New data from the Flinders University-led International Vitreoretinal B-Cell Lymphoma Registry are helping to clarify how vitreoretinal lymphoma first presents, addressing long-standing gaps in knowledge about the rare malignancy.
The findings, published in Clinical & Experimental Ophthalmology, draw on the largest global dataset assembled to date for vitreoretinal lymphoma, an aggressive intraocular cancer that can be difficult to distinguish from more common inflammatory eye conditions.
Vitreoretinal lymphoma originates inside the eye but frequently mimics uveitis, leading to delays in diagnosis and, in some cases, inappropriate initial treatment. The condition can cause permanent vision loss and is often associated with lymphoma in the central nervous system, including the brain.
According to corresponding author Professor Justine Smith, from FHMRI Eye & Vision at Flinders University, the registry is helping to fill critical knowledge gaps.
“Our international registry creates an unprecedented opportunity to study this rare cancer in depth and across continents and provides hope for better understanding, better treatment and better quality of life for people affected,” Professor Smith said in a statement from Flinders University.
“Delayed diagnosis of this eye cancer can carry serious consequences, but our findings offer practical information that will help clinicians consider the disease earlier, which can protect vision and reveal cases linked to brain lymphoma.”
The study analysed 138 newly diagnosed patients from centres across Europe, the Americas, the Western Pacific and South-East Asia. Most patients were in their 60s at diagnosis, although men tended to develop symptoms at a younger age than women. Nearly two-thirds had bilateral disease at presentation.
“This level of detail is only possible because the registry brings together data from many research centres that could not do this work alone,” Professor Smith said.
The analysis found that approximately one in four patients had evidence of lymphoma in the brain or elsewhere in the body at the time the ocular disease was first identified.
“This connection highlights how important coordinated care is for people, because eye findings may be the first sign of a much broader illness,” she said.
Early signs of vitreoretinal lymphoma are typically detected through routine ophthalmic examination and standard imaging modalities. Subtle clinical features may prompt further investigation, particularly when a patient’s presentation does not align with the usual pattern of inflammatory eye disease.
“Our registry’s global data helps us understand the early warning signs that doctors should look for, especially when a patient’s symptoms don’t fit the usual pattern,” Professor Smith said.
The study also confirmed that the vast majority of patients had the same lymphoma subtype, a finding that has been difficult to establish in smaller cohorts.
“This finding shows how global data collection gives clarity that individual studies cannot provide,” she said.
Visual outcomes at presentation varied considerably. While many patients retained functional vision at diagnosis, others had already experienced significant sight impairment.
“Understanding these patterns helps clinicians explain what patients may experience and feeds into decisions about treatment,” Professor Smith said.
The International Vitreoretinal B-Cell Lymphoma Registry collects real-world clinical data from participating centres using a shared protocol. Professor Smith said this collaborative approach enables researchers to identify trends that would otherwise remain undetected.
“It collects real-world clinical information from multiple countries using a shared protocol, enabling researchers to detect trends that would otherwise go unnoticed. In this way, patient experiences contribute to discoveries that can improve diagnosis and care,” she said.
As additional centres contribute data, researchers plan to examine long-term visual outcomes and treatment responses, with the aim of improving survival and quality of life for affected patients.
The paper, Presenting Clinical Features of Vitreoretinal Lymphoma, has been published in Clinical & Experimental Ophthalmology (DOI: 10.1111/ceo.70067). Lead author is Professor Justine R Smith, with the full list of authors available in the published article.
The research was supported by Tour de Cure, the Queensland Eye Institute Foundation (RSP-569-2024) and the National Health and Medical Research Council (2025222), according to Flinders University.
