Systemic diffuse large B-cell lymphoma with bilateral ciliary body involvement

Intraocular lymphoma—cancer that affects the eye—is one of the rarest forms of malignancy, with primary cases (starting in the eye) occurring in just 4.8 per million people annually in the U.S. But even rarer? Secondary intraocular lymphoma (SIOL), where cancer spreads to the eye from another part of the body. Now, a team from Peking Union Medical College Hospital has shared a unique case of SIOL: a man with systemic diffuse large B-cell lymphoma whose cancer spread to the ciliary body—a tiny, muscle-rich structure that produces eye fluid and helps focus the lens—in both eyes. The findings offer critical insights for how doctors diagnose and treat this overlooked complication.

The Patient’s Story: Vision Loss, Misdiagnosis, and Deterioration

The case centers on a 54-year-old man who arrived at the hospital in July 2019 with red, painful, blurry vision in both eyes. For three months prior, he’d been treated for bilateral anterior uveitis (inflammation of the eye’s middle layer) and secondary glaucoma (high pressure inside the eye) with steroid eye drops and glaucoma medications. But his vision worsened—until he developed a pseudohypopyon (a collection of cells in the eye that mimics pus) and even higher eye pressure.

Importantly, the man had a history of systemic diffuse large B-cell lymphoma (a fast-growing blood cancer) and had completed six rounds of chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). But his eye symptoms weren’t initially linked to his lymphoma—until tests revealed something unexpected.

Diagnosing the Hidden Cancer: How Ultrasound and Biopsy Revealed the Truth

To figure out why the man’s eyes weren’t improving, doctors used two key tools:

B-scan ultrasound: This showed a clear vitreous (the gel inside the eye) and no issues with the retina or choroid (the eye’s light-sensitive and blood-rich layers).
Ultrasound biomicroscopy (UBM): A high-resolution ultrasound for the front of the eye, UBM revealed a critical clue: 360° thickening of the iris and ciliary body (the entire circumference of both structures) and closed drainage angles (the part of the eye that controls fluid pressure).

Next, doctors performed anterior chamber paracentesis (draining a small amount of fluid from the front of the eye) and diagnostic vitrectomy (removing a tiny sample of the vitreous gel). Tests on these samples found atypical lymphoid cells—abnormal immune cells—that matched his original lymphoma. Gene rearrangement analysis (a test to confirm cancer cell origin) confirmed the cells were from diffuse large B-cell lymphoma.

Treatment: Injecting Chemo Directly into the Eye—and Rapid Results

The man was diagnosed with secondary bilateral ciliary body lymphoma (diffuse large B-cell). His treatment? Intravitreal methotrexate—a chemotherapy drug injected directly into the eye—at a dose of 0.4 mg in 0.1 mL. The schedule: twice weekly for 4 weeks, weekly for another 4 weeks, then monthly for 10 months.

The results were dramatic:

Vision improved: His best-corrected visual acuity (BCVA)—how well he could see with glasses—went from 20/100 (right eye) and 20/200 (left eye) to 20/63 and 20/100, respectively.
Pressure normalized: Eye pressure dropped from 42 mmHg (right) and 45 mmHg (left) to 13 mmHg and 19 mmHg—well within the normal range (10–21 mmHg).
Inflammation resolved: The pseudohypopyon (the “pus-like” collection) disappeared, and anterior chamber inflammation (redness, cells) eased.

The patient continued systemic chemotherapy for his lymphoma.

Why This Case Matters for Patients and Doctors

This case highlights three key takeaways for anyone dealing with lymphoma or eye health:

SIOL can mimic common eye conditions—The man was initially misdiagnosed with uveitis and glaucoma because SIOL symptoms (inflammation, high pressure) overlap with benign eye issues. But when standard treatments fail, doctors should always consider cancer—especially in patients with a history of systemic lymphoma.
UBM is a game-changer for diagnosis—The ciliary body is hard to see with regular exams, but UBM’s high resolution let doctors spot the 360° thickening that signaled lymphoma. As researchers Konstantinidis et al. noted in a 2013 study, this “ring-like” growth pattern is a red flag for ciliary body lymphoma.
Intraocular chemo works—and fast—Injecting methotrexate directly into the eye targeted the cancer where it was growing, leading to quick improvements in vision and pressure. This aligns with previous research showing intraocular therapy can preserve vision in SIOL patients.

The Bottom Line

For patients with systemic lymphoma, eye symptoms like redness, pain, or blurred vision shouldn’t be dismissed as “routine.” For doctors, tools like UBM and biopsy are essential to rule out SIOL—especially when inflammation or high pressure doesn’t improve with standard care. As the Peking Union team concludes: “Ciliary body recurrence of lymphoma should be considered in SIOL patients with pseudohypopyon and elevated eye pressure. Biopsy is the gold standard for diagnosis, and multidisciplinary care (eye doctors + oncologists) offers the best chance for personalized treatment.”

This study was published in the Chinese Medical Journal (2020;133:109–110) by Yu Di, Jun-Jie Ye, and Bi-Lei Zhang from the Department of Ophthalmology at Peking Union Medical College Hospital (Chinese Academy of Medical Sciences, Beijing). The full study is available at doi.org/10.1097/CM9.0000000000000580.

The patient gave informed consent for his clinical information and images to be published, with efforts made to protect his identity (though anonymity can’t be guaranteed). The authors declare no conflicts of interest.

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