Data Provide Updates on Ocular Lymphoma Treatment, Survival-Associated Factors

During an oncology session at the American Society of Ophthalmic Plastic & Reconstructive Surgery 2021 Fall Scientific Symposium, experts presented new research on orbital and conjunctival lymphomas.

Chelsea Reighard, MD, MS, of the Department of Ophthalmology, Division of Eye Plastic, Orbital and Facial Cosmetic Surgery at the Kellogg Eye Center in Ann Arbor, Michigan, outlined findings of a study¹ assessing whether treatment with intralesional rituximab with fewer doses at higher concentrations constitutes an effective primary treatment of low-grade orbital lymphoma.

“Orbital lymphomas are the most frequent malignancy of the ocular adnexa and comprise about 50 to 60% of all orbital tumors,” said Reighard, while the majority are B-cell in origin. Although orbital lymphomas are typically treated with external beam radiotherapy (EBRT), this treatment can lead to significant ocular morbidity. Rituximab is typically used as a systemic treatment for orbital lymphoma, she added.

Reighard and colleagues carried out a retrospective chart review of patients with biopsy-proven orbital lymphoma who were treated with intralesional rituximab at a single center. Patients with lymphoma in other parts of the eye or those with orbital adnexa were excluded from the analysis.

“We graded each response as either a complete or partial response; complete meaning the complete tumor resolution on imaging, partial meaning decreased tumor type size but not completely resolved on clinical examination and on imaging,” Reighard explained.

A total of 7 eyes from 6 patients were included in the analysis, which showed:

• 3 eyes of 2 patients received 1 monthly injection, and 4 eyes of 4 patients received 2 monthly injections
• 71.4% of treated eyes had complete resolution of their orbital lymphoma
• 3 eyes of 2 patients had a complete response after 1 intralesional 50 mg rituximab injection, and 2 eyes of 2 patients had a complete response after 2 injections
• 2 eyes of 2 patients had a partial response after 2 monthly 50 mg rituximab injections
• The majority of the patients (62.5%) reached complete resolution after only 1 or 2 monthly injections without recurrence

“The side effects were varied but were transient, so anything from post-injection pain, chills, intermittent diplopia, headache, some systemic symptoms as well,” Reighard said. No long-term sequalae were reported.

Overall, results indicated “intralesional rituximab is a suitable in-office primary treatment for low grade orbital lymphoma,” she said. The study’s small sample size and varied follow-up intervals mark limitations.

An additional presentation² explored factors associated with survival outcomes among patients with conjunctival lymphomas in the United States. Kevin Wu, MD, a third-year ophthalmology resident at Mount Sinai Hospital in New York City, outlined results of the epidemiological study, which used nationally representative data from the Surveillance, Epidemiology, and End Results (SEER) database recorded between 1998 and 2018.

Including data from 1066 patients, it marks the largest epidemiological study conducted on conjunctival lymphomas, while “the cases including this database is estimated to account for approximately 35% of the entire US population,” Wu explained.

Although mean patient age was 59, this factor varied depending on histological subtypes, and 10-year overall survival was 72.1%. “Among the B-cell lineage lymphoma subtypes extra marginal zone lymphoma conferred the best 10 year overall survival at 76%, while mantle cell lymphoma conferred the worst 10 year overall survival at 29.4%,” Wu said.

Multivariate analyses revealed the following negative predictors of overall survival:

• Older age (hazard ratio [HR] 1.09; 95% CI, 1.08-1.11; P < .001)
• Indian/Alaska Native race (HR 5.20; 95% CI, 1.62-16.65; P = .018)
• Lymphoplastacytic histological subtype (HR 24.17; 95% CI, 7.23-80.79; P < .001)
• Mantle cell lymphoma histological subtype (HR 1.95; 95% CI, 1.05-3.62; P = .036)
• Natural killer/T-cell histological subtype (HR 30.46; 95% CI, 6.89-134.72; P < .001)
• No surgery (HR 1.34; 95% CI, 1.02-1.76; P = .036)

Asian or Pacific Islander race was the only positive predictor of overall survival (HR 0.53; 95% CI, 0.31-0.90; P = .018).

In addition, “patients that did not receive chemotherapy had improved disease specific survival,” Wu said. “However, it should be noted that in previous studies, they show the utility of radiation therapy on decreasing rates of recurrence, which was not examined in this study.”

The retrospective design of the study marks a limitation. Wu also noted that treatment modalities have changed throughout the study period.

References

1. Reighard C, Demirci H. Primary treatment of orbital lymphoma with intralesional injections of rituximab. ASOPRS Fall Scientific Symposium; November 11-12, 2021; New Orleans, Louisiana. Accessed November 12, 2021. https://asoprsfall.virtualbyvario.com/wp-content/uploads/2021/10/ASOPRS_2021_fall_syllabus_FINAL.pdf

2. Wu K, Chelnis J. Epidemiology and survival outcomes for primary conjunctival lymphomas in the United States. ASOPRS Fall Scientific Symposium; November 11-12, 2021; New Orleans, Louisiana. Accessed November 12, 2021. https://asoprsfall.virtualbyvario.com/wp-content/uploads/2021/10/ASOPRS_2021_fall_syllabus_FINAL.pdf