**1. Introduction**

Malignant melanoma of uvea (iris, ciliary body, and choroid), is the most widely recognized essential intraocular danger in grown-ups. Uveal melanoma (UM) is analyzed generally in more established age, with a dynamically increasing ageexplicit frequency rate that tops close to the age of seventy. Ocular melanoma is probably going to metastasize in different lregions of the body, for example, breast, lung, kidney or liver.

There are many factors associated with the development of uveal melanoma. The most important include genetic factors, race, color of the eyes, fair coloring of the skin and the ability to tan. Many observational studies up to date have attempted to explore the relationship between sunlight exposure and risk of uveal melanoma development [1].

Usually, uveal melanomas are in early stages of their development completely asymptomatic. The comparatively low incidence of iris melanomas (anterior segment melanoma) has been attributed to the characteristic features of these tumors. Iris melanomas also rarely metastasize. Posterior melanoma - choroidal melanoma is the most common ocular melanoma type. This type is involved in over 75% of all intraocular melanomas. Iris melanoma wchis is in anterior segment is cytologically less malignant and metastatize less frequently tnak posterios uveal melanomas.

Ordinarily, choroidal melanoma is brown colored, raised mass, and the level of its pigmentation can go from dim earthy colored to thoroughly white, amelanotic.

In advanced stages the symptoms are dependent on tumor location. The most important test to establish the presence of intraocular melanoma, is the examination by an experienced clinician at specialized Ophthalmology Department. Diagnostic testing can be extremely valuable in establishing and confirming the diagnosis.

Prognosis can be influenced by number of factors. The most important are the histopathologic type of cells, the size of tumor, tumor volume, the margins of the tumor, karyotype and grading and staging by TNM Classification (e.g. extraocular extension). Cell type, however, remains the most often used predictor of outcome with genetic results.

The treatment relies upon the site of birthplace (choroid, ciliary body or iris), the size, volume and area of the injury, the general statis of the patient, age of the patient and whether extraocular attack, repeat or metastasis has happened. Extraocular augmentation, repeat, and metastasis are related with a very helpless guess and long term endurance cannot be normal [2].

Elective therapy modalities have been proposed as of late including extremist careful evacuation of the eye globe (enucleation), nearby resection, light procedures: plaque brachytherapy, charged-molecule radiotherapy, stereotactic photon bar illumination treatment or in start of the tumor transpupillary thermotherapy and photodynamic treatment.

Over the past 3–4 decades diagnostic methods have improved and radiotherapy (external beam, charged particle or brachytherapy) has become the preferred treatment for most of the patients with uveal melanoma. The aim of the treatment is to improve survival and preserve eye globe anatomically with aim to preserve the best vision in patients with uveal melanoma. Different radiation modalities are currently in use in treatment of posterior uveal melanoma in many Ophthalmology Centers. One of the methods of "conservative" approach is the stereotactic radiosurgery (SRS) by linear accelerator [2–5].

### **2. Uvea and uveal tumors**

The uveal parcel frames the center layer (or "vasculo-strong" coat) mass of the eyeball. Uvea layer is a combination of veins, pigmented cells and muscles, woven together by connective tissue. It has a nutritive capacity of the eye globe. The uveal parcel comprises of three anatomical parts, all profoundly vascular and pigmented. The noticeable part in front is the iris (part of the foremost portion of the eye) and it makes the shade of the eye globe. The iris consolidates in reverse into the ciliary body, and the ciliary body offers path to the choroid, to the back fragment of the eye globe, which is such a vascular undercoat between the sclera and the shade retina. It is substantial pigmented, along these lines engrossing light which has gone through the retina.

#### **2.1 Uvea layer**

The pigmented cells (the melanocytes) - are derived from the neural crests which have migrated to the skin and mucous membranes. Melanocytes synthesize a special organelle called a melanosome – this is responsible for the characteristic color of the skin in different races. Melanosis (melanocytosis) refers to increased pigmentation caused by hyperplasia or hypertrophy of melanocytes.

Changes in melanocytes usually cause melanomas. Melanocytes produce melanin, which is responsible for skin and hair tone. It can show up on ordinary skin or it might start as a mole or other territory that has changed in appearance. A few moles that are available upon entering the world may form into melanomas during the adulthood.

Benign tumor composed of nevus cells or melanocytes is nevus. In nevi cells contain melanosomes and are therefore capable of producing pigment melanin [1].

**13**

*Uveal Melanoma*

**2.2 Uveal melanoma**

stage of the tumor [6].

*DOI: http://dx.doi.org/10.5772/intechopen.95321*

Melanoma is a malignant tumor resulting from a transformation of melanocytes or nevus cells. It may be pigmented or non-pigmented. Melanoma is caused mainly by intense, occasional UV exposure (frequently leading to sunburn), especially in those who are genetically predisposed to the disease. Most melanomas are dark or earthy colored, however they can likewise be skin-shaded, pink, red, purple, blue or white. In the event that melanoma is perceived and treated early, it is quite often reparable, however on the off chance that it is not, the tumor can progress and spread to different pieces of the body, particularly liver, where it turns out to be difficult to treat and can be deadly. Melanomas frequently metastasize widely and

Intraocular melanoma is the most common primary ocular malignant tumor in adults and develops from uvea. Intraocular tumors might be benign or malignant. Intraocular melanoma is a quite rare type of tumor and it occurs most often in elderly people. There is lot of cases when ophthalmologists detected intraocular melanoma during a routine eye examination. The chance of recovery is depending on factors such as the size, localization and cell type of the tumor. Extraocular extension is the term used to describe the intraocular melanoma which spreads to the optic nerve or nearby tissue of the eye socket and is the sign of the advanced

Intraocular melanoma of the ciliary body and choroid (structures together called the posterior uvea), is the most common primary ocular malignant tumor in adults. Iris melanomas are a subset of uveal melanomas that tend to have a more benign course, in comparison with posterior uveal melanomas. Anterior segment melanomas have a lower incidence of metastases when compared to ciliary body and choroidal melanomas. Anterior segment melanomas account for about 15% of all uveal melanomas. The incidence of uveal melanoma increases with age and reaches a maximum between the 6th and 7th decade of life. It is more common in males and is uncommon or rare in kids and darker looking people. Uveal melanomas are infrequently two-sided. Be that as it may, the quantity of patients with two-sided inclusion is more noteworthy than would be anticipated by chance alone,

As mentioned before, choroidal melanoma represents the most common primary intraocular tumor in adults. Peak incidence is in the early 60s representing about 7.5 cases per one million populations. Incidence is rare in younger adults under 30 years of age with an estimated peak incidence of about six cases per one hundred million. Caucasians are 8 times more likely to develop the melanoma than Africans or Afro-Americans and 3 times more likely than Asians. Intraocular melanoma is arising from choroid in more than 75% of all the cases. Whether some environmental exposure triggers the development of uveal melanoma remains an open question. Sunlight has been proposed as an environmental risk factor for melanoma generally. Unlike cutaneous melanoma, incidence rates for uveal melanoma have not increased

The first step to diagnose uveal melanoma is patient's history. Patients with uveal melanoma may present with complaints of visual acuity reduction, but many can be without symptoms and the condition is discovered on routine ocular examination or by glasses prescription. In eyes with clear optic media, the diagnosis of posterior

subsequently inferring a potential hereditary inclination.

over time and last decades and it does not vary by latitude [7, 8].

uveal melanoma can be made by indirect ophthalmoscopy.

**2.3 Diagnostic method of uveal melanoma**

the regional lymph nodes, liver, lungs and brain are likely to be involved.
