Abstract
Purpose :
To describe the clinical characteristics, treatment, and outcomes of patients with primary orbital lymphoma.
Methods :
Single center retrospective chart review of patients with biopsy-confirmed primary orbital lymphoma at Mayo Clinic from 1/1/1990 to 11/30/2020. Charts were reviewed for demographics, presenting symptoms, location, Ann Arbor Stage, treatment, and outcomes.
Results :
There were 127 biopsy-confirmed cases of primary orbital lymphoma, with 81 (63.3%) female and 111 (88.2%) white. The average age at initial diagnosis was 61.6 years +/-15.3. There were 27 (21.3%) cases with bilateral orbit involvement. The most frequent lymphoma subtype was MALT lymphoma (N=74, 59.2%), followed by diffuse large B-cell lymphoma (N=18, 14.4%) and follicular lymphoma (N=12, 9.6%). Most cases were Ann Arbor Stage I (N=89, 63.0%), followed by Stage IV (N=18, 14.2%). There were 6 (4.7%) patients with Stage II and 6 (4.7%) with Stage III. There were 25 (19.7%) patients with distant metastasis by PET-CT. For all lymphoma subtypes, clinical presentation most frequently involved the orbital soft tissue (N=81, 64.8%), followed by lacrimal gland (N=22, 17.3%) and conjunctiva (N=16, 12.6). After diagnosis 88 (69.3%) patients received radiation and 35 (27.6%) had systemic chemotherapy. At 60 months from biopsy, 83.5% of patients were alive, with 3.1% dead from lymphoma-related causes, 3.9% dead from other causes, and 9.4% lost to follow-up. Eleven (9.1%) patients had recurrence, of whom 2 (1.7%) had a second recurrence. Orbital recurrence occurred most frequently in patients with diffuse large B-cell lymphoma (N=3, 16.7%) and follicular lymphoma (N=2, 16.7%). Most recurrences occurred in patients treated with local radiation (N=8, 10.3%) rather than chemotherapy (N=1, 5.9%). The mean best corrected logMAR visual acuity (BCVA) in the affected eye was 0.156 (SD=0.232), and 79 (62.2%) of cases had BCVA within 1 line of baseline or better at an average of 9.4 years (SD 7.6 years) of follow up.
Conclusions :
In this single center academic cohort, primary orbital lymphoma most frequently involved the orbital soft tissue, and MALT lymphoma subtype was most common. Orbital recurrence rate was low and was less frequent in those treated with chemotherapy. Long-term follow-up data showed that the majority of patients retained good visual acuity in the affected eye.
This abstract was presented at the 2024 ARVO Annual Meeting, held in Seattle, WA, May 5-9, 2024.