Eye cancer : treatment, causes, metastasis

Eye Cancer

Eye cancer is a rare disease that can affect both children and adults in different ways.

Eye Cancer

Photo Andrea Piacquadio in Pexels

In order to begin treatment early, it is necessary to be aware of recognizing symptoms as soon as they occur.

An update with Professor Nathalie Cassoux, Head of the Department of Surgical Oncology and Head of the Department of Ocular Oncology at the Curie Institute.

Definition

Eye cancer – or rather eye cancers, as there are several forms – are all rare diseases: 500 to 600 new cases per year for melanomas and 50 to 60 new cases per year for retinoblastoma.

In terms of primary eye cancers, there are basically

  • childhood cancers that are primarily retinoblastoma.
  • Adult cancers: the most common is choroidal melanoma, which develops inside the eye.
  • Other cancers that develop on the surface of the eye, i.e., in the conjunctiva or on the eyelids, can be both melanomas and carcinomas.

The eye is also an organ that can receive metastases from other cancers or locations of systemic lymphomas, mainly primary ocular lymphoma.

Causes

In children, retinoblastoma is related to a constitutional abnormality of the RB1 gene, a mutation present in all of their cells. Some cases are familiar but most are so-called “sporadic” cases.

In adults, the causes of choroidal melanoma are unknown,” explains Professor Cassoux.

This type of cancer is not linked to ultraviolet radiation, unlike melanomas and conjunctival carcinomas,” he adds. He added, “There is no prevention except early detection and better knowledge of the clinical signs.

People at risk

It is known that in general, eye cancer tends to affect blond people with light eyes and light skin that does not tan.

Symptoms

Retinoblastoma in children causes two basic signs:

Leucocoria, that is, a white or silvery glow in the pupil. “Parents notice this very often from the beginning because they are watching their baby so much. You can also see it in photos taken with a smartphone without the red eye filter,” explains Professor Cassoux.

The problem is that sometimes doctors and ophthalmologists do not see this reflection with the office lighting: it is, in fact, intermittent at first and then permanent at a later stage.

An eye exam with an enlarged fundus should be done as soon as the parents see this reflection.

The cross-eyed person. “You have to wring your neck before the preconceived idea that the baby’s squint is normal, insists Pr. It is not normal.

Both eyes must look in the same direction. Any strabismus in the baby requires a complete eye exam with dilation of the fundus.

If detected early, a child’s eye cancer can lead to conservative treatment to preserve the eyeball. In fact, at a late diagnosis of very advanced disease, the eye may need to be removed.

In adults, choroidal melanoma very often manifests itself by a bright flash always in the same place. This phosphene should lead to a complete ophthalmological examination with a dilated pupil in the fundus of the eye.

As the melanoma evolves and the tumor grows, the symptoms become increasingly problematic: blurred vision, visual field amputation, decreased visual acuity…

Diagnosis

The diagnosis requires a complete eye exam and a dilated fundus.

A child is never swabbed because, on the one hand, there is a risk of retinoblastoma coming out of the eye and, on the other hand, there are enough clinical signs to make a definitive diagnosis.

For melanoma (in adults): the ophthalmologist also performs a clinical examination, a fundus with dilated pupils and an ultrasound scan, which are usually sufficient to make the diagnosis.

In some cases of melanoma, a biopsy is performed to stratify the cancer and establish the risk of metastasis in order to adapt surveillance accordingly.

Treatment: How is eye cancer treated?

The treatment of eye cancer is based on ultra-precise rays (protons). This is a ray that radiates with millimetric precision and does not affect the rest of the tissue.

There are two centers specialized in proton therapy in France. Brachytherapy (iodine disc) can also be used, but is more rare.

The ablation of the eye is only performed in the case of large and very advanced tumors.

Risks and treatments for metastasis

The overall risk of metastasis is 30% at 10 years. This risk depends on the size of the tumor at the time of diagnosis, as well as the change of chromosomes in the tumor.

Patients usually do not respond to immunotherapy or chemotherapy. And liver surgery and radiofrequency are only used to prolong survival.

Eye cancer and liver metastases

Liver metastases account for 80% of eye cancer metastases. “There is no lymphatic network in the eye,” explains Professor Cassoux.

Tumor cells go into the bloodstream and, for some unknown reason, have a tropism in the liver. They stop there and fall asleep to develop two to five years after the initial diagnosis.

Life Prognosis: Is eye cancer fatal?

Today, 100% of child eye cancer cases in France can be treated.

In adults, once metastases occur, that is, in 30% of cases, the prognosis is more difficult and the cancer can be fatal if not treated properly.

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