Primary uveal diffuse large B-cell lymphoma: A population-based study

Primary involvement of the uveal tract by diffuse large B-cell lymphoma is exceedingly rare and poorly characterized. Most reported ocular lymphomas in this location are indolent mucosa-associated lymphoid tissue lymphomas with favourable outcomes. We aimed to evaluate the epidemiologic and clinical features of primary uveal diffuse large B-cell lymphoma using a large population-based dataset.

We performed a retrospective analysis of cases recorded between 2000 and 2022 in the Surveillance, Epidemiology, and End Results (SEER) database. Eligible patients had histologically confirmed diffuse large B-cell lymphoma with primary localization in the choroid (C69.3) or ciliary body/iris (C69.4). Localized primary uveal diffuse large B-cell lymphoma was defined as Ann Arbor stage I disease without systemic involvement. Survival outcomes were analysed using Kaplan-Meier estimates and Cox univariate models.

Among 134,366 diffuse large B-cell lymphoma cases, 51 involved the uveal tract (0.038 %), with 42 (82.4 %) classified as localized primary uveal diffuse large B-cell lymphoma. Median age was 65 years. The 5-year overall survival and cancer-specific survival rates were 45.5 % and 58.4 %, respectively. For patients with localized diseases, 5-year cancer-specific survival rate was 59.4 %, with a survival plateau beyond 6 years. No significant associations were found between overall- or cancer-specific survival and clinical or treatment variables, including radiotherapy or chemotherapy.

Primary uveal diffuse large B-cell lymphoma is an ultra-rare and aggressive malignancy, distinct from uveal mucosa-associated lymphoid tissue lymphoma. While outcomes are poorer, a subset of patients with localized disease may achieve long-term remission. Radiotherapy may be a treatment alternative for selected patients, though optimal management remains undefined. Further prospective studies are warranted.