Incidence and Survival Rates of Primary Ocular Lymphoma: A Population-Based Study : The Asia-Pacific Journal of Ophthalmology

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Scientific Correspondence

Incidence and Survival Rates of Primary Ocular Lymphoma: A Population-Based Study

Dibas, Mahmoud MD; Alkalaf, Mustafa MD

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Asia-Pacific Journal of Ophthalmology ():10.1097/APO.0000000000000554, October 24, 2022. | DOI: 10.1097/APO.0000000000000554

Primary ocular lymphoma (POL) is a rare lymphocytic malignancy of predominantly B-cell origin of primary central nervous system.1 It represents 4% to 6% of all primary central nervous system tumors and 1% to 2% of extranodal lymphomas.2,3 The tumor can arise from any part of the eye: retina, uvea, vitreous, Bruch membrane, and optic nerve.1,3

Although this type of tumors is rare, its incidence has increased over the past years.3 Recent studies have reported an increase in the incidence of POL from 0.027/100,000 in 1973 to 1/100,000 in the early 1990s in the United States.1,3,4 The increase in the incidence has been hypothesized to be linked to the increased number of immunosuppressed and immunodeficient individuals, increased life expectancy, and improvement of diagnostic tools.1,4 Few reports studied the prognostic factors of the tumor.2,5 They found that individuals aged ≥60 years, diffuse large B-cell type lymphoma, and nonconjunctival tumors were all associated with poorer survival rate.2,5 Liu and Zheng5 used the Surveillance, Epidemiology, and End Results (SEER) database for the period between 1973 and 2014 to study POL. They found that the incidence was 0.48/100,000 individuals. Moreover, the 10-year overall survival for patients aged ≥60 years compared with younger patients was 36.2% and 75.8%, respectively.5 We aimed in this study to explore the updated incidence and survival rates of POL.

This was a secondary analysis of publicly available data from the SEER database (2000–2018). Therefore, ethical approval was waived for this study. The age-adjusted incidence rate (AAR) was calculated for the total population, and based on age, sex, race, ethnicity, year of diagnosis, and histology. Furthermore, the overall survival rates were calculated for the total population and based on the selected population characteristics.

Table 1 summarizes the patients’ AAR. Out of the 3437 patients, 1518 were males (44.2%) and 1919 were females (55.8%), with an overall AAR of 0.206/100,000 individuals. When stratifying the AAR by year, we noticed a slight, stable reduction over the recent years (0.206 in 2000 to 0.167 in 2018, Supplementary Digital Content Table 1, Similarly, we observed a stable reduction in the AAR in 2000 to 2008 as compared with 2009 to 2018 (0.235 vs 0.184). While the majority of individuals were under 60 years of age (38.6%), individuals aged ≥70 years had a higher AAR (0.92 vs 0.094 per 100,000). The AAR was higher in Asian or Pacific Islander (0.248) and White patients (0.202) as compared with Black (0.15) and American Indian/Alaska Native patients (0.078). Conjunctival tumors accounted for 941 cases (27.4%) with an AAR of 0.057/100,000. Stratifying by histology, we found that mucosa-associated lymphoid tissue lymphoma was the most reported type (55.8%) followed by diffuse large B-cell type lymphoma, (12.3%) with an AAR of 0.115 and 0.025 per 100,000, respectively (Supplementary Digital Content Table 2, We found that old age, race, and tumor site influenced survival rates, with individuals <60 years of age achieving 96.9% 5-year survival rate compared with 91.1% and 88.6% for individuals between 60 and 69 and 70 and older, respectively (Supplementary Digital Content Table 3, Asian or Pacific Islander patients had the least 5 years survival rates. The conjunctival tumors had a 100% 5-year survival rate compared with other tumor sites which ranged from 54.3% to 96.8%.

TABLE 1 - The Age-Adjusted Incidence Rates of Primary Ocular Lymphoma and Rates Stratified to Selected Characteristics, SEER Database Between 2000 and 2018
Count %* Rate SE
Total 3437 100 0.206 0.004
Age group (y)
 <60 1327 38.609 0.094 0.003
 60–69 818 23.8 0.585 0.02
 ≥70 1292 37.591 0.92 0.026
 Male 1518 44.166 0.199 0.005
 Female 1919 55.834 0.212 0.005
 White 2692 78.324 0.202 0.004
 Black 265 7.71 0.15 0.009
 American Indian/Alaska Native 14 0.407 0.078 0.023
 Asian or Pacific Islander 390 11.347 0.248 0.013
 Unknown 76
 Hispanic 440 12.802 0.202 0.01
 Non-Hispanic 2997 87.198 0.207 0.004
Primary Site
 C69.0-Conjunctiva 941 27.379 0.057 0.002
 C69.1-Cornea, NOS 5 0.145 0 0
 C69.2-Retina 10 0.291 0.001 0
 C69.3-Choroid 28 0.815 0.002 0
 C69.4-Ciliary body 59 1.717 0.004 0
 C69.5-Lacrimal gland 434 12.627 0.026 0.001
 C69.6-Orbit, NOS 1720 50.044 0.103 0.003
 C69.8-Overlapping lesion of eye and adnexa 17 0.495 0.001 0
 C69.9-Eye, NOS 223 6.488 0.013 0.001
*Percent is for total cases.
Rates are per 100,000 and age-adjusted to the 2000 US standard population.
NOS indicates not otherwise specified; SE, standard error; SEER, Surveillance, Epidemiology, and End Results.

In this study we observed a slight, stable reduction in the AAR of POL when compared with other studies.4,5 We assume that the increased adoption of the highly active antiretroviral therapy for human immunodeficiency virus positive patients might explain this trend.6 In regards to overall survival, our findings are conforming to that of previous published studies.5 Older age, Asian or Pacific Islander race, and nonconjunctival tumors were associated with reduced 5-year survival. Interestingly, our population had a larger female-to-male ratio when compared with other studies.2,5

In summary, the AAR for POL had a stable reduction over the past years. In addition, we found that survival of POL was affected mostly by old age, Asian or Pacific Islander race, and tumor site.


1. Tang LJ, Gu CL, Zhang P. Intraocular lymphoma. Int J Ophthalmol. 2017;10:1301–1307.
2. Raval V, Binkley E, Aronow ME, et al. Primary central nervous system lymphoma—ocular variant: an interdisciplinary review on management. Surv Ophthalmol. 2021;66:1009–1020.
3. Sagoo MS, Mehta H, Swampillai AJ, et al. Primary intraocular lymphoma. Surv Ophthalmol. 2014;59:503–516.
4. Woolf DK, Ahmed M, Plowman PN. Primary lymphoma of the ocular adnexa (orbital lymphoma) and primary intraocular lymphoma. Clin Oncol (R Coll Radiol). 2012;24:339–344.
5. Liu DL, Zheng ZJ. Survival in B-cell primary ocular lymphoma 1997-2014: a population-based study. J Investig Med. 2018;66:1133–1140.
6. Goldberg DE, Smithen LM, Angelilli A, et al. HIV-associated retinopathy in the HAART era. Retina. 2005;25:633–649.

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