Intraocular melanoma is an aggressive malignant disease in which the tumor develops from melanocytes (cells that produce melanin pigment) in eye area. Neoplasm can be localized on eyelids, in conjunctiva, iris or choroid. This is a rare pathology among children. Ocular melanoma accounts for only 5-7% of total number of cases for this malignant tumor.
The main types of intraocular melanoma:
- Eyelid melanoma is rarest localization among eye lesions, but at the same time the most malignant. Unfortunately, it has an unfavorable prognosis and a low 5-year survival rate among patients.
- Conjunctival melanoma is also a rare and aggressive melanoma that is most often diagnosed in older patients.
- Choroidal melanom – in this case, all parts of choroid can be affected: choroid itself in 85% of cases, iris in 6% of cases and ciliary body in 9% of cases. It is this localization of melanoma that is mistakenly called retinal melanoma (retina is not affected by melanoma, since it does not contain melanocytes). This tumor location is considered less aggressive and has a better prognosis.
Important! Intraocular melanoma is quite rare in childhood. According to statistics, pathology is diagnosed in one child per 1 million. The incidence rate is 0.3% among all malignant eye tumors in children. Among all melanoma localizations, eye damage in a child accounts for only 5-7% of the total.
If your child is suspected of having ocular melanoma or has already been diagnosed, do not waste precious time. Seek highly qualified medical care from specialists who have practical experience in dealing with this disease. These are the doctors who work in Pediatric Oncology Department of Tel Aviv Medical Clinic. Thanks to coordinated work of a highly professional team of doctors, we are able to achieve success even in the most difficult cases.
All our specialists are highly qualified and have extensive practical experience in treating children with eye cancer. Most of them have repeatedly completed internships at the best oncology centers in USA and Europe, where they acquired unique knowledge and practical skills. In their work, doctors are guided by modern clinical recommendations and global treatment protocols.
Practice of a multidisciplinary approach to each case of ocular melanoma in children at TAMC is also important. Assistance to one patient is provided immediately by an entire highly professional team – oncologists, ophthalmologists, onco-ophthalmologists, radiation therapists, chemotherapists, rehabilitation specialists, pediatricians and other specialized doctors if necessary.
Causes of intraocular melanoma in children
Unfortunately, the true causes of intraocular melanoma development, like most malignant tumors, are currently unknown. It is assumed that melanocytes gain the ability to grow and reproduce uncontrollably due to certain mutations in cells.
Risk factors for developing intraocular melanoma:
- Green, gray or blue eye color.
- Light skin color.
- Age over 50-60 years.
- Patient has dysplastic nevi (those that have a high risk of degeneration into malignant melanoma).
- Long-term and intense exposure to ultraviolet radiation (natural or artificial).
- A hereditary disease is ocular melanocytosis, in which normal process of iris pigmentation is disrupted.
Symptoms of intraocular melanoma in a child
Clinical manifestations of intraocular melanoma depend entirely on tumor location and its stage. Tumors that are located on eyeball surface look like dense, dark-colored neoplasms; their surface can be smooth, or maybe bumpy, with cracks. As the tumor grows, other eye symptoms appear: eyelids swelling, blurred vision, eyes redness, lacrimation, etc. If melanoma grows inside the eye, patient’s intraocular pressure increases, pain appears in eye, vision decreases sharply, pupil shape changes, and hemorrhages appear in affected eye.
Choroid melanoma is completely asymptomatic in the first stages. At this time, it can be discovered accidentally during ophthalmoscopy (examination of fundus by an ophthalmologist). As tumor grows in eye, additional vessels appear, which can lead to retinal detachment and hemorrhages into the tumor itself, into retina, and into vitreous body of eye. Then patient develops other eye symptoms: pain, various visual disturbances, eye redness, lacrimation, increased intraocular pressure, swelling of the eyelids, etc.
If melanoma grows into orbit, eye deformation appears, its protrusion (exophthalmos). If the tumor spreads to the brain tissue, a variety of neurological symptoms occur.
At the last stage of the disease, general symptoms of cancer appear: weight loss, lack of appetite, constant fever, nausea and vomiting, headache, general weakness, complete exhaustion, signs of damage to target organs by metastases.
Modern diagnostic methods
To establish the diagnosis of ocular melanoma, a thorough ophthalmological examination is necessary. Pediatric Oncology Department at TAMC employs the best Oncology Ophthalmologists who have unique experience in diagnosing and treating a wide variety of eye cancer in children, including melanoma.
Diagnostic complex may include the following studies:
- External examination of child’s eyes.
- Ophthalmoscopy is an examination of eye fundus with a special instrument, an ophthalmoscope, which magnifies the image.
- Biomicroscopy is an examination of all internal eye structures using a special microscope. Allows us to examine in detail all internal structures of eye under magnification and detect changes specific to melanoma. The procedure is painless, non-invasive, but requires preliminary dilation of pupil with special eye drops.
- Angiography is a study of blood vessels condition; in case of tumor, certain changes are present in images. Patient is injected intravenously with a special fluorescent dye, which accumulates in intraocular vessels. Next, photographs are taken with a special camera. If there is melanoma, doctor will note the accumulation of dye in that area on pictures. Ultrasound of eyeball allows us to diagnose a tumor, but only at stages 3-4 of pathological process. Optical Coherence Tomography (OCT) allows us to obtain layer-by-layer images of eye back surface and all its internal structures. Therefore, the study is very valuable in terms of eye tumors diagnostics, including melanoma, as it allows us to identify specific changes at the earliest stages.
- Recording fundus condition using a special RetСam camera. These images can then be used to control treatment or monitor relapse of the disease.
- CT, MRI, digital radiography, bone scintigraphy, PET-CT, PET-MRI are used to diagnose tumor spread, determine its stage and to identify metastatic damage to distant organs and tissues.
As a rule, for a malignant tumor, the final diagnosis is made after a biopsy and pathological examination of a tissue sample. For melanoma, a biopsy is not recommended, since such manipulation has a high risk of spreading malignant cells. Pathohistological examination is performed after surgery to remove the tumor. Our clinic routinely conducts innovative molecular genetic studies of tumor cells, which make it possible to detect specific changes. They, in turn, can become a target for modern immunobiological drugs.
Prognosis for intraocular melanoma in a child
The prognosis for intraocular melanoma depends on its location and the stage at which the diagnosis is made.
The best prognosis is when the iris is affected, but this location of melanoma is rare.
The prognosis for uveal melanoma, if diagnosed in the early stages, is relatively favorable. The 5-year survival rate is 80%. For patients with melanoma that has spread to adjacent anatomical structures, the 5-year survival rate is 10-15%.
Modern treatment of intraocular melanoma in children
The treatment program for intraocular melanoma in a child depends on tumor location, the stage of process, concomitant diseases and general health of little patient. The main treatment method is surgery. Surgical tumor removal can be supplemented with radiation therapy, polychemotherapy, and treatment with targeted drugs (immunotherapy). These therapies can be prescribed both before and after surgery.
Surgery for uveal melanoma involves removing the tumor affected tissue and a small amount of surrounding healthy tissue. Enucleation (complete removal) of eyeball is performed only in extreme cases. Most often, TAMC doctors practice minimally invasive and organ-preserving surgical interventions. For example, it is possible to destroy a tumor using a laser, low or high temperatures: laser and diathermocoagulation, cryodestruction, etc.
Laser photocoagulation is a minimally invasive procedure that uses a special laser to heat and destroy tumor tissue. Most patients are prescribed laser photocoagulation after brachytherapy. The first session is usually performed during surgery to remove the applicator from eye. After this, patient will receive 2 more identical procedures.
Brachytherapy is a type of contact radiation therapy. With this treatment option, ophthalmologist, together with radiation oncologist, will make a small disc (applicator) to fit the eye, which will specifically irradiate the tumor. Ophthalmologist places the applicator into eye during surgery. Aapplicator is left in place for several days until melanoma has received the required amount of radiation. Patient remains in hospital in Radiation Therapy Department for the entire period of time until the applicator is removed. Once the tumor has received required amount of ionizing radiation, applicator is removed, after which patient can return home.
Proton therapy is another innovative way to aim a beam of radiation directly at the tumor. This treatment can be especially effective on tumors located near the optic nerve. To receive a course of proton therapy, patient first undergoes a small surgery to place markers on the affected eye. After this, proton therapy sessions are performed every day for two weeks.
For systemic treatment of ocular melanoma, chemotherapy and immunotherapy can be used. Their task is to destroy malignant cells and activate antitumor immunity, unmasking the tumor so that it becomes recognizable to immune system. In Onco Ophthalmology Departments of leading medical centers in Israel, including TAMC, clinical trials of new drug Selumetinib, which blocks the MEK protein, which is responsible for tumor cells growth, are actively underway. Patients participating in study are prescribed parenteral chemotherapy (Dacarbazine DTIC) in combination with Selumetinib medication.
For metastatic melanoma, the TIL protocol is used in TAMC. TIL immunotherapy is an innovative and effective method of treating patients with stage 3 and 4 melanoma, when standard treatments have little or no positive response. 50% of these patients improve with TIL therapy, and approximately 25% of patients are cured of their melanoma.
