to diagnosis was 26 years (range, 24-27 years). We identified no other common exposure or environmental risk factor. There were 68 cases of primary IOL during 1992-2013 in the 13 SEER areas. IOL rates per 100,000 person-years for both sexes were highest among Asian/Pacific Islanders (0.010 for males and 0.014 for females) followed by whites (0.009 for males and 0.008 for females) and (based on small numbers) by blacks. Analysis of incidence trends showed an increase in rates among Asian/Pacific Islander females from 0.005 in 1992-2002 to 0.018 in 2003-2013 and among males from 0.006 to 0.013. Rates among whites increased for both genders. Analysis of NYSCR data revealed 44 cases of primary IOL. IOL rates per 100,000 person-years for both genders were highest among whites (0.010 for males and 0.012 for females) followed by Asian/Pacific Islanders (0.007 for males and females). Analysis of trends revealed increase in rates for both genders and racial groups.

Conclusions: Our analyses highlight the rarity of VRL and reveal an increase in incidence, particularly among Asian/Pacific Islanders where IOL rates tripled within two decades. Based on incidence rates, 8 cases of IOL (with a subset being VRL) are expected in 4 years in NYS. Our observation of 10 cases of VRL in 4 years in our practices in NYC is unanticipated. The sole common environmental factor we identified was residence in regions proximal to the Chernobyl nuclear disaster.

Citation Format: Sanford Kempin, Paul Finger, Robert Gale, John Rescigno, Jeffrey Rubin, Walter Choi, Rebecca Fisher, Alexander Aizman, Ilona Genis, Roxana Moslehi* (Corresponding Author). A case series of vitreoretinal lymphoma in New York City with exposure to Chernobyl nuclear disaster decades earlier [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2017; 2017 Apr 1-5; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2017;77(13 Suppl):Abstract nr 259. doi:10.1158/1538-7445.AM2017-259