Ocular Oncology Understanding Eye Tumors, Risks & Treatments

Ocular oncology is a subspecialty within ophthalmology dedicated to the detection, diagnosis, treatment, and management of tumors that occur in or around the eye, including both benign and malignant forms. This field addresses a wide range of conditions—such as eyelid cancers, conjunctival neoplasia, intraocular tumors like uveal melanoma, retinoblastoma, and ocular lymphoma. While eye tumors are rare—with approximately 3,400–3,500 new cases per year in the U.S. —they can pose serious threats to vision and life if left untreated. Today’s ocular oncology focuses not only on saving lives but also on preserving sight through early detection and advanced multidisciplinary care .

Why Ocular Oncology Matters

Although uncommon, eye cancers are particularly dangerous due to their potential to spread (metastasize), often to organs like the liver, lungs, or brain. Without proper management, these tumors can cause permanent vision loss—or even death. Early detection during routine exams (especially dilated fundus exams) dramatically improves outcomes . Ocular oncologists work in multidisciplinary teams—partnering with cornea specialists, radiation oncologists, pathologists, and medical oncologists—to balance cancer control with eye preservation.

Symptoms of Ocular Oncology

Recognizing signs early ensures prompt evaluation:

• Visual changes: blurred vision, blind spots, loss of peripheral field
• Floaters or flashes (photopsia)
• Dark pupil or iris spots, especially if growing
• Change in pupil shape or eye displacement/proptosis
• Redness, irritation, or a persistent lump on the eyelid or conjunctiva

In many cases—especially with small or posterior tumors—there may be no symptoms, making routine dilated exams essential.

Causes & Risk Factors of Ocular Oncology

• Ocular tumors can be primary (originating in the eye) or secondary (metastatic), spreading from elsewhere in the body.

Key risk factors include:

• Age: Most ocular melanomas occur after 50; retinoblastoma affects children under 5.
• Light pigmentation: Individuals with fair skin and blue or green eyes are at higher risk.
• Genetic predispositions: Includes BAP1 mutation, RB1 gene (retinoblastoma), dysplastic nevus syndrome, and Von Hippel-Lindau disease.
• UV exposure: Sunlight exposure can promote eyelid and conjunctival cancers.
• Immunosuppression & viruses: HPV is linked to ocular surface squamous neoplasia; lymphoma risk increases with immune deficiency.
• Systemic cancers: Breast and lung cancers often metastasize to the eye.

Diagnosis of Ocular Oncology

Accurate diagnosis involves several key steps:

1. Comprehensive clinical exam – includes slit-lamp and dilated fundus exam; most cases are identified clinically .

2. Imaging modalities:

• Ultrasound (A/B‑scan) to assess size and internal structure
• OCT for retinal and choroidal details
• Fluorescein angiography for tumor vascularity
• CT or MRI for orbital or systemic spread

3. Biopsy / FNAB: Reserved for select surface tumors or diagnostic uncertainty; often avoided in uveal melanoma .

4. Genetic profiling: e.g., DecisionDx‑UM, to stratify metastatic risk in uveal melanoma.

5. Systemic workup: Includes liver imaging and systemic scans to evaluate for metastasis, especially for uveal tumors .

Treatment of Ocular Oncology

Treatment is tailored based on tumor type, size, location, and spread:

Radiation-Based Therapies

• Plaque brachytherapy: Gold standard for many uveal melanomas—with ~95% local control in small to medium cases.
• Proton beam therapy: Delivers precise radiation, minimizing damage to surrounding tissues—ideal for complex or large tumors .
• External beam & TTT: Suitable for select tumors; often combined with other modalities.

Surgical Options

• Enucleation: Removal of the eye, used for advanced tumors.
• Local resection (e.g., iridocyclectomy): Eye‑sparing removal for accessible tumors .
• Surface excision (eyelid, conjunctiva): Often combined with cryotherapy or topical chemotherapy (e.g., mitomycin C) .

Laser & Cryotherapy

• Transpupillary thermotherapy and photocoagulation for small intraocular tumors.
• Cryotherapy for external lesions .

Medical & Systemic Therapies

• Immunotherapy: Agents like tebentafusp (Kimmtrak®) for metastatic uveal melanoma; rituximab or methotrexate for lymphoma.
• Chemotherapy: Mainly for lymphoma; used as eye drops, injections, or systemic therapy.

Supportive Care

Vision rehabilitation and low-vision support for residual impairment.

• Prosthetic eyes post-enucleation.
• Lifelong follow-up: Monitoring recurrence or metastasis, especially for high‑risk molecular profiles.

Conclusion

Ocular oncology is a critical discipline aimed at preserving both sight and life through prompt detection and comprehensive management of eye tumors. Though rare, these cancers demand expert, multidisciplinary care. Early diagnosis—often during a routine exam—enables effective, eye-conserving treatments like radiation, surgery, and immunotherapy. Advances in genetics and therapy continue to improve outcomes. Awareness of symptoms, risk factors, and treatment avenues ensures timely referral to ocular oncology specialists and better patient prognoses.