Abstract
Introduction
The aim of this study was to characterize the clinical, imaging, diagnostic, and treatment features of vitreoretinal lymphoma (VRL) in a tertiary referral center.
Methods
Retrospective review of patients with VRL managed at Inselspital, Bern University Hospital, between February 2022 and February 2026. Demographic, clinical, multimodal imaging, biopsy, MYD88 mutation, treatment, and survival data were analyzed.
Results
Seventeen eyes from 9 patients were included (median age 70 years): ten eyes with biopsy-confirmed VRL without concurrent CNS lymphoma, and seven eyes with ocular involvement in the context of confirmed CNS lymphoma, including two eyes where ocular diagnosis relied on clinical and imaging findings alone. Mean symptom-to-diagnosis interval was 41 weeks (range 1–104 weeks). At presentation, vitreous cells were present in 94.1% and anterior chamber cells in 29.4%. Posterior segment findings included inner/outer retinal disruption (47.1%), retinal infiltration (29.4%), intraretinal hyperreflective foci (52.9%), irregularity of the RPE (23.5%), CME (23.5%). All patients underwent vitreous or transretinal biopsy, with 11 biopsies performed overall; 8 (72.7%) were cytology-positive. MYD88 mutations were detected in 80% of tested cases (8/10). All cases were reviewed in a multidisciplinary tumor board. VRL without concurrent CNS involvement was mainly treated with local radiotherapy, whereas cases associated with extraocular involvement predominantly received combined systemic immunochemotherapy and radiotherapy. Mean follow-up was 18 months, with no deaths recorded during follow-up.
Conclusion
VRL remains a diagnostic challenge due to heterogeneous and nonspecific presentation often leading to a delayed diagnosis. Multimodal imaging, vitreous biopsy, and MYD88 analysis are essential, with MYD88 providing key support in cytology-negative cases. Multidisciplinary management optimizes outcomes.
Keywords: Vitreoretinal lymphoma, Vitreous biopsy, MYD88
Introduction
Vitreoretinal lymphoma (VRL) is a rare ocular lymphoma confined to the vitreous, retina, and subretinal space, representing the predominant form of intraocular lymphoma. The vast majority of cases are of large B-cell origin, although rare T-cell variants have been described [1]. VRL may occur in isolation or in association with primary central nervous system lymphoma, from which it is pathophysiologically distinct. VRL is considered a form of primary disease whether it occurs in isolation or with concurrent CNS involvement and is classified as a variant of primary CNS lymphoma [2]. This contrasts with secondary intraocular lymphoma, which represents metastatic spread from systemic lymphoma outside the CNS and typically involves the uvea rather than the vitreoretinal compartment [3]. In all its forms, intraocular lymphoma is a rare condition that poses a significant diagnostic challenge because it can closely mimic more common inflammatory ocular disorders, particularly chronic intermediate or posterior uveitis [4]. Its clinical presentation is often nonspecific and may include vitreous cells, anterior chamber inflammation, and subtle retinal or subretinal lesions, which can delay recognition and appropriate management. Recent advances in multimodal imaging, including optical coherence tomography (OCT), fundus autofluorescence (FAF), and fluorescein angiography (FA), have improved the ability to detect features suggestive of intraocular lymphoma [5]. Nevertheless, a definitive diagnosis still depends on tissue sampling, most commonly through vitreous or retinal biopsy, which may yield false-negative results in up to 45% of cases [6] because of the fragility and low cellularity of lymphoma cells.
Molecular diagnostics have become an important adjunct in this setting, particularly the identification of MYD88 mutations, which can support the diagnosis when cytology is inconclusive [7]. Despite these advances, diagnostic delay remains common and may negatively affect both ocular and systemic outcomes.
In this study, we present a retrospective case series of patients diagnosed with VRL at a tertiary referral center, including cases with and without concurrent CNS lymphoma. We aimed to characterize the clinical presentation, multimodal imaging findings, diagnostic approaches, and treatment outcomes, with particular attention to diagnostic delay, biopsy yield, OCT biomarkers, and survival.
Methods
This retrospective single-center analysis included all patients diagnosed with VRL who presented to the tertiary referral department of ophthalmology at Inselspital, Bern University Hospital, Switzerland, between February 2022 and February 2026. In cases with suspected VRL, the standard diagnostic workup included cytology, flow cytometry, and MYD88 mutation analysis. Additional testing was performed at the discretion of the pathologists and when recommended by the multidisciplinary lymphoma board, to which all cases were referred.
Electronic medical records were retrospectively reviewed to collect demographic, clinical, imaging, diagnostic, and treatment-related data. Heidelberg Spectralis SD-OCT images obtained at the first presentation to our clinic were analyzed in all patients to assess retinal involvement at baseline. SD-OCT examinations were subsequently repeated at each follow-up visit to monitor disease course and detect signs of recurrence or lymphoma-related ocular complications.
The OCT images of the first consultation at our clinic were independently reviewed by two retina specialists, and findings were recorded by consensus. The following OCT features were considered suggestive of intraocular lymphoma: inner and outer retinal disruption, retinal infiltration, intraretinal hyperreflective foci (HRF), irregularity of the RPE, cystoid macular edema.
Results
A total of 17 eyes from 9 patients with a clinical or biopsy-proven diagnosis of VRL were included in this study. The cohort comprised 5 males and 4 females, and the median age at presentation was 70 years (range, 46–85 years; SD, ± 11.0). Demographics and clinical features of our cohort are reported in Table 1.
Table 1.
Demographics and clinical features
| Demographics and clinical features | n = 9 patients, 17 eyes |
|---|---|
| Age, median (IQR), years | 70 (46–85) |
| Age, mean (SD), years | 70 (±11.0) |
| Sex, n (%) | |
| Female | 4 (44.4) |
| Male | 5 (55.6) |
| Laterality, n (%) | |
| Unilateral | 1 (11.1) |
| Bilateral | 8 (88.9) |
| Diagnosis, eyes n (%) | |
| VRL without concurrent CNS lymphoma | 10 (58.8) |
| VRL with concurrent CNS lymphoma | 7 (41.2) |
| Biopsy results, n (%) | |
| Positive (B-cell) | 8 (72.7) |
| Negative | 3 (27.3) |
| MYD88 mutation detected, n (%) | 8/10 tested (80) |
| Time between symptoms and diagnosis, weeks (range) | 41 (1–104) |
| Retinal involvement, eyes, n (%) | 11 (64.7) |
| Therapy – VRL without CNS involvement (10 eyes), n (%) | |
| Radiotherapy alone | 8 (80) |
| Intravitreal rituximab → radiotherapy | 1 (10) |
| Intravitreal rituximab + methotrexate → radiotherapy | 1 (10) |
| Therapy – VRL with CNS involvement (7 eyes), n (%) | |
| Immunochemotherapy (PRIMAIN protocol) alone | 1 (14.3) |
| Immunochemotherapy (PRIMAIN protocol) + radiotherapy | 6 (85.7) |
| Mean follow-up, months (range) | 18 (1–41) |
| Deaths during follow-up | 0 |
VRL, vitreoretinal lymphoma; CNS, central nervous system; IQR, interquartile range; SD, standard deviation.
Eight patients had bilateral involvement, while 1 patient presented with unilateral lymphoma. Ten eyes (58.8%) had biopsy-confirmed isolated VRL. Seven eyes (41.2%) presented with ocular involvement in the context of concurrent central nervous system B-cell non-Hodgkin lymphoma, of which five were biopsy-confirmed and 2 eyes in one patient were diagnosed on clinical and imaging grounds alone, as both vitreous cytology and MYD88 mutation analysis were negative, but clinical findings were highly suggestive and supported by the concurrent diagnosis of CNS lymphoma; these two eyes are therefore formally classified as presumed VRL, as no definitive proof of intraocular involvement could be established. Most patients (n = 8) presented with visual symptoms prompting ophthalmologic evaluation and subsequent diagnosis of intraocular lymphoma. Two patients were asymptomatic, and the diagnosis was made either during follow-up of the fellow eye with previously diagnosed lymphoma or incidentally during surveillance for central nervous system lymphoma. All patients (9/9, 100%) underwent tissue sampling, 2 of the patients in both eyes. Tissue sampling consisted of vitreous biopsy (n = 10) or transretinal biopsy (n = 1). Of the 11 biopsies performed, 8 (72.7%) were positive for B-cell lymphoma, while 3 (27.3%) were negative. MYD88 mutation analysis was performed in 10 of 11 cases; in one case, it was not carried out because the vitreous biopsy was already diagnostic. Overall, MYD88 mutations were detected in 8 of 10 tested cases (80%). Notably, 1 of 3 biopsy-negative cases showed a MYD88 mutation. A detailed breakdown of biopsy type, cytology, MYD88 status, and additional testing per eye is provided in Table 2.
Table 2.
Diagnostic workup by eye
| Eye | Patient | Biopsy type | Cytology | MYD88 | Additional testing beyond standard workup |
|---|---|---|---|---|---|
| 1 | 1 | Vitreous | B-cell positive | Positive | – |
| 2 | 1 | Not performed | – | – | – |
| 3 | 2 | Vitreous | B-cell positive | Positive | CD79a (CSF) |
| 4 | 2 | Vitreous | B-cell positive | Positive | CD79a (CSF) |
| 5 | 3 | Transretinala | B-cell positive | Positive | IHC (CD20+, BCL2+); NGS panel (PIM1, BCL2, CD79B, MYD88, ETV6, KMT2D) |
| 6 | 3 | Not performed | – | – | – |
| 7 | 4 | Not performed | – | – | – |
| 8 | 4 | Vitreous | B-cell positive | Not performed | IHC (CD20+, CD30+, CD10−, MUM1+, BCL6+, BCL2+, CD5−, Cyclin D1–, Ki-67>50%) |
| 9 | 5 | Vitreous | B-cell positive | Positive | – |
| 10 | 5 | Not performed | – | – | – |
| 11 | 6 | Vitreous | B-cell positive | Not performed | — |
| 12 | 7 | Not performed | – | – | – |
| 13 | 7 | Vitreous | B-cell positive | Positive | – |
| 14 | 8 | Vitreous | Negative | Positive | – |
| 15 | 8 | Not performed | – | – | – |
| 16 | 9 | Vitreous | Negative | Negative | Cerebellar biopsy (positive) |
| 17 | 9 | Vitreous | Negative | Negative | – |
CSF, cerebrospinal fluid; IHC, immunohistochemistry; NGS, next-generation sequencing.
Transretinal biopsy performed after an initial negative vitreous biopsy at an external hospital.
Mean time between symptoms and diagnosis was 41 weeks (range 1–104 weeks). At presentation, 29.4% of eyes (5/17) had anterior chamber cells, and 94.1% (16/17) had vitreous cells.
Retinal involvement was observed in 11/17 eyes in the form of inner/outer retinal disruption (n = 8, 47.1%), retinal infiltration (n = 5, 29.4%), intraretinal HRF (n = 9, 52.9%), irregularity of the RPE (n = 4, 23.5%), CME (n = 4, 23.5%). Representative examples of these findings are shown in Figure 1.
Fig. 1.
Representative OCT images showing characteristic findings of intraocular lymphoma in different patients. a, b Subretinal infiltration appearing as a confluent band, irregular/indistinct RPE. c Cystoid macular edema.
Four patients underwent FA as part of their diagnostic workup. Two of these patients showed a typical pattern characterized by RPE mottling alternating with granular hyperfluorescence, corresponding to the characteristic “leopard spot” pattern observed on FAF (shown in Fig. 2). One patient demonstrated marked retinal vasculitis on FA. While this finding is not classically associated with intraocular lymphoma, it has been reported and may represent a potential confounder [8, 9]. In this case, the angiographic appearance initially prompted a diagnostic workup for uveitis before the correct diagnosis was established.
Fig. 2.
FA (a) and FAF (b) showing «mottling» or blockage at the level of the RPE alternate with hyperfluorescent granularity, which corresponds to a «leopard pattern» in FAF.
In patients without known central nervous system involvement, a comprehensive staging workup was performed, including whole-body PET-CT and brain MRI. Subsequently, all patients, including those with primary intraocular lymphoma, were discussed in a multidisciplinary lymphoma board hosted by the department of hemato-oncology.
Of the 10 eyes with isolated VRL, 8 (80%) were treated with local radiotherapy alone. The remaining 2 eyes initially received intravitreal pharmacotherapy – one with rituximab alone and one with a combination of rituximab and methotrexate (MTX) – but both subsequently required consolidation radiotherapy due to insufficient response. Of the 7 eyes with concurrent CNS involvement, 1 (14.3%) was treated with immunochemotherapy according to the PRIMAIN protocol alone, while 6 (85.7%) received a combination of radiotherapy and immunochemotherapy according to the PRIMAIN protocol.
At baseline, mean visual acuity was 0.70 (SD ± 0.28), improving to 0.73 (SD ± 0.30) at the last follow-up visit. Of the 17 eyes included, 5 showed an improvement in visual acuity, 5 remained stable, and 7 experienced a deterioration. One patient showed a marked decrease in visual acuity, from 1.0 at baseline to finger counting at the last follow-up, attributable to postoperative endophthalmitis occurring between the two visits. The mean follow-up time, defined as months between diagnosis and last follow-up, was 18 months (range, 1–41 months; SD ± 12.7). Since no patient in our cohort deceased during follow-up, we cannot report survival data. Regarding disease activity after therapy, 2 patients required further treatment during follow-up. One patient, initially diagnosed with VRL in the left eye in 2022 and treated with local radiotherapy, developed new contralateral involvement of the right eye in 2025, subsequently treated with radiotherapy. The second patient, diagnosed with CNS lymphoma and treated with systemic immunochemotherapy in 2024, experienced a recurrence in the left eye in January 2025, managed with local radiotherapy.
Discussion
In this retrospective study, we describe the clinical, imaging, diagnostic, and therapeutic characteristics of patients with VRL managed at a tertiary referral center. The study is notable for the complete follow-up of all included cases, the systematic use of vitreous biopsy, and the availability of MYD88 mutation analysis in nearly all patients. These features strengthen the diagnostic reliability of our cohort and provide a comprehensive overview of intraocular lymphoma in a real-world clinical setting in Europe.
One of the main findings of our series is the relatively long diagnostic delay, with a mean interval of 41 weeks (approximately 10 months) between symptom onset and diagnosis, likely influenced by the nonspecific initial presentation, variable degree of intraocular inflammation, and need for repeated diagnostic workup in some cases. This is consistent with previous literature: Zhao et al. [10] reported diagnostic delay in 85% of cases in a comprehensive meta-analysis, while other studies reported a mean time to diagnosis of 6 and 10 months, respectively [11, 12].
Our diagnostic yield was favorable, with positive vitreous biopsy results in 72.7% of procedures performed. This is particularly relevant given the known limitations of cytology in intraocular lymphoma, where low cellularity and cellular fragility frequently lead to false-negative results.
Molecular diagnostics substantially increased diagnostic sensitivity in our series. MYD88 L265P mutational analysis was performed by droplet digital PCR, targeting the p.L265P (c.794T>C) point mutation in exon 5, with a lower detection limit of approximately 0.5% mutated allele. The mutation was detected in 80% of tested cases (8/10), including a cytology-negative sample, underscoring the complementary role of molecular testing in cases where cytology alone is inconclusive. This detection rate is consistent with published sensitivities of 69–89% for MYD88 L265P across different assay platforms, with specificity ranging from 91 to 100% [13–15].
In our cohort, the standard diagnostic workup included cytology, flow cytometry, and MYD88 mutation analysis, with additional testing performed at the discretion of the pathologists and when recommended by the multidisciplinary lymphoma board. In 7 of 11 biopsied eyes, concordant positive cytology and MYD88 results provided a robust diagnostic basis; several of these cases were further characterized with immunohistochemistry, NGS panels, or CD79a testing in cerebrospinal fluid, reinforcing the diagnosis. In one eye with negative cytology, a positive MYD88 result combined with a highly suggestive clinical and multimodal imaging findings supported the diagnosis, which was subsequently corroborated by a favorable radiotherapy response – illustrating the well-recognized limitation of vitreous cytology due to low cellularity and cell fragility, which often necessitates an integrated diagnostic approach. In two eyes with negative vitreous biopsy and negative MYD88, no definitive proof of intraocular involvement could be established; however, given the concurrent positive cerebellar biopsy confirming CNS lymphoma and the highly suggestive clinical and imaging picture, these eyes were formally classified as presumed VRL and managed with systemic immunochemotherapy for the underlying CNS lymphoma, with the addition of local radiotherapy given the suspected ocular involvement.
These cases also highlight the diagnostic value of broader molecular characterization beyond MYD88 alone. While MYD88 L265P remains the cornerstone of molecular diagnosis in VRL, the field is rapidly moving toward multi-gene panel testing. Targeted next-generation sequencing of intraocular fluid combining MYD88 with CD79B, PIM1, BTG2, and ETV6 has demonstrated superior diagnostic accuracy, with sensitivities exceeding 90% in recent series [16]. Beyond diagnosis, CD79B Y196 mutation has emerged as a prognostic marker, with CD79B-mutated cases showing significantly earlier CNS progression compared to wild-type patients [17]. Our cohort was not tested systematically for CD79B or broader panel mutations; incorporating multi-gene sequencing represents a natural next step for future prospective studies.
Beyond mutation-based testing, interleukin-10 (IL-10) and the interleukin-10/interleukin-6 (IL-6) ratio represent an established complementary approach in the diagnosis of intraocular lymphoma. IL-10 and the IL-10/IL-6 ratio were not routinely assessed in our cohort, as MYD88 mutational analysis alone yielded satisfactory diagnostic results in the majority of cases. However, current evidence supports their combined use, as cytokine analysis provides complementary diagnostic value in clinical scenarios where MYD88 testing alone may be insufficient: MYD88 wild-type VRL, in which the L265P mutation is absent by definition; low cellularity samples, where IL-10 may remain detectable even when DNA yield is insufficient for PCR; and non-DLBCL histologies, in which MYD88 L265P is rarely present [18]. In our center, cytokine testing is reserved for diagnostically challenging cases; in the present cohort, it was not required.
The imaging findings in our series were in keeping with previously reported features of intraocular lymphoma [5]. OCT commonly demonstrated sub-RPE deposits, subretinal infiltrates, HRF, and cystoid macular edema. These findings reflect the heterogeneous anatomical patterns of involvement and reinforce the role of OCT as a useful noninvasive tool in the evaluation of suspected cases. The association between typical FAF and FA findings in several of our patients further supports the complementary role of multimodal imaging in raising suspicion for intraocular lymphoma, although none of these features is diagnostic on its own.
An interesting observation in our cohort was the case with marked retinal vasculitis on FA. While vasculitis is not a classic hallmark of intraocular lymphoma, it has been reported and may represent a confounding feature that initially directs attention toward inflammatory disease [19, 20]. In our patient, this angiographic pattern contributed to the differential diagnosis of uveitis before the correct diagnosis was established. This illustrates that intraocular lymphoma can present with atypical inflammatory signs and should remain in the differential diagnosis even when angiographic findings appear more suggestive of a uveitic process.
Regarding treatment, most eyes with primary ocular involvement received local therapy, predominantly radiotherapy, while eyes with concomitant CNS involvement were generally managed with combined systemic immunochemotherapy and local treatment. Local radiotherapy remains an effective option for ocular control in isolated VRL, whereas VRL with concurrent CNS involvement often requires systemic treatment. Intravitreal pharmacotherapy represents an alternative or adjunct to radiotherapy for local ocular disease control. Two agents are currently in use: MTX and rituximab. Intravitreal MTX has the longest evidence base, with response rates of 80–90% reported in prospective series, although recurrence after discontinuation is not uncommon [21, 22]. Intravitreal rituximab (1 mg/0.1 mL) has emerged as an alternative with a more favorable local tolerability profile, and retrospective data suggest comparable efficacy to MTX in terms of vitreous clearance and visual outcomes [23, 24]. In our cohort, both patients who received intravitreal pharmacotherapy, one treated with rituximab alone and one with a combination of rituximab and MTX, ultimately required consolidation radiotherapy due to insufficient disease control with intravitreal treatment alone. This supports the role of radiotherapy as an effective salvage or consolidation option in refractory or recurrent ocular disease.
Our overall survival follow-up was relatively short, but all patients were observed longitudinally, and no deaths occurred during the study period. Because VRL is a rare and heterogeneous condition, complete follow-up in a single-center series remains valuable, particularly when combined with uniform diagnostic workup and imaging review. Nevertheless, several limitations must be acknowledged. The retrospective single-center design in a tertiary care center introduces potential referral and selection bias toward more complex cases, which may partly explain the prolonged diagnostic delay observed in our cohort. The small sample size reflects the rarity of the disease but limits generalizability. In addition, the short follow-up precludes meaningful survival analysis and a reliable assessment of CNS progression rates. VRL carries a well-recognized risk of subsequent CNS involvement, with estimates ranging from 33% to 90% over the disease course, depending on patient selection criteria, treatment approaches, and follow-up duration [12, 25, 26]. In our cohort, despite the short follow-up, 1 patient who initially presented with isolated VRL developed CNS lymphoma 15 months after diagnosis, detected incidentally on routine surveillance MRI in the absence of neurological symptoms. This underscores the importance of systematic neurological surveillance and interdisciplinary management involving oncology and hematology in all VRL patients, even in the absence of neurological symptoms at diagnosis.
Finally, heterogeneity in treatment regimens and incomplete data availability (e.g., FA was performed in only a subset of patients) further limit the robustness of our findings. In summary, our case series highlights the diagnostic complexity of VRL and the real-world challenges it poses, as reflected by the prolonged diagnostic delay observed in our cohort. Integrating clinical examination, multimodal imaging, vitreous biopsy, and molecular diagnostics – in particular MYD88 mutation analysis – proved essential in establishing diagnosis, including in biopsy-negative cases. Heightened clinical suspicion in patients presenting with atypical uveitis-like features, combined with early referral to a multidisciplinary lymphoma board, remains the most effective strategy to reduce diagnostic delay and individualize treatment.
Statement of Ethics
This study was conducted in accordance with the Declaration of Helsinki and approved by the Local Ethics Committee of the Canton of Bern, Switzerland (BASEC-ID 2023-00768; decision reference number 5491). Patients provided written informed consent for the use of their anonymized clinical data in accordance with institutional and local regulations.
Conflict of Interest Statement
The authors have no conflicts of interest to declare.
Funding Sources
This study was not supported by any sponsor or funder.
Author Contributions
L.A. was responsible for data curation and was a major contributor in writing the manuscript. L.F.D., E.B., and M.Z. were major contributors in writing the manuscript. R.A. was a major contributor in the conception of the work. F.H. was the leader of the project and was a major contributor in writing the manuscript. All authors approved the final draft of this work.
Funding Statement
This study was not supported by any sponsor or funder.
Data Availability Statement
The data that support the findings of this study are not publicly available due to privacy reasons but are available from the corresponding author upon reasonable request.
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Data Availability Statement
The data that support the findings of this study are not publicly available due to privacy reasons but are available from the corresponding author upon reasonable request.