IntraOcular Lymphoma

Intraocular lymphoma is a rare and aggressive cancer that originates within the eye, most commonly affecting the retina and vitreous (the gel-like substance inside the eye). It is a type of non-Hodgkin lymphoma, often classified as primary intraocular lymphoma (PIOL) or as part of primary central nervous system lymphoma (PCNSL) due to its frequent association with brain involvement.

This cancer typically affects older adults and can be difficult to diagnose because it mimics chronic eye inflammation (uveitis). Common symptoms include blurred vision, floaters, eye pain, and sometimes vision loss. Diagnosis usually requires vitreous biopsy, imaging, and cytologic and molecular testing of eye fluid samples.

Treatment often includes intravitreal chemotherapy (direct injection into the eye), systemic chemotherapy, and sometimes radiation therapy, especially if the central nervous system is involved. Prognosis depends on early detection and whether the disease has spread beyond the eye.

Because of its subtle symptoms and high risk of recurrence or CNS involvement, intraocular lymphoma requires careful monitoring, multidisciplinary management, and long-term follow-up.

🧿 Intraocular Lymphoma Fact Sheet

Cancer Type: Rare Eye Cancer (Subtype of Primary Central Nervous System Lymphoma)
Primary Site: Retina, vitreous, and/or optic nerve within the eye
Subtype: Usually Non-Hodgkin’s Lymphoma (Diffuse Large B-cell Lymphoma)
Website Reference: OcularCancer.com

🔍 What is Intraocular Lymphoma?

Intraocular lymphoma (IOL) is a rare and aggressive eye cancer that develops in the lymphoid tissue within the eye, often affecting the retina and vitreous. It typically arises as a primary intraocular lymphoma (PIOL), but may also be secondary, spreading from systemic or central nervous system lymphoma.

It is considered a subset of primary central nervous system lymphoma (PCNSL) when confined to the eye and/or brain.

📊 Key Facts:

Prevalence: Extremely rare; exact incidence unknown, but ~1 case per million people annually
Most Common Type: Diffuse Large B-Cell Lymphoma (DLBCL)
Age of Onset: Typically affects individuals aged 50 - 70
Laterality: Can be unilateral or bilateral (often becomes bilateral)

🧬 Causes & Risk Factors:

No known definitive cause
Increased risk in immunocompromised individuals (e.g., HIV/AIDS, post-transplant patients)
Association with Epstein-Barr Virus (EBV) in some cases
Most patients are otherwise immunocompetent

👁️ Signs and Symptoms:

Painless, progressive vision loss (most common)
Floaters or blurred vision
Redness and inflammation
Chronic uveitis that does not respond to steroids
May mimic autoimmune or infectious eye diseases
Vitreous haze or retinal infiltrates seen on exam

🩺 Diagnosis:

Often misdiagnosed as chronic uveitis
Requires high suspicion and multiple tests:
- Dilated eye exam with slit lamp and fundus exam
- Optical coherence tomography (OCT)
- Fluorescein angiography
- Vitreous biopsy (gold standard)
- Cytology and immunohistochemistry
- Flow cytometry & PCR for gene rearrangements (clonality testing)
MRI brain and lumbar puncture to assess CNS involvement

⚙️ Types of Intraocular Lymphoma:

Primary Intraocular Lymphoma (PIOL):
- Originates in the eye
- Often part of or progresses to Primary CNS Lymphoma
Secondary Intraocular Lymphoma:
- Spread from systemic lymphoma
- Often involves uvea (choroid)

🧪 Treatment Options:

Intravitreal chemotherapy:
- Methotrexate or rituximab injections directly into the eye
Systemic chemotherapy:
- High-dose methotrexate-based regimens
- Rituximab for B-cell lymphoma
Radiation therapy:
- External beam radiation (especially if bilateral or recurrent)
Autologous stem cell transplant:
- In recurrent or CNS-involved disease
CNS-directed treatment is often needed even if lymphoma appears confined to the eye

🎯 Prognosis:

Prognosis varies depending on spread to CNS and response to treatment
Median survival: ~3 - 5 years in many studies with CNS involvement
Better outcomes with early detection and CNS management
Risk of relapse is high; requires ongoing surveillance

🔄 Follow-Up & Monitoring:

Regular ophthalmic exams (OCT, fundus photography)
Neurological monitoring and imaging
Ongoing coordination with hematologist/oncologist
Long-term surveillance for recurrence or CNS spread

❤️ Support & Resources:

Lymphoma support organizations
Rare cancer advocacy groups
Ocular oncologists and uveitis specialists
Mental health support for coping with vision loss or cognitive symptoms

📌 Key Takeaways:

Intraocular lymphoma is rare, serious, and often misdiagnosed.
It mimics inflammation - any persistent or steroid-resistant uveitis should raise suspicion.
Diagnosis is challenging and requires specialized testing and multidisciplinary care.
Early and aggressive treatment can preserve vision and extend life.
Close monitoring is essential due to high recurrence and CNS involvement risk.