Eye cancer is one of the rarer cancers. However, if he does appear, he is very variable: he can appear in all areas of the eye - for example, on the eyelid - and even in infancy and early childhood. The most common tumor among children is called retinoblastoma. Adults are more likely to develop choroidal melanoma. Tumors in the eye require early treatment. Often, however, they remain undetected for a long time because they cause little discomfort. All the more important are regular preventive examinations at the ophthalmologist. Some eye cancers are hereditary, others are caused by external influences (eg solar radiation). Treatment and prognosis depend on location, size and type of eye cancer. The latter can be:
This eye tumor occurs almost exclusively in children under five years. It arises when the immature retinal cells divide unchecked. A retinoblastoma proliferates very fast. It starts in the vitreous body of the eye, then the cancer spreads over the optic nerve to the brain. Either one eye (unilateral retinoblastoma) may be involved or both eyes (bilateral retinoblastoma) may be affected. However, the latter is the case, according to the Children's Cancer Foundation, only one third of the small patients. To cure the eye cancer, it must be detected early and treated. Retinoblastoma is the most common type of eye cancer in Germany among children, with around 60 children a year. However, it accounts for only two percent of all childhood cancers, which makes it one of the rarer forms of cancer.
Choroidal melanoma is the "adult counterpart" to retinoblastoma: the most common type of eye cancer, also called uveal melanoma. It arises when the pigment cells of the choroid proliferate unchecked. Read more about the causes, symptoms and treatment of choroidal melanoma in the choroidal melanoma contribution.
Eyelid tumors can be benign and malignant. The first category includes, among others, warts or fatty deposits, the malignant the aggressively growing basal cell carcinoma, the highly prone to spreading black skin cancer and the spinalioma. The main cause of eyelid tumors is UV radiation, which means that people with (very) fair skin are more vulnerable. Sunscreens with a high sun protection factor can reduce the risk of illness.
It is difficult to detect eye cancer early. For example, very small retinoblastomas do not cause discomfort for a long time, and there are no symptoms. Only when the tumor has grown larger or grows into other parts of the eye, he makes himself felt by impairing vision or even blindness.
The most common first symptom of retinoblastoma is when the pupil lights up white (leukocoria) when it is exposed to light, for example when looking into the flash of a camera. This phenomenon is also called "cat's eye", it indicates that a tumor grows behind the lens.
Other symptoms of eye cancer (retinoblastoma) may be:
- Squint: According to the information portal kinderkrebshilfe.de this symptom occurs in about 25 to 30 percent of the affected children. It may be an indication that the tumor has grown into the middle of the retina. Squinting is triggered by the different visual acuity of the eyes.
- Eye inflammation: Sometimes the affected eye hurts, is reddened or swollen. This happens when the intraocular pressure increases. If the retinoblastoma is externally irradiated, the eyes or the skin around it can begin to itch.
- Altered color of the iris: The so-called iris can discolor at one or more places.
- Reduced vision: It may happen that in a retinoblastoma the child can no longer see properly and / or focus less well and no longer or otherwise reacts to visual stimuli.
All these symptoms may or may not be indicative of retinoblastoma. Often they have relatively harmless causes. Nevertheless, you should go to the doctor with your offspring if you notice one or more of the above warning signs. Because the sooner the eye cancer is detected, the higher the chances of a successful treatment.
How to recognize eye cancer: self-tests for the home
The cat's eye, that is the illumination of a white spot in the pupil in certain light conditions, is the most important symptom of childhood eye cancer. Whether it's with their own offspring, parents can, according to children's eye cancer help with a simple test control: Make a frontal photo with flash of your child or light it with a flashlight in a slightly darkened room from the front and from the side in the eyes.
Tip: With the "White Eye Detector" app, developed by two scientists at Baylor University and Harvard Medical School, which is very easy to use, you can search the photos on your smartphone for the eyes with a white pupil. However, the Kinderkrebsstiftung warns that this app is to be understood as a helpful means of early diagnosis and not to equate with a medical diagnosis.
Almost always, retinoblastoma is diagnosed in the first two years of life. Left untreated, this disease causes death. However, if detected early, more than 95 percent of small children will survive, according to Childhood Cancer Aid.
In addition to the history, the ophthalmologist will always perform a physical examination to find evidence of a retinoblastoma. An ophthalmoscopic examination (ophthalmoscopy) under anesthesia with maximally enlarged pupil is always carried out according to the University Hospital Essen. Likewise an ultrasound examination. To detect pathological changes in the eye socket and the skull, the doctor can arrange for magnetic resonance imaging (MRI) or computed tomography. The investigations in detail:
It is the most important study to detect a retinoblastoma. In this fluoroscopy test, the doctor looks at the patient with an illuminated ophthalmoscope in the eye. Basically, this test is prescribed in the regular examinations U4 to U7.
Ultrasound and MRI
If there is actually a retinoblastoma, the spread of the eye tumor must be clarified in further investigations. According to the Children's Cancer Foundation, two of the most important methods are ultrasound (sonography) and magnetic resonance imaging (MRI). Sonography makes use of the fact that sound waves propagate at different rates in different tissues - thus the tumor can be differentiated from healthy tissue. The MRI is based on very strong magnetic fields, with the help of which sectional images of the body can be made.
If the eye cancer is already well advanced and / or chemotherapy is imminent, further investigations may become necessary. These include examinations of the cerebrospinal fluid, the bone marrow and / or the bones as well as the chest X-ray. These are only necessary in rare cases. Since a retinoblastoma can be hereditary, the parents and siblings of the ill child are also examined by ophthalmologists. A genetic test provides information about the inheritance risk. Once all examinations have been completed, the doctor decides together with the family which treatment measures are required.
Retinoblastoma is caused by a mutation in the retinoblastoma gene. This mutation can be inherited but also occur spontaneously. According to the information portal kinderkrebsinfo.de two genetic changes in the progenitor cells of the retina are the cause, the so-called retinoblasts. Only if both types of retinoblastoma gene are altered can a tumor develop. In about 60 percent of cases, the mutations are not inherited, but occurred spontaneously.
There is no scientific evidence, however, that (too much) television can cause eye cancer. There is also no correlation between eye cancer and smartphone use, according to scientists from the medical faculties of Martin Luther University Halle Wittenberg and the University of Duisburg-Essen. The ultraviolet rays in the solarium, on the other hand, are suspected to cause eye cancer.
The course of a retinoblastoma may vary widely, so treatment must be tailored to the individual patient. If the tumor is detected early and treated, this form of eye cancer is curable. If left untreated, it can be deadly.
According to the guidelines of the University Hospital Essen, after the treatment of a retinoblastoma, both eyes or the eye sockets must be checked regularly. Only in this way can new tumors be recognized in time.
According to the retinoblastoma guideline of the Essen Hospital, the distance between the examinations depends on the age of the child as well as the therapy chosen and the genetic findings. Examination under anesthesia makes sense until the fourth or fifth year of life, after which a reliable examination is usually not possible.
Due to the great advances in therapy, the risk of developing new tumors is significantly lower and the prognosis for the small patients has improved. For developed countries, five-year survival rates of 95 percent are currently reported for unilateral and bilateral retinoblastomas. All children with unilateral retinoblastoma still have a healthy eye without impaired vision. You can lead a normal life. And most children with bilateral retinoblastoma also have at least one eye with relatively good visual acuity.
If the suspicion of a retinoblastoma exists or is confirmed, an individual treatment plan is drawn up based on the findings and the overall situation after a personal consultation with the patient or, in this case, with his parents.
Basically, according to the Children's Cancer Foundation, the choice of therapy is the preservation of life above the preservation of vision. The tumor must be completely destroyed or removed. There are two treatment strategies available for this:
- Surgical removal: the tumor is removed together with the eye (enucleation)
- The eyeball-preserving therapy: In this form of therapy, the eye should be preserved and the malignant tumor growth stopped, for example by radiation and chemotherapy
In this form of therapy, the eye is surgically removed. According to children's eye cancer help, this eye cancer surgery is the most common treatment for existing retinoblastoma and the only way to completely remove the tumor.
Under general anesthesia, the doctor removes the eyeball and as long as possible part of the optic nerve. In the orbit then first a silicone ball is used and sutured muscles and conjunctiva over it.
After about ten to 14 days, swelling and possible bruising should be healed, so that the first prosthesis - a custom made artificial eye made of glass - can be used. As the muscles are sutured over the implant, it can participate in the eye movements to a limited extent.
After each enucleation, specialists examine the distant tumor tissue molecular genetically in order to determine the inheritance risk more accurately.
This treatment may be used for smaller tumors on the posterior pole and on the peripheral retina. The heat of a laser beam directed at the pupil destroys the tumor tissue. The laser coagulation is done under anesthesia.
The icing is virtually the opposite of the laser treatment, in which, however, a larger part of the healthy retina is lost. The tumor is localized during icing by means of a metal probe and repeatedly frozen.
As the tumor cells are sensitive to cold, they break down. For small tumors one session may be sufficient, larger ones usually have to be iced up several times or the therapy should be supplemented by other treatment methods. Typical side effects of icing are temporarily swollen eyelids and conjunctiva.
Treatment with beam carriers (applicators)
Single medium sized tumors can be treated under general anesthesia using an applicator. After the doctor has pushed away the conjunctiva, he sews the applicator, which is coated with radiating material, on the dermis. Once a certain radiation dose has been reached, it is removed again. How long the applicator remains on the tumor depends on its size. The advantage of this treatment is that the radiation has a targeted effect on the growth and the healthy tissue is spared. However, depending on the location of the tumor through this therapy, the lens may tarnish, retina or optic nerve may be damaged.
Here, the eye is held in position by means of vacuum contact lens and irradiated. The therapy usually runs for five weeks, with five sessions per week. If necessary, it can be used under narcosis in small children. Radiolucent retinoblastoma is converted to inactive scar tissue by this treatment.
According to the Children's Cancer Foundation, children with hereditary retinoblastoma are at an increased risk for non-ocular, ie out-of-the-eye malignant secondary tumors. One third of patients successfully treated with percutaneous radiotherapy died of a second tumor by the age of 30. The children's eye cancer help advises therefore to consider this form of therapy only as a last resort.
Certain substances (eg cyclophosphamide, carboplatin, etoposide or vincristine) are administered via a vein to treat retinoblastoma by chemotherapy. In addition, however, the tumor must be treated locally. Although it is considered certain that tumors shrink by chemotherapy. However, they are not completely destroyed, which is why this treatment form must be supplemented accordingly to cure the eye cancer.
Even after an enucleation, ie the surgical removal of the eyeball, chemotherapy may be indicated if the choroid covers a large area or the optic nerves are affected. When metastasized, so the children's eye cancer help, the survival of the children can be improved by chemotherapy.