Background: Ocular-adnexal non-Hodgkin lymphoma (OANHL) is a rare lymphoid malignancy, accounting for only 1–2% of all non-Hodgkin lymphomas. Its development has been associated with endemic infections, such as Chlamydia species. However, data on OANHL remain limited, even in subtropical regions where these infections are more prevalent. Our aim was to describe the sociodemographic, clinical, and pathological characteristics of this neoplasm in Hispanic-Latino population. As a secondary objective is to compare the main characteristics according to the aggressiveness.

Methods: We conducted a retrospective cohort study including all cases of ocular-adnexal NHL diagnosed and treated at a tertiary institution between 2000 and 2017. Descriptive statistics were used to describe the population according to the tumor aggressiveness. Kaplan-Meier curves were used to estimate survival rates. Cox regression analyses were used to identify the prognostic factors of event-free (EFS) and overall survival (OS).

Results: A total of 106 patients were included in the study. The mean age was 58.8 years (± 17.3), and 51.9% were female. The mean time since diagnosis was 8.5 months (± 6.0), and 51.9% of patients presented with proptosis. The most common tumor location was the orbit (41.5%), followed by the eyelid (31.1%) and the conjunctiva (26.4%). When classified according to aggressiveness, 58.5% and 41.5% were indolent and aggressive, respectively. Most patients were diagnosed at Clinical Stage I (71.7%), with 17.9% at Stage II and 7.5% at Stage III. Most patients presented with an International Prognostic index of 1 (37.7%), followed by 0 (16.0%) and 2 (16.0%). Tumor size exceeded 2 cm in 48.1% of cases, while 8.5% showed bilateral involvement. A majority had a good performance status (ECOG 0–1: 87.8%), and 8.5% reported B symptoms. Regarding treatment, 4.7% received antibiotics, 6.6% underwent surgery, 29.2% received chemotherapy, 23.6% had radiotherapy, and 11.3% were treated with combined chemoradiotherapy. In contrast, 35.8% did not receive any local or systemic therapy. The most commonly used chemotherapy regimen was CHOP (27.1%). The most frequent toxicity was conjunctivitis (3.8%), followed by cataracts and keratitis (1.9%). Following treatment, 34 patients (32.1%) achieved a complete response to chemotherapy. During follow-up, 13.2% experienced local recurrence, and 3.8% had distant relapse.

When comparing indolent versus aggressive ocular NHL, indolent tumors were more common in women (59.7% vs. 40.9%; p = 0.057). Aggressive tumors were more likely to be located in the eyelid (47.7% vs. 19.4%; p = 0.007) and more often exceeded 2 cm in size (77.1% vs. 46.2%; p = 0.004). Patients with aggressive disease also had worse functional status (ECOG ≥ 2 in 22.7% vs. 4.8%; p = 0.006), although there were no differences between groups in clinical stage or B symptoms. Treatment modalities differed significantly: no difference in surgical rates, but aggressive tumors were more likely to be treated with chemotherapy alone (47.7% vs. 16.1%; p < 0.001) or chemoradiotherapy (18.2% vs. 6.5%; p < 0.001), whereas indolent tumors received radiotherapy more frequently (35.5% vs. 6.8%; p < 0.001). Rates of pathologic complete response to chemotherapy did not differ significantly (indolent vs. aggressive: 57.5% vs. 42.3%, p = 0.271).

With a median follow-up of 20.2 years, the 5-year OS was 77.3%, and the event-free survival EFS was 59.2%. For indolent lymphomas, the 5-year EFS and OS were 73.1% and 91.9%, respectively; in contrast, aggressive subtypes had significantly lower rates, with 40.8% EFS and 56.6% OS at 5 years (p < 0.01). Multivariate cox regression analysis showed that prognostic factors of worse EFS were aggressive subtypes (HR=2.965, 95%CI: 1.471-5.977, p=0.002), Stage III vs. I (HR=3.351, 95%CI: 1.200-9.358, p=0.021), while radiotherapy and chemotherapy (HR=0.245, 95%CI: 0.081-0.842, p=0.026) was a factor of better EFS. Prognostic factors for worse OS were greater age (HR=1.032, 95%CI: 1.008-1.056, p=0.010) and aggressive subtype (HR=2.884, 95%CI: 1.399-5.947, p=0004).

Conclusions: The ocular-adnexal NHL has unique characteristics among the Latin-Hispanic population. Patients are diagnosed at greater stages. The EFS and OS rates were worse than other high- and low-middle income countries. There is an urgent need for further guidelines for standardized treatment among patients affected with this neoplasm.

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2025
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