is evidence in the literature that the incidence of iris melanoma has been increasing during the last few decades [14], which is likely related to the impact of solar radiation [2; 11; 12; 14]. [PERSON] states the average age of patients with malignant melanomas of the iris is 46 years; i.e., about 10 to 20 years younger than that of patients with malignant melanoma elsewhere in the uvea [15]. In different studies, the mean patient age varies between 45 and 65 (range 10-89 years) [1; 8; 14; 15; 16; 17; 18]. In 1992, [PERSON] et al. estimated the average patient with iris melanoma to be between 40 and 47 years of age, with few cases of patients below the age of 20 and with the risk of developing the disease increasing with age [3]. The corresponding age-adjusted incidence rates are 0.10 for men and 0.09 for women, while between 70-89 years of age, the age-specific incidence becomes higher in men (0.41) than in women (0.28) [14]. However, there is no statistically significant difference in incidence between genders in other age groups [14]. Prevalence does not seem to be different according to geographic location [13]. [PERSON] reported that iris melanomas are most frequently detected in white patients with a light-colored iris. These conditions were eight times less frequent in black people [9]. On the basis of 3680 cases analyzed in the United States, 96% of sufferers were estimated to be Caucasians, while African Americans were estimated to comprise 2% of the total, Hispanics 1% and Asians and others \(<\)1% [19]. An international, multicenter, Internet-assisted study in ophthalmic oncology demonstrated that, among 131 patients diagnosed from August 1990 to July 2010 with iris tumors, 125--the majority (95.4%)--were white [20]. A pre-existing nevus is the most common origin of iris melanoma, although de novo cases do occur [9]. In 2013, [PERSON] et al. reported that 3% of iris nevus turns into melanoma at 5 years, 4% at 10 years and 11% at 20 years based on follow-up [19]. Epidemiological, physiological and genetic data suggest that high levels of solar ultraviolet B radiation can be involved in the pathogenesis of iris melanomas, although this evidence is controversial [9]. There is also evidence that a light-colored iris and skin color are risk factors for iris melanoma [9]. The loss of chromosome 3 and loss of chromosomal region 9p21, equivalent to tumor suppressor gene CDKN2A, also play a role in developing iris melanoma [18]. Moreover, studies show that chromosomal alterations observed in iris melanoma are different from posterior uveal melanoma [9]. Iris melanomas vary in their size, shape, degree of pigmentation and clinical behavior [15] and usually present as a solitary nodular tumor [7]. The variability of pigmentation (a sign of cellular heterogeneity) is more often associated with a malignant histology, fast progression and poor prognosis [8; 15]. Pigmentation may change between being heavily diffuse, variable or amelanotic The areas of origin--from the most to the least frequent--are the peripupillary iris, the midzone and the iris periphery [7]. They can extend to the anterior and/or the posterior chamber, commonly being limited by the lens [21]. In sequential order, the most affected quadrant is the inferior (between the positions of 5 and 7 o'clock), followed by the temporal, nasal and then superior quadrant [7]. Ten percent of all iris melanomas are diffuse iris melanoma (DIM). DIM appears as a flat, infiltrating tumor with possible seeding that progressively changes the iris coloration and rate of invasion to the angle, resulting in a secondary glaucoma that responds poorly to hypotensive eye drops, causing severe disc cupping and functional loss [21]. Tapioca melanoma, which is a particular form of DIM--named for its macroscopic resemblance to tapioca pudding--is characterized by the diffuse, circumferential, neoplastic involvement of the iris and anterior chamber angle and may involve the ciliary body causing glaucoma [21].