What Is Cancer-Associated Retinopathy?

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Cancer-associated retinopathy is a progressive, autoimmune form of retinopathy, a disease of the retina, the layer of tissue in the back of the eye that detects light and sends images to your brain. It can cause vision loss and often precedes a cancer diagnosis. It appears to affect women more than men and generally affects individuals aged 40–85 years of age.

The condition is considered an autoimmune disease, a disorder in which your immune system attacks healthy cells by mistake. In cancer-associated retinopathy, the antibodies, blood proteins that protect your body from pathogens, generated from the autoimmune response will affect different retinal tissues, resulting in vision loss.

a doctor checking a patient's eyes

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Cancer-Associated Retinopathy Symptoms

The most common symptom of cancer-associated retinopathy is an acute decline in vision over a period of weeks to months. The vision loss is painless and accompanied by flashes or a flickering of light (photopsia) and as sensitivity to light, also known as photosensitivity.

Depending on which photoreceptors are affected, people with cancer-associated retinopathy may experience different symptoms. There are two types of photoreceptors in the retina: rods and cones. If the rods, which are responsible for vision at low light levels, are impacted, you may have constriction of the visual field, have difficulty seeing in low light, and see blind spots in the mid peripheral visual fields.

If the cones, which are responsible for color vision and vision at higher light levels, are affected, symptoms may include photosensitivity, central visual blind spots, reduction in visual acuity, and decrease in color perception.

Recap

Cancer-associated retinopathy symptoms can differ depending on the part of the retina that is affected. Common symptoms are loss of visual acuity and blind spots in the visual fields.

Causes

The most common cancers co-occurring with cancer-associated retinopathy are small cell lung carcinoma (a fast-growing lung cancer), breast cancer, and gynecological cancers. This condition has also been linked to:

  • Colon cancer
  • Other types of lung cancer
  • Kidney cancer
  • Skin cancer
  • Pancreatic cancer
  • Lymphoma
  • Prostate cancer

The theory that cancer-associated retinopathy is autoimmune was developed in 1983. The autoimmune response occurs when tumor antigens trigger antibodies, which react with retinal proteins. This reaction leads to cell death, also known as apoptosis, and retinal degeneration.

Diagnosis

Diagnosis can be challenging since there is no set diagnostic criteria for cancer-associated retinopathy. An eye care doctor, such as an optometrist or an ophthalmologist, can utilize a variety of tests and tools to make a diagnosis:

  • Visual fields: Assessment of visual fields may identify a central or ring-like blind spots.
  • Optical coherence tomography: This test can determine if there is loss of the outer retinal layer.
  • Electroretinogram: This measures the electrical activity of the retina in response to a light stimulus. Full-field electroretinogram is usually abnormal with cancer-associated retinopathy.
  • Fundus autofluorescence: Fundus autofluorescence imaging is used to record fluorescence (emission of light by a substance that has absorbed light or other electromagnetic radiation) that may occur naturally in structures in your eyes or as a result of a disease process. Fundus autofluorescence imaging can provide information about the health and function of the retina.

Your doctor may also order an anti-retinal antibody testing to determine the presence of certain antibodies linked to cancer-associated retinopathy.

Recap

Diagnosis of cancer-associated retinopathy can be challenging, so any vision changes should be evaluated by an eye doctor, such as an optometrist or ophthalmologist.

Treatment

Cancer-associated retinopathy often precedes a cancer diagnosis, therefore, obtaining a complete medical workup for potential cancer diagnosis is crucial. The treatment of the underlying cancer does not usually affect cancer-associated retinopathy. However, cancer treatment is a significant determination of long-term survival.

Cancer-associated retinopathy is an autoimmune disease. One method of treatment is to suppress the immune response to prevent further formation of anti-retinal antibodies. Some of the different treatments vonsidered for cancer-associated retinopathy and that lower the risk of irreversible vision loss include:

  • Local and high-dose corticosteroids: Research suggests that these medications can take up to a year to take effect and usually result in disease stabilization.
  • Intravenous gammaglobulin (IVIG): IVIG is thought to neutralize antibodies and has been used with some success.
  • Plasmapheresis: This treatment, in which blood is removed through one area and run through a filtration system to allow antibodies to be returned to the patient in a different location, has shown some success with preventing vision loss.
  • Monoclonal antibody therapy: Rituxan (rituximab) is a monoclonal antibody therapy that attacks the antibodies causing cancer-associated retinopathy and has shown success at stabilizing vision.

Recap

At this time, there are no treatments for cancer-associated retinopathy that can cure vision loss. However, there are several treatments that have shown promise in preventing further vision loss.

Prognosis

Prognosis of cancer-associated retinopathy depends on the type of cancer, the extent of damage to retinal rods and cones, and your response to the above treatments. Vision that is lost cannot be regained, and preserving the remaining vision can sometimes be achieved with the above cancer treatments.

Summary

Cancer-associated retinopathy is a rare autoimmune disease associated with certain types of cancer. It causes a progressive loss of vision, generally resulting in difficulty seeing in low light, blind spots in the visual fields, and decrease in visual acuity. Cancer-associated retinopathy is challenging to diagnose, so any vision changes should be evaluated by an eye doctor, such as an optometrist or ophthalmologist, as soon as possible.

A Word From Verywell

Cancer-associated retinopathy is a progressive autoimmune disease associated with certain types of cancers. Since the vision loss often precedes the cancer diagnosis, cancer-associated retinopathy can be difficult to diagnose. Early diagnosis not only can help you preserve your vision, but it can also help you get treated for the associated cancer sooner.

Establishing care and going through routine eye examinations with an eye doctor, such as an optometrist or ophthalmologist, can help detect this condition. You should also schedule an evaluation with your eye doctor whenever you notice vision changes.

Frequently Asked Questions

Frequently Asked Questions

  • Does a cancer diagnosis also mean a diagnosis of cancer-associated retinopathy?

    No. Cancer-associated retinopathy is linked to specific cancers, such as small cell lung cancer, breast cancer, and gynecological cancers. It typically affects women more than men and generally affects people aged 40–85 years old. However, it is rare.

  • Does cancer-associated retinopathy cause blindness?

    Yes, cancer associated retinopathy can cause blindness. The antibodies produced during the autoimmune response attack the rods and cones, which are photoreceptors, in the retina, which detects light and sends images to the brain.

  • Is there any treatment for cancer-associated retinopathy?

    Yes. Most treatments for cancer-associated retinopathy focus on suppressing the immune system to prevent further production of antibodies that damage the retina and create vision loss. Although cancer-associated retinopathy is a progressive disease, when identified and treated early, further vision loss can be prevented. Newer research with monoclonal antibodies indicates that this therapy may not just stabilize vision but also reverse some of the visual loss caused by cancer-associated retinopathy.

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4 Sources
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