May 31, 2019 Conference


Ocular Adnexal Lymphoma in the Pediatric Population: Clinicopathologic Features and Comparison with Adults

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Dr. Giannis Moustafa, Mass. Eye and Ear (Presenter)

Objective: To investigate the incidence, clinicopathologic features, and survival of ocular adnexal lymphoma (OAL) in the pediatric population and compare these data with adults.

Design: Retrospective cohort study.

Participants: The Surveillance, Epidemiology, and End Results database was accessed to identify individuals with OAL less than or equal to 18 years of age, diagnosed between 1973 and 2015. OAL located in the eyelid, conjunctiva, lacrimal apparatus, and orbit were included. An adult cohort was queried for comparison.

Methods: Age-adjusted incidence rates (AIRs) and descriptive statistics were calculated for comparison of clinicopathologic characteristics. Overall (OS) and cancer-specific (CS) survival were evaluated with Kaplan-Meier curves and compared among subgroups using the log-rank test.

Main Outcome Measures: AIRs per 1,000,000 population at risk, descriptive statistics of clinicopathologic features, OS and CS

Results: The AIR of pediatric OAL was 0.13 (95% confidence interval [CI], 0.09-0.17) per 1,000,000. OAL AIRs showed a higher trend towards pediatric males and Blacks: males 0.17 (95% CI, 0.12-0.25), females 0.08 (95% CI, 0.04-0.13), Whites 0.11 (95% CI, 0.06-0.15), Blacks 0.26 (95% CI 0.14-0.44), Hispanics 0.12 (95% CI, 0.06-0.20), and Asians 0.03 (95% CI, 0.00-0.17). The conjunctiva was the most common site (45.0%), as opposed to adult OAL which originated primarily in the orbit (58.7%). The majority of pediatric OAL were categorized as localized SEER stage (66.7%) at the time of diagnosis. T-cell and lymphoblastic lymphoma comprised 5.0% and 15.0% of pediatric OAL, but only 0.2% and 0% of adult OAL, respectively. Advanced SEER stage, orbital involvement, diffuse-large-B-cell lymphoma, and anaplastic-large-cell lymphoma subtype were associated with increased mortality. In the pediatric cohort, the 5-year OS and CS was 91.0% (95% CI, 79.6%-96.2%) and 92.6% (95% CI, 81.4%-97.2%), respectively. The final OS and CS was 85.7% (95% CI, 71.9%-93.1%) and 89.6% (95% CI, 76.3%-95.7%), respectively. Both OS (p<0.001) and CS (p=0.02) were superior in pediatric individuals compared to adults.

Conclusions: Compared with adults, OAL in the pediatric population is characterized by significant clinicopathologic differences and better OS and CS. These results can assist clinicians in predicting long-term outcomes and in educating patients and their families.