Choroidal Melanoma: Causes, Symptoms & Treatment

The term choroidal melanoma refers to a malignant tumor formation in the eye. It is a primary tumor that develops directly in the eye itself and usually affects people of advanced age. Choroidal melanoma is the most common cancer of the eye.

What is uveal melanoma?

The term choroidal melanoma refers to a malignant tumor formation in the eye. Medical professionals use the term choroidal melanoma to refer to a cancer of the eye in which a malignant tumor forms. This develops directly on the choroid, from which the name of the disease is derived. It is the most common type of cancer in the eye: statistics say that it occurs about 6 times in 100000 people. The most affected persons are those who have passed the age of 50; a particular accumulation is observed between the ages of 60 and 70. The disease is often only detected at an advanced stage. Approximately half of the choroidal melanomas diagnosed are therefore not treated until metastases have already formed in other organs.

Causes

The causes of uveal melanoma are not yet fully understood. The disease develops whenever melanin-containing cells in the choroid begin to degenerate, leading to malignant tissue growth as it progresses. Scientists have not yet been able to clearly determine why this degeneration of the cells occurs in some people. It is assumed that UV radiation has an influence on the development of the cancer. However, this influence is estimated to be less than, for example, in the formation of skin cancer (cutaneous melanoma). A low presence of pigment is also thought to be related to susceptibility to cutaneous melanoma. Fair-skinned people are about 150x more likely to be affected by the disease, according to statistics.

Symptoms, complaints and signs

At first, choroidal melanoma does not cause any clear symptoms. It is only with the progression of the disease that various complaints occur, mainly affecting vision. For example, those affected often suffer from visual complaints such as double vision or blurred vision. In most cases, a shadow also appears in the visual field, a so-called scotoma. This is manifested by a subjective darkening of the visual field on one or both sides, which increases in the course of the disease and eventually leads to permanently impaired vision. In addition, choroidal melanoma can lead to distorted perception, deterioration of farsightedness, or macropsia, in which objects are perceived to be larger than they are. In isolated cases, the tumor may break through the sclera, which may be indicated by pain and acute worsening of vision. If the melanoma spreads to other parts of the body, further symptoms may occur. Depending on which part of the body is affected, there may be loss of function of the internal organs, bones or skin. The lungs and skin may also be affected. Metastasis can manifest itself in the form of pressure pain, paralysis, sensory disturbances and a general decrease in physical and mental performance.

Diagnosis and progression

Choroidal melanoma is often not noticed by affected individuals until the disease has progressed. If a physician is consulted because symptoms such as deterioration of vision or seeing shadows occur, he or she can often detect the tumor during a general examination of the eye. With the help of an ultrasound examination, the so-called echography, the size and the exact location of the tumor can be determined. If a choroidal melanoma is not treated, the visual disturbances are followed by pain and possibly detachment of the retina in the further course. If the disease progresses, the patient may lose his eye. If metastases form and spread, reaching the liver, for example, the disease can be fatal.

Complications

Treatment for uveal melanoma is absolutely necessary and should be given as soon as possible. If no treatment is given, the eye cannot be saved. Because tumors can form in the body, death occurs in the worst case. One of the common methods of treatment is radiation therapy. The eye is preserved. Complications of this treatment include the optic nerve itself receiving too high a dose of radiation.This can lead to partial or even complete loss of vision. Another treatment option is irradiation with a particle beam of nuclei of the hydrogen atom. The advantage of this is that almost only the tumor can be irradiated and the neighboring tissue is spared. However, not every tumor can be treated with this method, as it depends on the size and location of the tumor. A final treatment option is the removal of the eye (enucleation). In this case, the tumor is removed, but there is no complete certainty that daughter tumors have not already formed. The main disadvantage is, of course, the irreversible loss of vision. Pain may occur after the procedure. In addition, although rather rare, there is the possibility of the formation of a hematoma as well as an infection, which may require the removal of the inserted implant.

When should you go to the doctor?

Coupled with the fact that this is a tumor disease with a high risk of metastasis, a trip to the doctor is strongly advised. If blurred vision or generally worsened vision suddenly occurs, as is common in the case of malignant uveal melanoma, a trip to the ophthalmologist is advised. In particular, fair-skinned people who already have moles on the choroid represent a risk group. However, most choroidal melanomas are discovered by chance during routine examinations. If a choroidal melanoma is considered likely, the affected person should not refrain from having it clarified in more detail as soon as possible – also by taking a tissue sample. It has been shown that the form of uveal melanoma in which the cells have monosomy 3 is particularly prone to metastasis. Investigations for the presence of metastases, if any, should follow. Choroidal melanoma, if unfavorably located and shaped, can lead to a number of complications, most notably involving colonization of other organs with cancer cells. Accordingly, therapies against melanoma should not be postponed. Many treatment methods are available to those affected, the effectiveness of which is determined by the exact nature of the tumor. Accompanying therapy and also during ophthalmologic follow-up is an examination of the liver, because this is particularly often affected by metastasis.

Treatment and therapy

If choroidal melanoma has been diagnosed, the attending physician will initiate appropriate treatment. If the tumor has not yet grown too large, it can be treated with local radiation therapy (this is called brachytherapy). For this purpose, a radioactive platelet is sewn onto the eye under local anesthesia and remains there for up to 14 days. Alternatively, the tumor can also be treated externally with proton radiation therapy. This therapy is also suitable for larger tumors. If all types of radiation are unsuitable, the malignant tumor may also be removed surgically. If all these treatment methods fail, the eye must be completely removed (enucleation) to prevent the cancer from spreading to the rest of the organism. Even after successful treatment of uveal melanoma, a comprehensive physical examination must be performed regularly to rule out daughter tumors. Since in rare cases choroidal melanoma can itself be a daughter tumor for a cancer existing in another location, a detailed general examination is also advisable even before the start of therapy. If the disease is diagnosed and treated early enough, the chances of a complete cure are very good, since the cancer is generally confined to the eye for quite a long time before metastases are formed. In contrast, recent findings on the likelihood of metastasis from the University Hospital Essen and Charité Berlin suggest a different picture: according to these findings, uveal melanoma can form micrometastases at a very early stage, and metastasis occurs almost exclusively because of monosomy 3. Thus, it is not dependent on early detection of the tumor nor on its size.

Prospect and prognosis

In most cases, choroidal melanoma causes severe limitations in the eyes. In particular, visual disturbances develop, and a visual impairment may also be developed that was not previously present. The everyday life of the affected person is made more difficult by the choroidal melanoma.In many cases, psychological complaints also occur, as patients are afraid of complete blindness. As a rule, the affected person goes completely blind in the eye if no treatment is initiated. In many cases, treatment involves irradiation, which, however, can also damage surrounding tissue. Although the underlying disease is removed, the affected person may suffer from further visual problems. In the very worst case, the patient’s eye must be completely removed. Implants can then be used to alleviate these complaints. However, in many cases, the course of the disease is positive if treatment is initiated at an early stage. No further complications occur. Life expectancy is usually not limited, since the tumor cannot spread particularly far.

Prevention

Since uveal melanoma is a cancer whose causes are not yet precisely known, prevention in the true sense is not possible. However, if the first signs appear, such as a deterioration of vision, a doctor should be consulted promptly to determine the exact cause and, if necessary, initiate therapy.

Follow-up care

Direct follow-up is not possible in most cases of choroidal melanoma. In this case, the tumor must be removed from the eye as soon as possible to prevent further complications or metastasis. Early diagnosis and treatment of uveal melanoma have a very positive effect on the further course of the disease. As a rule, the disease is treated with the help of radiation therapy or surgery. The patient should take care of his body and rest after the therapy or surgery. Strenuous activities or other physical exertions and physical activities should be refrained from. Especially the region of the eyes must be protected and treated with care in case of choroidal melanoma. As a rule, the tumor does not spread to the other regions of the body. However, regular examinations are still very useful and should be performed by a doctor in any case. Since the tumor can have a negative effect on the vision of the affected person, the patient must wear a visual aid to counteract this discomfort. In most cases, the life expectancy of the affected person is not negatively affected by choroidal melanoma.

What you can do yourself

After being diagnosed with uveal melanoma, patients can do a few things themselves to accept the cancer and live a full life despite the disease. It is important to maintain an active lifestyle with plenty of exercise and a cancer diet that is adapted to the particular symptoms and complaints. Patients should take their own experiences into account and use them to make dietary changes that support the healing process. Possible diets include the ketogenic diet, which is composed of fat, protein and few carbohydrates and sugars, and the oil-protein diet with products containing oil and protein. Patients can work out a suitable diet together with their doctor. Just as important as a healthy and active daily routine is the exchange with other affected persons. Self-help groups for uveal melanoma patients are a first port of call where cancer patients can exchange ideas and gather information. Good alternatives are internet forums or even a counseling session with a psycho-oncologist. A uveal melanoma can be treated well nowadays and the patients should use this circumstance to increase their well-being through hobbies and a fulfilled everyday life. This also helps with recovery and alleviates pain, depression, and other side effects of the disease and therapy.