Materiały źródłowe

• #1 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  An increased risk of uveal melanoma is most significantly associated with ocular or oculodermal melanocytosis. Individuals with light-colored eyes, fair skin, and skin that does not tan but burns easily are also at increased risk. The influence of ultraviolet (UV) light exposure remains unclear. If UV light exposure does cause an increased risk, the conferred risk is lower compared to its contribution to cutaneous melanoma. Additional risk factors include cutaneous nevi, cutaneous freckles, iris nevi, and arc welding.[1] […] Various genetic risk variants also exist. A recent discovery highlights a genetic correlation between uveal and cutaneous melanoma on chromosome 5p15.33.[2] Researchers believe that 2 potential genes in this area, telomerase reverse transcriptase (TERT) and CLPTM1 regulator of GABA type A receptor forward trafficking (CLPTM1L), may be responsible for conferring an increased risk of ocular melanoma. Recurrent mutations in the TERT promoter are associated with cutaneous melanoma. Other malignancies are associated with variants of CLPTM1L.

• #2 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  UM is the most common primary intraocular malignancy in adults, representing 3%5% of all melanomas.1,2 UM most commonly arises from choroidal melanocytes (85%90%), but can also arise from the iris (3%5%) and ciliary body (5%8%).1,3 The median age of diagnosis is approximately 62 years; however, the peak range for diagnosis is between 70 and 79 years.1,4,5 […] A variety of risk factors have been identified, including the presence of light eyes, fair skin, an inability to tan, ocular melanocytosis, dysplastic nevus syndrome, and BAP1 mutations.1013 BAP1 is a tumor-suppressor gene located on chromosome 3 that is mutated in 47% of UM.12 UM in BAP1 germ-line mutants is usually diagnosed between the ages of 30 and 59 years, and is driven by inactivating mutations in the lone functional BAP1 gene, analogous to the frequent loss of chromosome 3 observed in high-risk sporadic disease.14 Interestingly, patients with UM have an 11% higher risk of second malignancies, such as cutaneous melanoma and renal cell carcinoma, than the general population, which may be driven by the presence of germ-line BAP1 mutations.

• #3 Uveal melanoma: relatively rare but deadly cancer | Eye

  https://www.nature.com/articles/eye2016275

  Although it is a relatively rare disease, primarily found in the Caucasian population, uveal melanoma is the most common primary intraocular tumor in adults with a mean age-adjusted incidence of 5.1 cases per million per year. […] The host susceptibility factors for uveal melanoma include fair skin, light eye color, inability to tan, ocular or oculodermal melanocytosis, cutaneous or iris or choroidal nevus, and BRCA1-associated protein 1 mutation. […] The increased occurrence of uveal melanoma in lighter-skinned people and those with light (blue or grey) colored eyes may be related to less melanin being present in the choroid and retinal pigment epithelium, which results in less protection from ultraviolet light, and increased risk of developing uveal melanoma. […] Oculodermal melanocytosis represents a congenital pigmentary abnormality with slate-grey pigmentation of the periocular skin, sclera, uvea, orbit, meninges, palate, and tympanic membrane and is an important risk factor for development of uveal melanoma.

• #4 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  Unlike cutaneous melanoma, UM is genetically characterized by a small number of alterations; however, several of these alterations have been well characterized and been found to alter intracellular signaling, surface-receptor expression, and ligand production. While cutaneous melanomas are driven by MAPK activation through mutations in BRAF (~50% of cases), NRAS (10%25% of cases), or loss of function in NF1 (14% of cases), UM rarely harbors such alterations and rather is characterized by point mutations in the G-protein -subunit.35 GNAQ and GNA11 are genes that code for the -subunit of G proteins that act in conjunction with G-protein-coupled receptors. […] Van Raamsdonk et al characterized a mutually exclusive mutation pattern affecting GNAQ/GNA11 in 83% of UM samples.37,38 Further analyses have shown GNAQ/GNA11-mutation rates as high as 96% in metastatic UM samples.3840 Just as there are ethnic variations in UM incidence, there may also be ethnic differences in mutational landscape. Xu et al found a much lower GNAQ/GNA11-mutation burden of 38% in a small Chinese UM population sample.41 Recently, additional mutations that are mutually exclusive to GNAQ/GNA11 but work along similar pathways have been identified.

• #5 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11(GNA11) genes are the most common genetic variations.[3][4] In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3.[5][6] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain.

• #6 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11(GNA11) genes are the most common genetic variations.[3][4] In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3.[5][6] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain.

• #7 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  Unlike cutaneous melanoma, UM is genetically characterized by a small number of alterations; however, several of these alterations have been well characterized and been found to alter intracellular signaling, surface-receptor expression, and ligand production. While cutaneous melanomas are driven by MAPK activation through mutations in BRAF (~50% of cases), NRAS (10%25% of cases), or loss of function in NF1 (14% of cases), UM rarely harbors such alterations and rather is characterized by point mutations in the G-protein -subunit.35 GNAQ and GNA11 are genes that code for the -subunit of G proteins that act in conjunction with G-protein-coupled receptors. […] Van Raamsdonk et al characterized a mutually exclusive mutation pattern affecting GNAQ/GNA11 in 83% of UM samples.37,38 Further analyses have shown GNAQ/GNA11-mutation rates as high as 96% in metastatic UM samples.3840 Just as there are ethnic variations in UM incidence, there may also be ethnic differences in mutational landscape. Xu et al found a much lower GNAQ/GNA11-mutation burden of 38% in a small Chinese UM population sample.41 Recently, additional mutations that are mutually exclusive to GNAQ/GNA11 but work along similar pathways have been identified.

• #8 Ocular Melanoma: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily

  https://oncodaily.com/oncolibrary/cancer-types/ocular-melanoma-oncodaily

  On a molecular level, mutations in GNAQ and GNA11 are detected in up to 93% of uveal melanomas. BAP1 loss is linked with a higher risk of metastasis and poor outcomes. Conversely, EIF1AX mutations are associated with a better prognosis, while SF3B1 mutations suggest a delayed onset of metastasis. […] Though the link between UV radiation and uveal melanoma is less clear than in cutaneous melanoma, prolonged sun exposure without adequate eye protection may still contribute. Other potential risk factors include exposure to welding equipment, chemical irritants, immunosuppression, and smoking.

• #9 Melanoma in the Eye | The Law Office Of Gerald Oginski, LLC

  https://www.oginski-law.com/news/melanoma-in-the-eye.cfm

  What causes melanoma in the eye? […] Two genetic mutations GNAQ and GNA11 have long been implicated in the development of uveal melanoma, occurring in up to 80 percent of all cases of this form of cancer. […] Researchers further delved into how these mutations actually cause the cancer, Both of these genes code for what are known as G proteins, which act like molecular switches, regulating the transportation of information from the outside of cells to their cells. […] Upon further analysis of these genes, Guan and his team revealed that genetic mutations in both GNAQ and GNA11 cause the G proteins to be permanently on, letting all forms of signals to pass freely in and out of cells. This leads to the over-activation of the Yes-associated protein (YAP), a potent oncogene that has the potential to cause cancers.

• #10 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11(GNA11) genes are the most common genetic variations.[3][4] In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3.[5][6] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain.

• #11 Ocular Melanoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26124

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11 (GNA11) genes are the most common genetic variations. In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3. […] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain. Patients with an SF3B1 mutation tend to obtain a diagnosis 10 years earlier compared to those with other mutations and are more susceptible to late onset of metastases. Additional but less common genetic variations associated with uveal melanoma include phospholipase C beta 4 (PLCB4), cysteinyl leukotriene receptor 2 (CYSLTR2), RNA-binding motif protein 10 (RBM10), and SRSF2 pseudogene 1 (SRSF2).

• #12 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11(GNA11) genes are the most common genetic variations.[3][4] In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3.[5][6] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain.

• #13 Ocular Melanoma: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily

  https://oncodaily.com/oncolibrary/cancer-types/ocular-melanoma-oncodaily

  On a molecular level, mutations in GNAQ and GNA11 are detected in up to 93% of uveal melanomas. BAP1 loss is linked with a higher risk of metastasis and poor outcomes. Conversely, EIF1AX mutations are associated with a better prognosis, while SF3B1 mutations suggest a delayed onset of metastasis. […] Though the link between UV radiation and uveal melanoma is less clear than in cutaneous melanoma, prolonged sun exposure without adequate eye protection may still contribute. Other potential risk factors include exposure to welding equipment, chemical irritants, immunosuppression, and smoking.

• #14 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11(GNA11) genes are the most common genetic variations.[3][4] In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3.[5][6] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain.

• #15 Ocular melanoma. Melanoma of the eye

  https://dermnetnz.org/topics/ocular-melanoma

  Like other forms of melanoma, ocular melanoma occurs because of genetic changes within melanocytes that cause the cells to proliferate. Further changes in the cells cause them to invade surrounding tissues and to spread elsewhere round the body (metastasise). […] Many reports suggest exposure to sunlight may be an important factor in the development of ocular melanoma. […] Those most at risk of ocular melanoma are of Caucasian race and have fair skin and light iris colour. However, melanoma may also affect those with darker skin and eye colour. […] Melanosis, congenital ocular melanocytosis and neurofibromatosis are also associated with increased risk. […] Patients with conjunctival melanoma tend to have many melanocytic naevi (moles). It is thought that about 20% arise from naevi and about 60-75% within conjunctival melanosis. […] Genetic alterations associated with poorer prognosis in uveal melanoma include: Inactivation of BRCA1 associated protein-1 (BAP1); Subsequent loss of one copy of chromosome 3 (Monosomy 3), which reduces 5 year survival by 50%; Additional chromosome 8 gain.

• #16 Uveal melanoma – Wikipedia

  https://en.wikipedia.org/wiki/Uveal_melanoma

  Uveal melanoma is a type of eye cancer in the uvea of the eye. […] The cause of uveal melanoma is unclear. Uveal nevi are common (5% of Caucasians), but rarely progress to melanoma. […] Uveal melanoma is distinct from most skin melanomas associated with ultraviolet exposure; however, it shares several similarities with non-sun-exposed melanomas, such as acral melanomas and mucosal melanomas. […] BRAF mutations are extremely rare in posterior uveal melanomas; instead, uveal melanomas frequently harbor GNAQ/GNA11 mutations, a trait shared with blue nevi, nevus of Ota, and ocular melanosis. […] Other risk factors, such as blue light exposure and arc welding, have been put forward, but are still debated in the field. […] The most important genetic alteration associated with poor prognosis in uveal melanoma is inactivation of BAP1, which most often occurs through mutation of one allele and subsequent loss of an entire copy of chromosome 3 (monosomy 3) to unmask the mutant copy. […] Monosomy 3 correlates strongly with metastatic spread. […] The most accurate prognostic factor is molecular classification by gene expression profiling of uveal melanomas.

• #17 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Genetic mutations linked to iris melanoma share similarities, with rare occurrences of BAP1 and SF3B1 mutations. An alternative form of the B-Raf proto-oncogene, serine/threonine kinase (BRAF), is connected to iris, conjunctival, and cutaneous melanomas. Conjunctival melanoma involves additional mutations such as the TERT promoter, NRAS proto-oncogene (NRAS), and neurofibromin 1 (NF1). TERT genetic variations significantly impact prognosis. Although some studies reveal an association between BRCA2 pathogenic variants and ocular melanoma, others do not.[7][8].

• #18 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Genetic mutations linked to iris melanoma share similarities, with rare occurrences of BAP1 and SF3B1 mutations. An alternative form of the B-Raf proto-oncogene, serine/threonine kinase (BRAF), is connected to iris, conjunctival, and cutaneous melanomas. Conjunctival melanoma involves additional mutations such as the TERT promoter, NRAS proto-oncogene (NRAS), and neurofibromin 1 (NF1). TERT genetic variations significantly impact prognosis. Although some studies reveal an association between BRCA2 pathogenic variants and ocular melanoma, others do not.[7][8].

• #19 Risks And Causes Of Eye Cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/eye-cancer/risks-causes

  Doctors have identified a rare inherited condition called BAP1 cancer syndrome. Families with this have a change (mutation) in the BAP1 gene. People with this gene change have an increased risk of uveal melanoma, skin melanoma and some other cancers. […] Welding increases the risk of melanoma of the eye in people working as welders. This may be due to exposure to ultraviolet radiation. […] Exposure to artificial UV radiation, such as sunbeds, increases the risk of eye melanoma.

• #20 Risk Factors for Eye Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/risk-factors.html

  This is a rare inherited condition in which family members are at increased risk for eye melanoma, as well as melanoma of the skin, mesothelioma, kidney cancer, and some other cancers. […] Different types of moles (nevi) and pigmented areas in the eye have been linked with an increased risk of eye melanoma. […] Welders, who can be exposed to intense ultraviolet (UV) light when they work, have been found to have a higher risk of eye melanoma. […] Too much exposure to UV light from sunlight (or sunlamps) is a known risk factor for melanoma of the skin. It has also been suggested as a possible risk factor for melanoma of the eye, but studies so far have shown mixed results. […] Some people with eye melanoma have a history of melanoma of the skin, but its not clear if having skin melanoma increases your risk of eye melanoma.

• #21 Uveal melanoma: relatively rare but deadly cancer | Eye

  https://www.nature.com/articles/eye2016275

  An association between uveal melanoma and atypical cutaneous nevi (dysplastic nevi), common cutaneous nevi, and cutaneous freckles has been established. […] The somatic or germline mutation of BAP1 predisposes patients to develop uveal melanoma, malignant mesothelioma, cutaneous melanomas, basal cell carcinoma, and renal cell carcinoma. […] Various studies have explored the particular association between ultraviolet light exposure and occurrence of uveal melanoma. […] However, published literature does not unequivocally implicate sunlight exposure as a risk factor for uveal melanoma. […] Some studies suggest that chronic ultraviolet light exposure is an independent risk factor for uveal melanoma, whereas other studies contradict this.

• #22 Ocular Melanoma: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily

  https://oncodaily.com/oncolibrary/cancer-types/ocular-melanoma-oncodaily

  Ocular melanoma develops due to a combination of genetic, environmental, and individual factors. One of the most recognized clinical risk indicators is the presence of choroidal nevi, especially when these show features like a thickness greater than 2 mm or proximity to the optic nerve. Ocular or oculodermal melanocytosis, along with light-colored eyes, fair skin, and a history of frequent sunburns, are also associated with a higher likelihood of developing the disease. […] Familial predisposition plays a role in a minority of cases, but its significant. Germline mutations in genes such as BAP1, PALB2, and MBD4 are increasingly recognized in familial uveal melanoma. For instance, individuals with BAP1 tumor predisposition syndrome have a heightened risk not only of uveal melanoma but also of mesothelioma, renal cell carcinoma, and cutaneous melanoma.

• #23 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  UM is the most common primary intraocular malignancy in adults, representing 3%5% of all melanomas.1,2 UM most commonly arises from choroidal melanocytes (85%90%), but can also arise from the iris (3%5%) and ciliary body (5%8%).1,3 The median age of diagnosis is approximately 62 years; however, the peak range for diagnosis is between 70 and 79 years.1,4,5 […] A variety of risk factors have been identified, including the presence of light eyes, fair skin, an inability to tan, ocular melanocytosis, dysplastic nevus syndrome, and BAP1 mutations.1013 BAP1 is a tumor-suppressor gene located on chromosome 3 that is mutated in 47% of UM.12 UM in BAP1 germ-line mutants is usually diagnosed between the ages of 30 and 59 years, and is driven by inactivating mutations in the lone functional BAP1 gene, analogous to the frequent loss of chromosome 3 observed in high-risk sporadic disease.14 Interestingly, patients with UM have an 11% higher risk of second malignancies, such as cutaneous melanoma and renal cell carcinoma, than the general population, which may be driven by the presence of germ-line BAP1 mutations.

• #24 Ocular Melanoma Risk Factors – AIM at Melanoma Foundation

  https://www.aimatmelanoma.org/melanoma-101/types-of-melanoma/ocular-melanoma-riskfactors/

  There is a well-known link between uveal melanoma and errors in a gene called BAP1 that can be passed down in families. […] Other gene mutations that are passed down through families, including mutations in the PALB-2 and MBD4 genes, are also linked to uveal melanoma. […] The size and location of the original melanoma and certain genetic features can help predict how likely ocular melanoma is to spread. […] Larger tumors are at higher risk of spreading than smaller tumors. […] Among uveal melanomas, the location of the melanoma within the uveal tract is linked to how likely the tumor is to spread. […] While up to 20% of ciliary and choroidal uveal melanomas spread to other parts of the body within five years, only about 5% of iris melanomas become metastatic within the same time period. […] Certain chromosomal changes are also linked to metastasis including loss of chromosome 3 and amplification of a part of chromosome 8. […] There are other mutations seen in the primary melanoma such as SF3B1 and EiF1AX that may also predict behavior of uveal melanoma.

• #25 Ocular Melanoma: Symptoms, Causes, Stages, Diagnosis and Treatment – OncoDaily

  https://oncodaily.com/oncolibrary/cancer-types/ocular-melanoma-oncodaily

  Ocular melanoma develops due to a combination of genetic, environmental, and individual factors. One of the most recognized clinical risk indicators is the presence of choroidal nevi, especially when these show features like a thickness greater than 2 mm or proximity to the optic nerve. Ocular or oculodermal melanocytosis, along with light-colored eyes, fair skin, and a history of frequent sunburns, are also associated with a higher likelihood of developing the disease. […] Familial predisposition plays a role in a minority of cases, but its significant. Germline mutations in genes such as BAP1, PALB2, and MBD4 are increasingly recognized in familial uveal melanoma. For instance, individuals with BAP1 tumor predisposition syndrome have a heightened risk not only of uveal melanoma but also of mesothelioma, renal cell carcinoma, and cutaneous melanoma.

• #26 Uveal melanoma: relatively rare but deadly cancer | Eye

  https://www.nature.com/articles/eye2016275

  Although it is a relatively rare disease, primarily found in the Caucasian population, uveal melanoma is the most common primary intraocular tumor in adults with a mean age-adjusted incidence of 5.1 cases per million per year. […] The host susceptibility factors for uveal melanoma include fair skin, light eye color, inability to tan, ocular or oculodermal melanocytosis, cutaneous or iris or choroidal nevus, and BRCA1-associated protein 1 mutation. […] The increased occurrence of uveal melanoma in lighter-skinned people and those with light (blue or grey) colored eyes may be related to less melanin being present in the choroid and retinal pigment epithelium, which results in less protection from ultraviolet light, and increased risk of developing uveal melanoma. […] Oculodermal melanocytosis represents a congenital pigmentary abnormality with slate-grey pigmentation of the periocular skin, sclera, uvea, orbit, meninges, palate, and tympanic membrane and is an important risk factor for development of uveal melanoma.

• #27 Risks And Causes Of Eye Cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/eye-cancer/risks-causes

  We dont know what causes most eye cancers. […] The possible risk factors for eye melanoma include: […] This type is more common in older people. The average age of diagnosis is around 60 years. […] Melanoma of the eye is more common in White people than Black, Asian or Hispanic people. […] People with blue or grey eyes are more likely to develop eye melanoma than people with brown eyes. The risk is also higher in people with fair skin, or with freckles. […] People who have abnormal brown spots (pigmentation) on their uvea (called oculodermal melanocytosis) or iris (called iris naevus) are at an increased risk of developing eye melanoma. […] Some families tend to have large numbers of moles on their skin or moles that are unusual (doctors call them atypical). The atypical moles tend to be an irregular shape or colour. They also have a tendency to become cancerous. People with moles like this have a higher than average risk of skin melanoma and eye melanoma.

• #28 Eye cancer

  https://www.nhs.uk/conditions/eye-cancer/

  Eye melanoma occurs when the pigment-producing cells in the eyes divide and multiply too rapidly. This produces a lump of tissue known as a tumour. […] It’s not clear exactly why this occurs, but the following factors may increase the risk of it happening: lighter eye colour if you have blue, grey or green eyes, you have a higher risk of developing eye melanoma compared with people who have brown eyes […] white or pale skin eye melanoma mostly affects white people and is more common in those with fair skin […] unusual moles if you have irregularly shaped or unusually coloured moles, you’re more at risk of developing melanoma skin cancer and eye melanoma […] use of sunbeds there’s some evidence to suggest that exposing yourself to ultraviolet (UV) radiation from sunbeds, for example, can increase your risk of eye melanoma […] overexposure to sunlight this increases your risk of skin cancer, and may also be a risk factor for eye melanoma. The risk of developing eye melanoma also increases with age, with most cases being diagnosed in people in their 50s.

• #29 Melanoma in the Eye (Intraocular): Uveal and Conjunctival

  https://www.cancercenter.com/cancer-types/melanoma/types/intraocular-melanoma

  Melanoma in the eye starts when a cell in the eye turns cancerous. A melanoma is a cancer that starts in a specific type of pigmented cell called a melanocyte. […] Intraocular melanoma may develop in either structure. Intraocular melanoma is quite rare, but it most commonly occurs in the uveal tract. […] About 90 percent of intraocular melanomas start in the choroid or ciliary body, according to the American Cancer Society. […] The connection between sunlight exposure and intraocular melanoma is unclear and needs more research. So far, studies have found no strong correlation. […] Certain people are at a higher risk for intraocular melanoma. Having one of these factors doesnt mean someone will get cancer, but it does raise the risk. […] High-susceptibility individuals: Have fair skin and/or light-colored eyes, Have freckles, Are older, Are white, Are male (men are slightly more likely to get eye melanoma than women), Have an inherited condition, such as dysplastic nevus syndrome (these individuals have many abnormal moles), or abnormal brown spots on the iris, choroid or ciliary body, Have a specific mutation in the gene BAP1, or a family history of eye melanomas that may indicate a genetic predisposition.

• #30 Ocular Melanoma Risk Factors – AIM at Melanoma Foundation

  https://www.aimatmelanoma.org/melanoma-101/types-of-melanoma/ocular-melanoma-riskfactors/

  Ocular melanoma is rare, accounting for only about 3 to 4% of all melanomas. It can affect people of any gender, age, ethnicity and racial background. Some groups are at greater risk for ocular melanoma than others. The risk increases as people get older, and ocular melanoma is more common in people with fair skin and light eyes and in people with certain inherited medical conditions. […] The cause of ocular melanoma is not known, but several risk factors have been identified. […] Risk increases with age. It is slightly more common in men than in women. […] Ocular melanoma is more common in people with fair skin, frequent sunburns, and/or light-colored eyes. It is about 8 to 10 times more common in people of Caucasian descent compared with people of African descent. […] Although the risk factors for uveal melanoma are generally similar to the known risk factors for skin melanoma, uveal melanoma is not thought to be caused by the sun.

• #31 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  UM is the most common primary intraocular malignancy in adults, representing 3%5% of all melanomas.1,2 UM most commonly arises from choroidal melanocytes (85%90%), but can also arise from the iris (3%5%) and ciliary body (5%8%).1,3 The median age of diagnosis is approximately 62 years; however, the peak range for diagnosis is between 70 and 79 years.1,4,5 […] A variety of risk factors have been identified, including the presence of light eyes, fair skin, an inability to tan, ocular melanocytosis, dysplastic nevus syndrome, and BAP1 mutations.1013 BAP1 is a tumor-suppressor gene located on chromosome 3 that is mutated in 47% of UM.12 UM in BAP1 germ-line mutants is usually diagnosed between the ages of 30 and 59 years, and is driven by inactivating mutations in the lone functional BAP1 gene, analogous to the frequent loss of chromosome 3 observed in high-risk sporadic disease.14 Interestingly, patients with UM have an 11% higher risk of second malignancies, such as cutaneous melanoma and renal cell carcinoma, than the general population, which may be driven by the presence of germ-line BAP1 mutations.

• #32 Risks And Causes Of Eye Cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/eye-cancer/risks-causes

  We dont know what causes most eye cancers. […] The possible risk factors for eye melanoma include: […] This type is more common in older people. The average age of diagnosis is around 60 years. […] Melanoma of the eye is more common in White people than Black, Asian or Hispanic people. […] People with blue or grey eyes are more likely to develop eye melanoma than people with brown eyes. The risk is also higher in people with fair skin, or with freckles. […] People who have abnormal brown spots (pigmentation) on their uvea (called oculodermal melanocytosis) or iris (called iris naevus) are at an increased risk of developing eye melanoma. […] Some families tend to have large numbers of moles on their skin or moles that are unusual (doctors call them atypical). The atypical moles tend to be an irregular shape or colour. They also have a tendency to become cancerous. People with moles like this have a higher than average risk of skin melanoma and eye melanoma.

• #33 Risk Factors for Eye Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/risk-factors.html

  There are a few known risk factors for eye cancer, particularly melanoma of the eye (also known as ocular melanoma or intraocular melanoma). […] The risk of eye melanoma is higher in people with lighter colored skin (and who sunburn or freckle easily) than in people with darker skin tones. […] People with light colored eyes are somewhat more likely to develop melanoma of the eye than are people with darker eye color. […] Eye melanomas can occur at any age, but the risk goes up as people get older. […] Eye melanoma is slightly more common in men than in women. […] People with this inherited condition have many abnormal moles (dysplastic nevi) on their skin. […] People with this syndrome are at increased risk of skin melanoma. They also seem to have a higher risk of developing melanoma of the eye.

• #34 Ocular Melanoma Risk Factors – AIM at Melanoma Foundation

  https://www.aimatmelanoma.org/melanoma-101/types-of-melanoma/ocular-melanoma-riskfactors/

  Ocular melanoma is rare, accounting for only about 3 to 4% of all melanomas. It can affect people of any gender, age, ethnicity and racial background. Some groups are at greater risk for ocular melanoma than others. The risk increases as people get older, and ocular melanoma is more common in people with fair skin and light eyes and in people with certain inherited medical conditions. […] The cause of ocular melanoma is not known, but several risk factors have been identified. […] Risk increases with age. It is slightly more common in men than in women. […] Ocular melanoma is more common in people with fair skin, frequent sunburns, and/or light-colored eyes. It is about 8 to 10 times more common in people of Caucasian descent compared with people of African descent. […] Although the risk factors for uveal melanoma are generally similar to the known risk factors for skin melanoma, uveal melanoma is not thought to be caused by the sun.

• #35 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  An increased risk of uveal melanoma is most significantly associated with ocular or oculodermal melanocytosis. Individuals with light-colored eyes, fair skin, and skin that does not tan but burns easily are also at increased risk. The influence of ultraviolet (UV) light exposure remains unclear. If UV light exposure does cause an increased risk, the conferred risk is lower compared to its contribution to cutaneous melanoma. Additional risk factors include cutaneous nevi, cutaneous freckles, iris nevi, and arc welding.[1] […] Various genetic risk variants also exist. A recent discovery highlights a genetic correlation between uveal and cutaneous melanoma on chromosome 5p15.33.[2] Researchers believe that 2 potential genes in this area, telomerase reverse transcriptase (TERT) and CLPTM1 regulator of GABA type A receptor forward trafficking (CLPTM1L), may be responsible for conferring an increased risk of ocular melanoma. Recurrent mutations in the TERT promoter are associated with cutaneous melanoma. Other malignancies are associated with variants of CLPTM1L.

• #36 Uveal melanoma: relatively rare but deadly cancer | Eye

  https://www.nature.com/articles/eye2016275

  Although it is a relatively rare disease, primarily found in the Caucasian population, uveal melanoma is the most common primary intraocular tumor in adults with a mean age-adjusted incidence of 5.1 cases per million per year. […] The host susceptibility factors for uveal melanoma include fair skin, light eye color, inability to tan, ocular or oculodermal melanocytosis, cutaneous or iris or choroidal nevus, and BRCA1-associated protein 1 mutation. […] The increased occurrence of uveal melanoma in lighter-skinned people and those with light (blue or grey) colored eyes may be related to less melanin being present in the choroid and retinal pigment epithelium, which results in less protection from ultraviolet light, and increased risk of developing uveal melanoma. […] Oculodermal melanocytosis represents a congenital pigmentary abnormality with slate-grey pigmentation of the periocular skin, sclera, uvea, orbit, meninges, palate, and tympanic membrane and is an important risk factor for development of uveal melanoma.

• #37 Eye cancer (ocular melanoma) – symptoms, staging, treatment | Macmillan Cancer Support

  https://www.macmillan.org.uk/cancer-information-and-support/melanoma/eye-cancer

  Eye melanoma is the most common type of eye cancer. […] Doctors do not know what causes eye melanoma. But there are risk factors that can increase the chance of developing it. […] Uveal melanoma is more common in people with skin that burns easily, fair or red hair, and blue eyes. It is rare in people with brown eyes or if you are Black or Asian. […] The main risk factor for skin melanoma is exposure to ultraviolet rays (UV rays). But it is not clear whether there is a link between UV ray exposure and uveal melanoma. There is some evidence linking conjunctival melanoma to exposure to UV rays through sunlight, sunbeds or sunlamps. […] Eye melanoma is more common in people who have atypical mole syndrome. People with this condition usually have more than 100 moles on their body. […] Conjunctival melanoma usually develops from a rare condition called primary acquired melanosis (PAM). This causes many brown or dark patches (pigmentation) on the conjunctiva, usually in one eye. PAM can be monitored so that any signs of it changing are found early and treated.

• #38 Ocular melanoma. Melanoma of the eye

  https://dermnetnz.org/topics/ocular-melanoma

  Like other forms of melanoma, ocular melanoma occurs because of genetic changes within melanocytes that cause the cells to proliferate. Further changes in the cells cause them to invade surrounding tissues and to spread elsewhere round the body (metastasise). […] Many reports suggest exposure to sunlight may be an important factor in the development of ocular melanoma. […] Those most at risk of ocular melanoma are of Caucasian race and have fair skin and light iris colour. However, melanoma may also affect those with darker skin and eye colour. […] Melanosis, congenital ocular melanocytosis and neurofibromatosis are also associated with increased risk. […] Patients with conjunctival melanoma tend to have many melanocytic naevi (moles). It is thought that about 20% arise from naevi and about 60-75% within conjunctival melanosis. […] Genetic alterations associated with poorer prognosis in uveal melanoma include: Inactivation of BRCA1 associated protein-1 (BAP1); Subsequent loss of one copy of chromosome 3 (Monosomy 3), which reduces 5 year survival by 50%; Additional chromosome 8 gain.

• #39 Risk Factors for Eye Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/risk-factors.html

  There are a few known risk factors for eye cancer, particularly melanoma of the eye (also known as ocular melanoma or intraocular melanoma). […] The risk of eye melanoma is higher in people with lighter colored skin (and who sunburn or freckle easily) than in people with darker skin tones. […] People with light colored eyes are somewhat more likely to develop melanoma of the eye than are people with darker eye color. […] Eye melanomas can occur at any age, but the risk goes up as people get older. […] Eye melanoma is slightly more common in men than in women. […] People with this inherited condition have many abnormal moles (dysplastic nevi) on their skin. […] People with this syndrome are at increased risk of skin melanoma. They also seem to have a higher risk of developing melanoma of the eye.

• #40 Risks And Causes Of Eye Cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/eye-cancer/risks-causes

  We dont know what causes most eye cancers. […] The possible risk factors for eye melanoma include: […] This type is more common in older people. The average age of diagnosis is around 60 years. […] Melanoma of the eye is more common in White people than Black, Asian or Hispanic people. […] People with blue or grey eyes are more likely to develop eye melanoma than people with brown eyes. The risk is also higher in people with fair skin, or with freckles. […] People who have abnormal brown spots (pigmentation) on their uvea (called oculodermal melanocytosis) or iris (called iris naevus) are at an increased risk of developing eye melanoma. […] Some families tend to have large numbers of moles on their skin or moles that are unusual (doctors call them atypical). The atypical moles tend to be an irregular shape or colour. They also have a tendency to become cancerous. People with moles like this have a higher than average risk of skin melanoma and eye melanoma.

• #41 Eye melanoma: Symptoms, causes, and risk factors

  https://www.medicalnewstoday.com/articles/183858

  Eye melanoma is usually a secondary cancer, meaning that it begins in a different location in the body and spreads to the eye. In fact, around 9 out of 10 eye melanomas begin in the skin. […] Experts are not sure exactly what causes ocular melanoma. […] Scientists have found links between eye cancer and some genetic changes, but it remains unclear exactly where these changes occur, or if they cause cancer to develop. […] The following factors seem to increase the risk of developing eye melanoma: Eye color: People with blue or green eyes have a higher risk of developing ocular melanoma than those with brown eyes. […] Exposure to ultraviolet (UV) light: Exposure to UV light may increase the risk of conjunctival melanoma. This is a melanoma that occurs on the surface of the eye. […] Dysplastic nevus syndrome: In this condition, a person develops atypical moles, known as dysplastic nevi. These are different to ordinary moles. They have irregular borders, may contain several different colors, and often appear in clusters. Dysplastic nevi are more likely to develop into malignant melanomas than ordinary moles. […] Ethnicity: Eye melanoma is most likely to develop in white people. […] Scientists have not yet proven that sunshine and outdoor work cause eye cancer, but they may increase the risk.

• #42 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  An increased risk of uveal melanoma is most significantly associated with ocular or oculodermal melanocytosis. Individuals with light-colored eyes, fair skin, and skin that does not tan but burns easily are also at increased risk. The influence of ultraviolet (UV) light exposure remains unclear. If UV light exposure does cause an increased risk, the conferred risk is lower compared to its contribution to cutaneous melanoma. Additional risk factors include cutaneous nevi, cutaneous freckles, iris nevi, and arc welding.[1] […] Various genetic risk variants also exist. A recent discovery highlights a genetic correlation between uveal and cutaneous melanoma on chromosome 5p15.33.[2] Researchers believe that 2 potential genes in this area, telomerase reverse transcriptase (TERT) and CLPTM1 regulator of GABA type A receptor forward trafficking (CLPTM1L), may be responsible for conferring an increased risk of ocular melanoma. Recurrent mutations in the TERT promoter are associated with cutaneous melanoma. Other malignancies are associated with variants of CLPTM1L.

• #43 Risk Factors for Eye Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/risk-factors.html

  This is a rare inherited condition in which family members are at increased risk for eye melanoma, as well as melanoma of the skin, mesothelioma, kidney cancer, and some other cancers. […] Different types of moles (nevi) and pigmented areas in the eye have been linked with an increased risk of eye melanoma. […] Welders, who can be exposed to intense ultraviolet (UV) light when they work, have been found to have a higher risk of eye melanoma. […] Too much exposure to UV light from sunlight (or sunlamps) is a known risk factor for melanoma of the skin. It has also been suggested as a possible risk factor for melanoma of the eye, but studies so far have shown mixed results. […] Some people with eye melanoma have a history of melanoma of the skin, but its not clear if having skin melanoma increases your risk of eye melanoma.

• #44 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  The role of sun exposure as a risk factor for UM is unclear. Tucker et al found that time spent outdoors, tanning, and rare use of ocular sun protection were risk factors for the development of UM.15 A subsequent meta-analysis found that chronic ultraviolet-light exposure and geographic latitude were not associated with disease, but identified welding as a risk factor.16 Interestingly, a recent study found that posterior choroidal melanomas occurring in illuminated areas were associated with frequent adenine-to-cytosine mutations, whereas ciliochoroidal melanoma arising from unilluminated areas are associated with frequent adenine-to-thymine mutations and light eye color. This suggests both light eye color and sunlight may be independent risk factors associated with different anatomic and mutation profiles.17

• #45 What is ocular melanoma? – Mayo Clinic News Network

  https://newsnetwork.mayoclinic.org/discussion/what-is-ocular-melanoma/

  Exposure to ultraviolet (UV) light. The role of ultraviolet exposure in eye melanoma is unclear. There’s some evidence that exposure to UV light, such as light from the sun or from tanning beds, may increase the risk of eye melanoma. […] Certain genetic mutations. Certain genes passed from parents to children may increase the risk of eye melanoma.

• #46 Ocular Melanoma Risk Factors – AIM at Melanoma Foundation

  https://www.aimatmelanoma.org/melanoma-101/types-of-melanoma/ocular-melanoma-riskfactors/

  People who work with welding may be at higher risk for ocular melanoma. […] Certain inherited medical conditions are linked to having a higher risk of ocular melanoma. People with ocular or oculodermal melanocytosis (pigmentation around the eye and forehead, also called Nevus of Ota) have a higher risk of uveal melanoma, while those with primary acquired melanosis or conjunctival nevi are at higher risk for conjunctival melanoma. […] Exposure to UV rays from the sun is a well-known risk factor for skin melanoma. There is some evidence linking UV exposure to some of the subtypes ocular melanoma (possibly uveal melanoma of the iris and conjunctival melanoma), but the majority of ocular melanomas are not linked to UV exposure. […] Uveal melanoma can run in families, although this is not common. Hereditary uveal melanoma is caused by specific inherited DNA errors, called mutations.

• #47 Uveal melanoma: relatively rare but deadly cancer | Eye

  https://www.nature.com/articles/eye2016275

  An association between uveal melanoma and atypical cutaneous nevi (dysplastic nevi), common cutaneous nevi, and cutaneous freckles has been established. […] The somatic or germline mutation of BAP1 predisposes patients to develop uveal melanoma, malignant mesothelioma, cutaneous melanomas, basal cell carcinoma, and renal cell carcinoma. […] Various studies have explored the particular association between ultraviolet light exposure and occurrence of uveal melanoma. […] However, published literature does not unequivocally implicate sunlight exposure as a risk factor for uveal melanoma. […] Some studies suggest that chronic ultraviolet light exposure is an independent risk factor for uveal melanoma, whereas other studies contradict this.

• #48 Risks And Causes Of Eye Cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/eye-cancer/risks-causes

  Doctors have identified a rare inherited condition called BAP1 cancer syndrome. Families with this have a change (mutation) in the BAP1 gene. People with this gene change have an increased risk of uveal melanoma, skin melanoma and some other cancers. […] Welding increases the risk of melanoma of the eye in people working as welders. This may be due to exposure to ultraviolet radiation. […] Exposure to artificial UV radiation, such as sunbeds, increases the risk of eye melanoma.

• #49 Eye cancer | NHS inform

  https://www.nhsinform.scot/illnesses-and-conditions/cancer/cancer-types-in-adults/eye-cancer/

  Eye melanoma occurs when the pigment-producing cells in the eyes divide and multiply too rapidly. This produces a lump of tissue known as a tumour. […] Its not clear exactly why this occurs, but the following factors may increase the risk of it happening: lighter eye colour if you have blue, grey or green eyes, you have a higher risk of developing eye melanoma compared with people who have brown eyes […] white or pale skin eye melanoma mostly affects white people and is more common in those with fair skin […] unusual moles if you have irregularly shaped or unusually coloured moles, youre more at risk of developing skin cancer and eye melanoma […] use of sunbeds theres some evidence to suggest that exposing yourself to ultraviolet (UV) radiation from sunbeds, for example, can increase your risk of eye melanoma […] overexposure to sunlight this increases your risk of skin cancer, and may also be a risk factor for eye melanoma. The risk of developing eye melanoma also increases with age, with most cases being diagnosed in people in their fifties.

• #50 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Causes-of-eye-cancer.aspx

  There are no definitive causes of eye cancers. […] However, there are certain risk factors that are associated with eye cancers. […] There is however no direct association with these risk factors. […] Intraocular melanoma is the commonest form of eye cancer. […] The commonly associated risk factors of this type of eye cancer are (1, 2, 3, 4):- Race or ethnicity Caucasians or whites are at a higher risk of melanomas than African Americans or Asian Americans. […] Age Primary intraocular melanoma commonly occurs after the age of 50. […] Colors of the eyes or iris Those with light colored eyes have raised risk of intraocular melanoma. […] Sun exposure Too much sun exposure or exposure to ultraviolet (UV) rays (either from the sun or tanning beds) raises risk of skin melanomas. […] Inherited conditions Those with conditions like dysplastic nevus syndrome are at a higher risk of eye melanomas. […] Occupational hazards Workers like farmers, fishermen, welders, or chemical and laundry workers have a greater risk of getting eye melanomas.

• #51 Risk Factors for Eye Cancer | American Cancer Society

  https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/risk-factors.html

  This is a rare inherited condition in which family members are at increased risk for eye melanoma, as well as melanoma of the skin, mesothelioma, kidney cancer, and some other cancers. […] Different types of moles (nevi) and pigmented areas in the eye have been linked with an increased risk of eye melanoma. […] Welders, who can be exposed to intense ultraviolet (UV) light when they work, have been found to have a higher risk of eye melanoma. […] Too much exposure to UV light from sunlight (or sunlamps) is a known risk factor for melanoma of the skin. It has also been suggested as a possible risk factor for melanoma of the eye, but studies so far have shown mixed results. […] Some people with eye melanoma have a history of melanoma of the skin, but its not clear if having skin melanoma increases your risk of eye melanoma.

• #52 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  The role of sun exposure as a risk factor for UM is unclear. Tucker et al found that time spent outdoors, tanning, and rare use of ocular sun protection were risk factors for the development of UM.15 A subsequent meta-analysis found that chronic ultraviolet-light exposure and geographic latitude were not associated with disease, but identified welding as a risk factor.16 Interestingly, a recent study found that posterior choroidal melanomas occurring in illuminated areas were associated with frequent adenine-to-cytosine mutations, whereas ciliochoroidal melanoma arising from unilluminated areas are associated with frequent adenine-to-thymine mutations and light eye color. This suggests both light eye color and sunlight may be independent risk factors associated with different anatomic and mutation profiles.17

• #53

  https://www.cancervic.org.au/cancer-information/types-of-cancer/eye_cancer/eye-cancer-overview.html

  certain skin conditions and pigmentation some people have a skin disorder (dysplastic naevus syndrome) which causes moles to grow abnormally, and this can increase your risk. […] occupational exposure being exposed to UV radiation or certain chemicals at work, particularly through welding, can increase your risk.

• #54 Melanoma of the eye Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/melanoma-of-the-eye

  Melanoma is a very aggressive type of cancer that can spread rapidly. […] Too much exposure to sunlight is an important risk factor for melanoma. People who have fair skin and blue eyes are most affected. […] The choroid layer is the most likely site of melanoma in the eye. This is the layer of blood vessels and connective tissue between the white of the eye and retina (back of the eye). […] Melanoma can also begin on the skin or other organs in the body and spread to the eye.

• #55 Intraocular Melanoma: Treatment, Symptoms & Definition

  https://my.clevelandclinic.org/health/diseases/6134-intraocular-melanoma

  Intraocular melanoma is a rare cancer that forms inside the eye. It causes melanocytes (cells that produce pigment, or color) to grow out of control. […] With intraocular melanoma, mutated DNA give the wrong instructions to melanocytes in the eye. The melanocytes grow and form tumors. […] The following risk factors make you more likely to develop eye melanoma: Being white, light-colored eyes or skin, being between 70 and 80 years old, family history of uveal melanoma (rare). […] Research shows that intraocular melanoma spreads to other parts of the body in about 40% to 50% of cases. In about 90% of those cases, the cancer spreads to the liver.

• #56 Melanoma in the Eye (Intraocular): Uveal and Conjunctival

  https://www.cancercenter.com/cancer-types/melanoma/types/intraocular-melanoma

  Melanoma in the eye starts when a cell in the eye turns cancerous. A melanoma is a cancer that starts in a specific type of pigmented cell called a melanocyte. […] Intraocular melanoma may develop in either structure. Intraocular melanoma is quite rare, but it most commonly occurs in the uveal tract. […] About 90 percent of intraocular melanomas start in the choroid or ciliary body, according to the American Cancer Society. […] The connection between sunlight exposure and intraocular melanoma is unclear and needs more research. So far, studies have found no strong correlation. […] Certain people are at a higher risk for intraocular melanoma. Having one of these factors doesnt mean someone will get cancer, but it does raise the risk. […] High-susceptibility individuals: Have fair skin and/or light-colored eyes, Have freckles, Are older, Are white, Are male (men are slightly more likely to get eye melanoma than women), Have an inherited condition, such as dysplastic nevus syndrome (these individuals have many abnormal moles), or abnormal brown spots on the iris, choroid or ciliary body, Have a specific mutation in the gene BAP1, or a family history of eye melanomas that may indicate a genetic predisposition.

• #57 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  UM is the most common primary intraocular malignancy in adults, representing 3%5% of all melanomas.1,2 UM most commonly arises from choroidal melanocytes (85%90%), but can also arise from the iris (3%5%) and ciliary body (5%8%).1,3 The median age of diagnosis is approximately 62 years; however, the peak range for diagnosis is between 70 and 79 years.1,4,5 […] A variety of risk factors have been identified, including the presence of light eyes, fair skin, an inability to tan, ocular melanocytosis, dysplastic nevus syndrome, and BAP1 mutations.1013 BAP1 is a tumor-suppressor gene located on chromosome 3 that is mutated in 47% of UM.12 UM in BAP1 germ-line mutants is usually diagnosed between the ages of 30 and 59 years, and is driven by inactivating mutations in the lone functional BAP1 gene, analogous to the frequent loss of chromosome 3 observed in high-risk sporadic disease.14 Interestingly, patients with UM have an 11% higher risk of second malignancies, such as cutaneous melanoma and renal cell carcinoma, than the general population, which may be driven by the presence of germ-line BAP1 mutations.

• #58 Ocular Melanoma Risk Factors – AIM at Melanoma Foundation

  https://www.aimatmelanoma.org/melanoma-101/types-of-melanoma/ocular-melanoma-riskfactors/

  There is a well-known link between uveal melanoma and errors in a gene called BAP1 that can be passed down in families. […] Other gene mutations that are passed down through families, including mutations in the PALB-2 and MBD4 genes, are also linked to uveal melanoma. […] The size and location of the original melanoma and certain genetic features can help predict how likely ocular melanoma is to spread. […] Larger tumors are at higher risk of spreading than smaller tumors. […] Among uveal melanomas, the location of the melanoma within the uveal tract is linked to how likely the tumor is to spread. […] While up to 20% of ciliary and choroidal uveal melanomas spread to other parts of the body within five years, only about 5% of iris melanomas become metastatic within the same time period. […] Certain chromosomal changes are also linked to metastasis including loss of chromosome 3 and amplification of a part of chromosome 8. […] There are other mutations seen in the primary melanoma such as SF3B1 and EiF1AX that may also predict behavior of uveal melanoma.

• #59 Uveal Melanoma | Skin Cancer | UPMC Hillman Cancer Center

  https://hillman.upmc.com/cancer-care/melanoma-skin/types/uveal

  Uveal melanoma is a rare type of cancer that occurs in the tissues of the eye. […] The cause of uveal melanoma is not yet known. […] Some factors that may increase the risk of getting this type of cancer include: Having fair skin. Having freckles. Skin that burns easily and does not tan. Having blue or green eyes. Older age. […] Uveal melanoma of the ciliary body and choroid is more likely to spread than uveal melanoma of the iris.

• #60 Conjunctival Melanoma: Terminology, Introduction, Etiology

  https://emedicine.medscape.com/article/1191840-overview

  Together with mucus-secreting goblet cells within the stratified epithelium, melanocytic cells are found in the basal layer of the conjunctiva. These melanocytic cells are of neuroectodermal origin, and melanocytic tumors may arise from these cells. […] Theoretically, conjunctival melanoma may originate from primary acquired melanosis, preexisting nevi, or de novo lesions (without any histologic or clinical evidence of a preexisting lesion). It may be challenging to determine the precursor lesion in many cases. […] Approximately 50% to 75% of cases of conjunctival melanoma arise in a setting of primary acquired melanosis. Typically, primary acquired melanosis is found in middle-aged whites; along with malignant melanomas, it is infrequent in the younger population. […] Evidence indicates that approximately 20% to 25% of patients with conjunctival melanoma have a history or microscopic evidence of a benign conjunctival nevus. […] Approximately 25% of cases of conjunctival melanoma come from de novo lesions. These lesions can be ulcerative, amelanotic, papillary, or fungating.

• #61 Conjunctival Melanoma: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/24895-conjunctival-melanoma

  Because conjunctival melanoma is so rare, researchers aren’t sure what causes it. Genetics may play a role, but it’s not contagious and doesn’t spread from one person to another. Two conditions have a more defined link to developing conjunctival melanoma. These are: […] Scientists think exposure to ultraviolet light may also be a risk factor.

• #62 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  Unlike cutaneous melanoma, UM is genetically characterized by a small number of alterations; however, several of these alterations have been well characterized and been found to alter intracellular signaling, surface-receptor expression, and ligand production. While cutaneous melanomas are driven by MAPK activation through mutations in BRAF (~50% of cases), NRAS (10%25% of cases), or loss of function in NF1 (14% of cases), UM rarely harbors such alterations and rather is characterized by point mutations in the G-protein -subunit.35 GNAQ and GNA11 are genes that code for the -subunit of G proteins that act in conjunction with G-protein-coupled receptors. […] Van Raamsdonk et al characterized a mutually exclusive mutation pattern affecting GNAQ/GNA11 in 83% of UM samples.37,38 Further analyses have shown GNAQ/GNA11-mutation rates as high as 96% in metastatic UM samples.3840 Just as there are ethnic variations in UM incidence, there may also be ethnic differences in mutational landscape. Xu et al found a much lower GNAQ/GNA11-mutation burden of 38% in a small Chinese UM population sample.41 Recently, additional mutations that are mutually exclusive to GNAQ/GNA11 but work along similar pathways have been identified.

• #63 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11(GNA11) genes are the most common genetic variations.[3][4] In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3.[5][6] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain.

• #64 Melanoma in the Eye | The Law Office Of Gerald Oginski, LLC

  https://www.oginski-law.com/news/melanoma-in-the-eye.cfm

  What causes melanoma in the eye? […] Two genetic mutations GNAQ and GNA11 have long been implicated in the development of uveal melanoma, occurring in up to 80 percent of all cases of this form of cancer. […] Researchers further delved into how these mutations actually cause the cancer, Both of these genes code for what are known as G proteins, which act like molecular switches, regulating the transportation of information from the outside of cells to their cells. […] Upon further analysis of these genes, Guan and his team revealed that genetic mutations in both GNAQ and GNA11 cause the G proteins to be permanently on, letting all forms of signals to pass freely in and out of cells. This leads to the over-activation of the Yes-associated protein (YAP), a potent oncogene that has the potential to cause cancers.

• #65 Ocular melanoma. Melanoma of the eye

  https://dermnetnz.org/topics/ocular-melanoma

  Like other forms of melanoma, ocular melanoma occurs because of genetic changes within melanocytes that cause the cells to proliferate. Further changes in the cells cause them to invade surrounding tissues and to spread elsewhere round the body (metastasise). […] Many reports suggest exposure to sunlight may be an important factor in the development of ocular melanoma. […] Those most at risk of ocular melanoma are of Caucasian race and have fair skin and light iris colour. However, melanoma may also affect those with darker skin and eye colour. […] Melanosis, congenital ocular melanocytosis and neurofibromatosis are also associated with increased risk. […] Patients with conjunctival melanoma tend to have many melanocytic naevi (moles). It is thought that about 20% arise from naevi and about 60-75% within conjunctival melanosis. […] Genetic alterations associated with poorer prognosis in uveal melanoma include: Inactivation of BRCA1 associated protein-1 (BAP1); Subsequent loss of one copy of chromosome 3 (Monosomy 3), which reduces 5 year survival by 50%; Additional chromosome 8 gain.

• #66 Ocular Melanoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/26124

  Present in 71% to 93% of patients affected by uveal melanoma, variations in the G-protein subunit alpha q (GNAQ) and G-protein subunit alpha 11 (GNA11) genes are the most common genetic variations. In addition, variations in BRCA1-associated protein 1 (BAP1), splicing factor 3b subunit 1 (SF3B1), and eukaryotic translation initiation factor 1A X-linked (EIF1AX) are also frequently encountered mutations. […] Genetic variations within BAP1, SF3B1, and EIF1AX affect prognosis. Patients with a BAP1 mutation or lacking BAP1 expression have a high risk of developing metastatic disease. An EIF1AX mutation is associated with a low risk of metastatic disease and a better prognosis. Additional indicators of poor prognosis include a gain of chromosome 8q and a loss of chromosome 3 or monosomy 3. […] Other chromosomal variations found in uveal melanoma are chromosome 1p loss and chromosome 6 gain. Patients with an SF3B1 mutation tend to obtain a diagnosis 10 years earlier compared to those with other mutations and are more susceptible to late onset of metastases. Additional but less common genetic variations associated with uveal melanoma include phospholipase C beta 4 (PLCB4), cysteinyl leukotriene receptor 2 (CYSLTR2), RNA-binding motif protein 10 (RBM10), and SRSF2 pseudogene 1 (SRSF2).

• #67 Uveal melanoma – Wikipedia

  https://en.wikipedia.org/wiki/Uveal_melanoma

  Uveal melanoma is a type of eye cancer in the uvea of the eye. […] The cause of uveal melanoma is unclear. Uveal nevi are common (5% of Caucasians), but rarely progress to melanoma. […] Uveal melanoma is distinct from most skin melanomas associated with ultraviolet exposure; however, it shares several similarities with non-sun-exposed melanomas, such as acral melanomas and mucosal melanomas. […] BRAF mutations are extremely rare in posterior uveal melanomas; instead, uveal melanomas frequently harbor GNAQ/GNA11 mutations, a trait shared with blue nevi, nevus of Ota, and ocular melanosis. […] Other risk factors, such as blue light exposure and arc welding, have been put forward, but are still debated in the field. […] The most important genetic alteration associated with poor prognosis in uveal melanoma is inactivation of BAP1, which most often occurs through mutation of one allele and subsequent loss of an entire copy of chromosome 3 (monosomy 3) to unmask the mutant copy. […] Monosomy 3 correlates strongly with metastatic spread. […] The most accurate prognostic factor is molecular classification by gene expression profiling of uveal melanomas.

• #68 Eye melanoma: Symptoms, causes, and risk factors

  https://www.medicalnewstoday.com/articles/183858

  Eye melanoma is usually a secondary cancer, meaning that it begins in a different location in the body and spreads to the eye. In fact, around 9 out of 10 eye melanomas begin in the skin. […] Experts are not sure exactly what causes ocular melanoma. […] Scientists have found links between eye cancer and some genetic changes, but it remains unclear exactly where these changes occur, or if they cause cancer to develop. […] The following factors seem to increase the risk of developing eye melanoma: Eye color: People with blue or green eyes have a higher risk of developing ocular melanoma than those with brown eyes. […] Exposure to ultraviolet (UV) light: Exposure to UV light may increase the risk of conjunctival melanoma. This is a melanoma that occurs on the surface of the eye. […] Dysplastic nevus syndrome: In this condition, a person develops atypical moles, known as dysplastic nevi. These are different to ordinary moles. They have irregular borders, may contain several different colors, and often appear in clusters. Dysplastic nevi are more likely to develop into malignant melanomas than ordinary moles. […] Ethnicity: Eye melanoma is most likely to develop in white people. […] Scientists have not yet proven that sunshine and outdoor work cause eye cancer, but they may increase the risk.

• #69 Eye melanoma: Symptoms, causes, and risk factors

  https://www.medicalnewstoday.com/articles/183858

  Eye melanoma is usually a secondary cancer, meaning that it begins in a different location in the body and spreads to the eye. In fact, around 9 out of 10 eye melanomas begin in the skin. […] Experts are not sure exactly what causes ocular melanoma. […] Scientists have found links between eye cancer and some genetic changes, but it remains unclear exactly where these changes occur, or if they cause cancer to develop. […] The following factors seem to increase the risk of developing eye melanoma: Eye color: People with blue or green eyes have a higher risk of developing ocular melanoma than those with brown eyes. […] Exposure to ultraviolet (UV) light: Exposure to UV light may increase the risk of conjunctival melanoma. This is a melanoma that occurs on the surface of the eye. […] Dysplastic nevus syndrome: In this condition, a person develops atypical moles, known as dysplastic nevi. These are different to ordinary moles. They have irregular borders, may contain several different colors, and often appear in clusters. Dysplastic nevi are more likely to develop into malignant melanomas than ordinary moles. […] Ethnicity: Eye melanoma is most likely to develop in white people. […] Scientists have not yet proven that sunshine and outdoor work cause eye cancer, but they may increase the risk.

• #70 Melanoma of the eye Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/melanoma-of-the-eye

  Melanoma is a very aggressive type of cancer that can spread rapidly. […] Too much exposure to sunlight is an important risk factor for melanoma. People who have fair skin and blue eyes are most affected. […] The choroid layer is the most likely site of melanoma in the eye. This is the layer of blood vessels and connective tissue between the white of the eye and retina (back of the eye). […] Melanoma can also begin on the skin or other organs in the body and spread to the eye.

• #71 Cancers Affecting the Retina – Eye Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/eye-disorders/retinal-disorders/cancers-affecting-the-retina

  Choroidal melanoma is a cancer that originates from the pigment-producing cells (melanocytes) of the choroid. Choroidal melanoma is the most common cancer originating in the eye. It is most common among White people. It occurs most frequently at age 55 to 60. […] Choroidal metastases are cancers that have spread to the choroid from other parts of the body. Because of its rich blood supply, the choroid is often a place to which cancers from other parts of the body may spread. In women, breast cancer is the most common cause. In men, cancers of the lung are the most common causes.

• #72 Uveal Melanoma: Treating Rare Eye Cancer | Cancer | Eyes and Vision | UT Southwestern Medical Center

  https://utswmed.org/medblog/uveal-melanoma-treatment/

  Uveal melanoma is the most common type of eye cancer. […] People with fair skin color or light-colored eyes (green or blue) are at increased risk of uveal melanoma. Some cases are caused by an inherited gene mutation. […] In nearly every case, there is no clear cause of uveal melanoma. It is not directly associated with UV light exposure or environmental factors, and there is a slight increased risk in men over women. […] There is an increased risk of uveal melanoma in people with fair skin color or light-colored eyes (green or blue). In particular, white people of northern European descent are at the highest risk, although people of any ancestry can develop uveal melanoma. […] Uveal melanoma is usually not passed from parents to children, but 2%-3% of cases are caused by an inherited gene mutation, most commonly in the BAP1 gene. Patients with an inherited gene mutation may develop uveal melanoma at a younger age.

• #73 Uveal Melanoma: Treating Rare Eye Cancer | Cancer | Eyes and Vision | UT Southwestern Medical Center

  https://utswmed.org/medblog/uveal-melanoma-treatment/

  Uveal melanoma is the most common type of eye cancer. […] People with fair skin color or light-colored eyes (green or blue) are at increased risk of uveal melanoma. Some cases are caused by an inherited gene mutation. […] In nearly every case, there is no clear cause of uveal melanoma. It is not directly associated with UV light exposure or environmental factors, and there is a slight increased risk in men over women. […] There is an increased risk of uveal melanoma in people with fair skin color or light-colored eyes (green or blue). In particular, white people of northern European descent are at the highest risk, although people of any ancestry can develop uveal melanoma. […] Uveal melanoma is usually not passed from parents to children, but 2%-3% of cases are caused by an inherited gene mutation, most commonly in the BAP1 gene. Patients with an inherited gene mutation may develop uveal melanoma at a younger age.

• #74 Uveal melanoma: epidemiology, etiology, and treatment of primary disease

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/

  UM is the most common primary intraocular malignancy in adults, representing 3%5% of all melanomas.1,2 UM most commonly arises from choroidal melanocytes (85%90%), but can also arise from the iris (3%5%) and ciliary body (5%8%).1,3 The median age of diagnosis is approximately 62 years; however, the peak range for diagnosis is between 70 and 79 years.1,4,5 […] A variety of risk factors have been identified, including the presence of light eyes, fair skin, an inability to tan, ocular melanocytosis, dysplastic nevus syndrome, and BAP1 mutations.1013 BAP1 is a tumor-suppressor gene located on chromosome 3 that is mutated in 47% of UM.12 UM in BAP1 germ-line mutants is usually diagnosed between the ages of 30 and 59 years, and is driven by inactivating mutations in the lone functional BAP1 gene, analogous to the frequent loss of chromosome 3 observed in high-risk sporadic disease.14 Interestingly, patients with UM have an 11% higher risk of second malignancies, such as cutaneous melanoma and renal cell carcinoma, than the general population, which may be driven by the presence of germ-line BAP1 mutations.

• #75 Uveal Melanoma: What You Need to Know

  https://www.curemelanoma.org/about-melanoma/types/uveal-melanoma

  Uveal melanoma is a rare form of melanoma derived from the melanocytes in the uvea, accounting for only about 5% of all cases of melanoma in the United States. […] The exact cause of uveal melanoma is not fully understood, but it is believed to be related to mutations that occur in the pigment cells of the eye. These mutations can cause the cells to grow and divide uncontrollably, leading to the development of a uveal tumor. The mutations found in uveal melanoma are distinct from those found in other melanoma subtypes. […] Some inherited genetic mutations have been linked to an increased risk of uveal melanoma which can be diagnosed at a younger age, including mutations in the BAP1 gene. […] Exposure to ultraviolet (UV) radiation: While UV radiation from the sun is a known risk factor for skin cancer, it is not clear if it plays a role in the development of uveal melanoma.

• #76 Uveal melanoma: relatively rare but deadly cancer | Eye

  https://www.nature.com/articles/eye2016275

  An association between uveal melanoma and atypical cutaneous nevi (dysplastic nevi), common cutaneous nevi, and cutaneous freckles has been established. […] The somatic or germline mutation of BAP1 predisposes patients to develop uveal melanoma, malignant mesothelioma, cutaneous melanomas, basal cell carcinoma, and renal cell carcinoma. […] Various studies have explored the particular association between ultraviolet light exposure and occurrence of uveal melanoma. […] However, published literature does not unequivocally implicate sunlight exposure as a risk factor for uveal melanoma. […] Some studies suggest that chronic ultraviolet light exposure is an independent risk factor for uveal melanoma, whereas other studies contradict this.

• #77 What Causes Eye Cancer? | Ocular Melanoma Causes | American Cancer Society

  https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/what-causes.html

  While we know about some of the risk factors for eye cancer (especially melanoma of the eye, or intraocular melanoma), the exact cause of most eye cancers is not known. […] A great deal of research is being done to learn more about genes inside cells and how changes in them can cause the cells to become eye cancer cells. […] Some people inherit gene changes (mutations) that increase their risk for eye cancer. […] One example is a mutation in the BAP1 tumor suppressor gene. […] A mutated BAP1 gene doesn’t work normally, which can allow cells to grow out of control. […] Most gene changes linked to eye cancer are acquired during a person’s life. […] Scientists are studying these and other DNA changes to learn more about them and how they might lead to eye cancer. […] Some changes might be caused by something in a person’s environment, like intense ultraviolet (UV) radiation.

• #78 Ocular (eye) melanoma cancer | Cancer Council

  https://www.cancer.org.au/cancer-information/types-of-cancer/rare-cancers/eye-ocular-melanoma

  The cause of ocular melanoma is not known in most cases. However, there are several risk factors including: […] While the causes of ocular melanoma are not known in most cases, protecting yourself from exposure to UV radiation can reduce your risk.

• #79 Ocular Melanoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK551647/

  An increased risk of uveal melanoma is most significantly associated with ocular or oculodermal melanocytosis. Individuals with light-colored eyes, fair skin, and skin that does not tan but burns easily are also at increased risk. The influence of ultraviolet (UV) light exposure remains unclear. If UV light exposure does cause an increased risk, the conferred risk is lower compared to its contribution to cutaneous melanoma. Additional risk factors include cutaneous nevi, cutaneous freckles, iris nevi, and arc welding.[1] […] Various genetic risk variants also exist. A recent discovery highlights a genetic correlation between uveal and cutaneous melanoma on chromosome 5p15.33.[2] Researchers believe that 2 potential genes in this area, telomerase reverse transcriptase (TERT) and CLPTM1 regulator of GABA type A receptor forward trafficking (CLPTM1L), may be responsible for conferring an increased risk of ocular melanoma. Recurrent mutations in the TERT promoter are associated with cutaneous melanoma. Other malignancies are associated with variants of CLPTM1L.

• #80 Uveal melanoma: relatively rare but deadly cancer | Eye

  https://www.nature.com/articles/eye2016275

  Although it is a relatively rare disease, primarily found in the Caucasian population, uveal melanoma is the most common primary intraocular tumor in adults with a mean age-adjusted incidence of 5.1 cases per million per year. […] The host susceptibility factors for uveal melanoma include fair skin, light eye color, inability to tan, ocular or oculodermal melanocytosis, cutaneous or iris or choroidal nevus, and BRCA1-associated protein 1 mutation. […] The increased occurrence of uveal melanoma in lighter-skinned people and those with light (blue or grey) colored eyes may be related to less melanin being present in the choroid and retinal pigment epithelium, which results in less protection from ultraviolet light, and increased risk of developing uveal melanoma. […] Oculodermal melanocytosis represents a congenital pigmentary abnormality with slate-grey pigmentation of the periocular skin, sclera, uvea, orbit, meninges, palate, and tympanic membrane and is an important risk factor for development of uveal melanoma.

• #81 What Causes Eye Cancer? | Ocular Melanoma Causes | American Cancer Society

  https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/what-causes.html

  While we know about some of the risk factors for eye cancer (especially melanoma of the eye, or intraocular melanoma), the exact cause of most eye cancers is not known. […] A great deal of research is being done to learn more about genes inside cells and how changes in them can cause the cells to become eye cancer cells. […] Some people inherit gene changes (mutations) that increase their risk for eye cancer. […] One example is a mutation in the BAP1 tumor suppressor gene. […] A mutated BAP1 gene doesn’t work normally, which can allow cells to grow out of control. […] Most gene changes linked to eye cancer are acquired during a person’s life. […] Scientists are studying these and other DNA changes to learn more about them and how they might lead to eye cancer. […] Some changes might be caused by something in a person’s environment, like intense ultraviolet (UV) radiation.

• #82 Survival For Eye Cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/eye-cancer/survival

  We don’t know what causes most eye cancers. But there are some factors that can increase your risk of developing it. […] Sometimes doctors can test for changes in the chromosomes of an eye melanoma that give an idea about how the cancer will behave and how likely it is to spread.

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