Czerniak oka
Epidemiologia

Czerniak oka, stanowiący 3-5% wszystkich czerniaków, jest najczęstszym pierwotnym nowotworem złośliwym wewnątrzgałkowym u dorosłych, z dominującą postacią czerniaka błony naczyniowej (85-90%). Mediana wieku diagnozy wynosi około 62 lata, a najwyższa zachorowalność obserwowana jest w grupie 70-79 lat. W USA roczna zachorowalność wynosi 5,1 na milion mieszkańców, wzrastając do 21 na milion u osób powyżej 50 roku życia, z wyższym wskaźnikiem u mężczyzn (5,9 vs 4,5 na milion). Czerniak oka wykazuje wyraźne zróżnicowanie geograficzne i rasowe, z najwyższą zachorowalnością u rasy białej, szczególnie pochodzenia północnoeuropejskiego. Czynniki ryzyka obejmują jasny kolor oczu i skóry, melanocytozę, mutacje genetyczne (m.in. BAP1) oraz pochodzenie z północnych szerokości geograficznych. Rola promieniowania UV pozostaje niejednoznaczna, choć może mieć znaczenie w czerniaku ciała rzęskowego i tęczówki.

Epidemiologia czerniaka oka

Czerniak oka jest najczęstszym pierwotnym nowotworem złośliwym wewnątrzgałkowym u dorosłych, stanowiąc 3-5% wszystkich przypadków czerniaka. Około 85-90% czerniaków oka to czerniaki błony naczyniowej (uveal melanoma), z czego 5% wywodzi się z tęczówki, a pozostałe powstają w ciele rzęskowym lub naczyniówce.12 Pomimo że czerniak oka jest rzadkim nowotworem, pozostaje najczęstszym pierwotnym nowotworem złośliwym gałki ocznej u dorosłych.3

Zachorowalność i statystyki

Mediana wieku w momencie diagnozy wynosi około 62 lat, przy czym największą częstość zachorowań obserwuje się w przedziale wiekowym 70-79 lat.12 W Stanach Zjednoczonych co roku diagnozuje się około 2000-2500 nowych przypadków czerniaka oka.45 Stanowi to około 3,2% wszystkich odnotowanych przypadków czerniaka.6

Średni współczynnik zachorowalności skorygowany względem wieku wynosi 5,1 na milion mieszkańców.23 W przypadku osób powyżej 50 roku życia wskaźnik ten wzrasta do około 21 przypadków na milion rocznie.57 Zachorowalność jest wyższa u mężczyzn (5,9 na milion) w porównaniu do kobiet (4,5 na milion), co oznacza, że mężczyźni mają o 30% wyższy wskaźnik zachorowalności.612

Zróżnicowanie geograficzne i rasowe

Czerniak oka wykazuje znaczące zróżnicowanie geograficzne i rasowe. Pacjenci rasy białej, szczególnie pochodzący z północnej Europy, mają najwyższą zachorowalność na tę chorobę.1 W Stanach Zjednoczonych zachorowalność jest znacząco wyższa wśród osób rasy białej pochodzenia nie-latynoskiego (6,02 na milion) w porównaniu do osób rasy czarnej i Azjatów (odpowiednio 0,31 i 0,39 na milion). Wskaźnik zachorowalności wśród Latynosów plasuje się pośrodku i wynosi 1,67 na milion.2

W Europie obserwuje się gradient zachorowalności wzrastający z południa na północ. Wskaźnik zachorowalności waha się od 2 przypadków na milion w Hiszpanii i Włoszech, przez 4-6 przypadków na milion w Europie Środkowej, do ponad 8 przypadków na milion w Danii i Norwegii.28 W Australii średni współczynnik zachorowalności skorygowany względem wieku wynosi 7,6 na milion.910

Czynniki ryzyka

Zidentyfikowano kilka czynników ryzyka związanych z rozwojem czerniaka oka, w tym:

Rola ekspozycji na promieniowanie ultrafioletowe jako czynnika ryzyka dla czerniaka oka pozostaje niejasna, a badania wykazują niejednoznaczne wyniki.416 Jednakże niektóre dane sugerują, że promieniowanie UV może być związane ze zwiększonym ryzykiem czerniaka ciała rzęskowego/tęczówki.4

Nadzór i obserwacja czerniaka oka

Pomimo doskonałych wskaźników miejscowej kontroli choroby dzięki zabiegom chirurgicznym lub radioterapii, nawet do 50% pacjentów ostatecznie rozwinie chorobę przerzutową.17 Najczęstszymi początkowymi miejscami przerzutów są wątroba (60,5%), płuca (24,4%), skóra/tkanki miękkie (10,9%) oraz kości (8,4%).17

Znaczenie nadzoru

W momencie diagnozy chorobę przerzutową stwierdza się tylko u około 3% pacjentów ze względu na mikroskopijny charakter przerzutów czerniaka oka.18 Jednakże wśród 50% pacjentów, którzy rozwiną chorobę przerzutową, ponad 90% będzie miało przerzuty do wątroby.1819 Z tego powodu większość technik nadzoru koncentruje się na wątrobie.18

Metody nadzoru

Nie istnieje konsensus co do tego, jakie radiologiczne lub serologiczne testy monitorujące są odpowiednie po leczeniu choroby miejscowej.20 Wykorzystywane są różne modalności obrazowania do nadzoru, w tym:

  • Testy funkcji wątroby18
  • Ultrasonografia jamy brzusznej1820
  • Rezonans magnetyczny jamy brzusznej (MRI)1820
  • Tomografia komputerowa (CT)1820
  • Pozytonowa tomografia emisyjna (PET)1820

W porównaniu z samymi testami funkcji wątroby, półroczne badanie MRI ma większą wartość predykcyjną dla wykrywania przerzutów i może być bardziej skuteczną, wolną od promieniowania alternatywą dla innych modalności obrazowania, ponieważ wykrywa również małe zmiany, które mogą być pominięte w badaniu ultrasonograficznym, tomografii komputerowej i obrazowaniu PET.20

Stratyfikacja ryzyka i protokoły nadzoru

Stratyfikacja ryzyka jest ważnym aspektem planowania nadzoru. Pacjenci są często klasyfikowani jako osoby o niskim, średnim lub wysokim ryzyku na podstawie cech klinicznych i molekularnych nowotworu.21

Anegdotycznie, według forów Fundacji Czerniaka Oka (OMF), najczęstszym schematem nadzoru jest wykonywanie badań obrazowych (zwykle CT lub MRI) co 6 miesięcy.18 Bardziej szczegółowe protokoły mogą obejmować:

  • Pacjenci z guzami o niskim ryzyku: badania obrazowe raz w roku21
  • Pacjenci z guzami o wysokim ryzyku: badania obrazowe co 3-6 miesięcy przez pierwsze 5 lat, następnie co 6-12 miesięcy przez lata 6-12, a później według wskazań klinicznych21

W Szkocji opracowano oświadczenie konsensusowe dotyczące nadzoru przerzutowego czerniaka błony naczyniowej, które zaleca stratyfikowane podejście do nadzoru, dzieląc pacjentów na grupy o niskim do średniego ryzyka oraz wysokiego ryzyka i określając dla każdej z nich metodę i czas trwania nadzoru.2223

Aktywny nadzór jako opcja terapeutyczna

W niektórych przypadkach, gdy pacjenci mają czerniaka oka bez objawów, może być zalecany aktywny nadzór (watchful waiting). Oznacza to, że zespół opieki zdrowotnej monitoruje nowotwór, zamiast od razu rozpoczynać leczenie. Wykorzystują testy i badania, aby sprawdzić, czy guz zaczyna rosnąć. Leczenie jest podejmowane, gdy u pacjenta wystąpią objawy lub nowotwór się zmieni.24

Aktywny nadzór może być zalecany, jeśli:

  • Guz jest mały, nie powoduje żadnych objawów i nie rozprzestrzenił się poza oko
  • Guz rośnie bardzo wolno
  • Guz znajduje się w jedynym oku z użytecznym widzeniem
  • Pacjent jest starszy lub bardzo chory, co może utrudniać radzenie sobie z leczeniem24

Trendy i prognozy w epidemiologii czerniaka oka

Ogólny wskaźnik przeżycia od momentu początkowej diagnozy wynosi 69% po 5 latach, 55% po 15 latach i 51% po 25 latach. Jednakże po rozwoju choroby przerzutowej mediana całkowitego przeżycia wynosi około 13,4 miesiąca, przy czym tylko 8% pacjentów przeżywa 2 lata.17

Stabilność wskaźników zachorowalności

W przeciwieństwie do czerniaka skóry, którego częstość występowania gwałtownie wzrosła w ostatnich dziesięcioleciach na całym świecie, zachorowalność na czerniaka oka pozostała stosunkowo stabilna.819 Jednakże niektóre badania wykazują, że w niektórych regionach szczyt zachorowalności przypadł na lata 90. XX wieku, a następnie nastąpił niewielki spadek.9 Analiza JoinPoint zachorowalności na czerniaka błony naczyniowej w Australii wykazała wzrost liczby przypadków w latach 1982-1993 (APC 2,5%; 95% CI 0,0% do 5,0%), a następnie znaczący (p≤0,05) spadek w latach 1993-2014 (APC -1,2%; 95% CI -2,0% do -0,4%).10

Trendy w leczeniu i wynikach

Odsetek przypadków czerniaka błony naczyniowej leczonych wyłącznie chirurgicznie zmniejszył się o 69,4% między okresami 1973-1977 a 2006-2012, przy jednoczesnym 62% wzroście pierwotnej radioterapii.6 Średni 5-letni wskaźnik przeżycia specyficznego dla nowotworu (76%) pozostał stabilny w okresie badania między 1973 a 2012 rokiem.6

Średnie przeżycie pacjentów leczonych pierwotną radioterapią było znacząco lepsze w porównaniu do leczonych wyłącznie chirurgicznie (15,4 ± 0,4 vs 13,6 ± 0,3, P<0,001).6 Analiza wieloczynnikowa wykazała, że płeć męska (iloraz szans [OR] 1,1, CI=1,0–1,3), wiek ≥50 lat (OR 4,0, CI=3,4–4,6), odległe przerzuty (OR 8,6, CI=4,7–15) i pierwotne leczenie chirurgiczne (OR 2,6, CI=2,0–3,3) były niezależnie związane ze zwiększoną śmiertelnością, P<0,005.25

Pomimo postępów w leczeniu pierwotnego guza oka, 50% wskaźnik śmiertelności pozostaje niezmieniony.5 Dane z programu Surveillance, Epidemiology, and End Results (SEER) pokazują, że wskaźniki przeżycia z czerniakiem błony naczyniowej w Stanach Zjednoczonych nie poprawiły się od 1973 do 1997 roku.7

Prognozy na przyszłość

Według Cancer Research UK, przewiduje się, że wskaźniki zachorowalności na nowotwory oka wzrosną o 50% w Wielkiej Brytanii między latami 2023-2025 a 2038-2040, osiągając średnio 3 przypadki na 100 000 osób rocznie do lat 2038-2040.26 Potrzebne są dalsze badania, aby poprawić wyniki leczenia pacjentów, szczególnie w odniesieniu do choroby przerzutowej.5

Zrozumienie epidemiologii czerniaka oka ma kluczowe znaczenie dla planowania strategii wczesnego leczenia i alokacji zasobów medycznych w celu ustanowienia wydajnej, demokratycznej i jednorodnej publicznej opieki zdrowotnej.27

Kolejne rozdziały

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  1. 10.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Ocular Melanoma – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK551647/
    Ocular melanoma is the most prevalent primary intraocular malignancy in adults, accounting for 3% to 5% of all melanomas. Uveal melanoma accounts for 85% to 90%, with 5% originating in the iris and the remainder arising from the ciliary body or choroid. The median age of diagnosis is approximately 62, with a peak incidence observed between 70 and 79. […] White patients of northern European descent have the highest incidence of disease. In contrast, the incidence among Black patients is low, whereas the incidence in patients who are Asian or Hispanic is considered moderate. Men have a 30% higher incidence compared to females. In the United States, the incidence of ocular melanoma is approximately 5 per 1 million population. Internationally, in countries with large populations of individuals of northern European descent, the incidence is 7.5 per 1 million people annually.
  • #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 […] UM incidence varies by sex, race, and country. Males have a 30% greater incidence than females.1 In the US, the incidence is approximately five per million individuals, with a significantly higher incidence in non-Hispanic whites (6.02 per million) when compared with blacks and Asians (0.31 and 0.39 per million, respectively). Incidence in Hispanics is in the middle, at 1.67 per million.1,6,7 In Europe, incidence increases with latitude, ranging from two per million in Spain and Italy, four to six per million in Central Europe, and greater than eight per million in Denmark and Norway.8 The incidence in South Korea is similar to Asians in the US, at 0.42 per million.9
  • #3 Choroidal and Ciliary Body Melanoma – EyeWiki
    https://eyewiki.org/Choroidal_and_Ciliary_Body_Melanoma
    Uveal melanoma is a malignant tumor arising from melanocytes in the uveal tract (iris, ciliary body, or choroid). It is the most common primary intraocular tumor in adults with an age-adjusted incidence of 5.1 per million. […] There is a strong tendency for metastasis, particularly to the liver, and prognosis is poor when the tumor has disseminated. The choroid is the most common site involved and gives rise to 90% of uveal melanomas. […] This article focuses on the treatment of posterior (ciliary body and choroidal) uveal melanoma. The treatment of anterior (iris) melanoma or conjunctival melanoma is discussed elsewhere. […] While development of uveal melanoma is largely considered to be a sporadic event, certain risk factors including light iris color, light skin color, ability to tan, northern European ancestry, and rarely a family history of uveal melanoma may predispose individuals to the disease.
  • #4 Ambient ultraviolet radiation and ocular melanoma incidence in the United States, 2000−2019 | Eye
    https://www.nature.com/articles/s41433-024-02959-9
    UVR may be associated with increased risk of ciliary body/iris melanoma. Reduced risk of choroidal melanoma may be due to higher diffuse UVR exposure to posterior ocular sites in locations at higher latitudes. […] Ocular melanoma is diagnosed in about 2000 new patients in the United States each year. […] Possible risk factors of ocular melanoma (mainly focused on uveal melanoma) include male sex, older age, light eye colour, sun-sensitive skin, higher numbers of cutaneous melanocytic nevi, ocular melanocytosis, ubiquitin carboxyl-terminal hydrolase BAP1 germline mutations, and exposure to ultraviolet radiation (UVR). […] Epidemiological studies on the relationship between solar UVR and risk of ocular melanoma have been inconsistent. […] We examined the associations between ambient UVR and incidence of different anatomical sites of ocular melanoma by sex, age, laterality, and race and ethnicity using data from National Cancer Institutes Surveillance, Epidemiology, and End Results (SEER) programme linked to United States county-level, satellite-based UVR.
  • #4 Ambient ultraviolet radiation and ocular melanoma incidence in the United States, 2000−2019 | Eye
    https://www.nature.com/articles/s41433-024-02959-9
    Ocular melanoma is a rare, but deadly cancer. This large cancer registry study examines the associations between solar ultraviolet radiation (UVR) and incidence of different anatomical sites of ocular melanoma by sex, age, laterality, and race and ethnicity. […] Incidence data were derived from 21 cancer registries in the US for the years 20002019. […] UVR was not associated with total ocular melanoma (N=18,089) comparing Q4 versus Q1 (IRR=0.98; 95%CI:0.94,1.03; p-trend=0.07) or conjunctival melanoma (IRR=0.99; 95%CI:0.82,1.19; p-trend=0.81). However, in analyses of continuous UVR (per 10mW/m2), risks were reduced for total ocular melanoma (IRR=0.97; 95% CI: 0.96, 0.99). Incidence was increased for ciliary body/iris melanoma in the highest UVR quartile (IRR=1.63; 95%CI:1.43,1.87; p-trend0.0001) and remained increased in non-Hispanic White individuals only. Incidence was reduced for choroidal melanoma in the highest UVR quartile (IRR=0.86; 95%CI:0.82,0.91; p-trend0.0001).
  • #5 OMF | Ocular Melanoma Foundation – disease
    https://ocularmelanoma.org/disease
    Ocular melanoma is the most common primary cancer of the eye in adults. It is diagnosed in about 2,500 adults every year in the United States and occurs most often in lightly pigmented individuals with a median age of 55 years. […] Ocular melanoma (OM for short) is a cancer of the eye diagnosed in approximately 2,000-2,500 adults annually in the United States. In both the U.S. and Europe, this equates to about 5 – 7.5 cases per million people per year and, for people over 50 years old, the incidence rate increases to around 21 per million per year. […] Approximately 2,500 adults are diagnosed with ocular melanoma every year. There is no known cause, though incidence is highest among people with lighter skin and blue eyes. […] Approximately 50% of patients with OM will develop metastases by 10 to 15 years after diagnosis (a small percentage of people will develop metastases even later i.e. 20-25 years after their initial diagnosis). Metastatic disease is universally fatal. This 50% mortality rate is unchanged despite treatment advances in treating the primary eye tumor. More research is needed urgently to improve patient outcomes.
  • #6 Trends in incidence, survival, and management of uveal melanoma: a pop | OPTH
    https://www.dovepress.com/trends-in-incidence-survival-and-management-of-uveal-melanoma-a-popula-peer-reviewed-fulltext-article-OPTH
    A total of 7,516 cases of UM represented 3.2% of all recorded cases of melanoma. […] The mean age-adjusted incidence was 5.1 per million (95% CI 4.26.1) and was higher in males (5.9, CI =4.47.6) compared to females (4.5, CI =3.35.8), P0.001. […] The initial diagnoses in 65.2% of cases were by histopathology, followed by clinical diagnosis (18.8%) and radiographic imaging (16.0%). […] The percentage of UM cases managed by surgery alone decreased by 69.4% between the 19731977 and 20062012 time periods, concomitant with a 62% increase in primary radiotherapy, P0.001. […] The mean 5-year cancer-specific survival rate (76%) remained stable during the study period between 1973 and 2012. […] The mean survival for patients treated with primary radiotherapy was significantly improved compared to those treated with surgery alone (15.40.4 vs 13.60.3, P0.001).
  • #7 Malignant Ocular Melanoma – BroadcastMed
    https://www.broadcastmed.com/4561/videos/malignant-ocular-melanoma
    I’m going to speak now about malignant melanoma of the eye. What is ocular melanoma? It’s the most common form of primary adult eye cancer. It involves the pigmented portions of the eye, the iris, the choroid, and the ciliary body. It’s also known as uveal melanoma, as these pigmented portions of the eye are called the uveal tract. The presence of ocular melanoma occurs most commonly in those with fair skin and blue eyes. There are 7.5 cases per million in Europe and the United States. Among people aged 50 plus, cases increased to 21 per million people. […] The SEER data, which is US Surveillance, Epidemiology, and End Results Program, which was evaluated by Singh colleagues, show survival rates with uveal melanoma in the United States have not improved from 1973 through 1997. They looked at 2,054 patients with uveal melanoma. Although the proportion treated with radiation increased from two to 28% in 20 years, the mortality was not affected, remaining essentially unchanged from 77 to 84%, which is not a statistically significant variation. […] Ocular melanoma, in summary, is the most common form of adult eye cancer.
  • #8 Differences and Similarities in Epidemiology and Risk Factors for Cutaneous and Uveal Melanoma
    https://www.mdpi.com/1648-9144/59/5/943
    In both CM and UM, the incidence in European countries follows a sharp north-to-south and west-to-east decreasing curve. This gradient is directly related to the protective factor of the eye pigment present in the southern population, also respecting the high exposure to ultraviolet (UV) light in lower latitudes. […] Melanoma is rare in non-Whites and several studies have demonstrated that Hispanics and Asians have a lower incidence of both CM and UM while Black individuals have the lowest incidence. […] Analyzing skin melanoma incidence data by sex, the studies noticed that women are more prevalent in young age groups, whereas men are more prevalent from the age of 55 onwards. […] UM develops more often in older people, with an incidence rate that increases in direct proportion to age, reaching a peak incidence at the age of 70 years and reaching a plateau phase after the age of 75. […] In children, UM is unusual. […] Furthermore, unlike UM, CM primarily affects young and middle-aged people (median age at diagnosis, 57 years).
  • #8 Differences and Similarities in Epidemiology and Risk Factors for Cutaneous and Uveal Melanoma
    https://www.mdpi.com/1648-9144/59/5/943
    Both cutaneous melanoma (CM) and uveal melanoma (UM) represent important causes of morbidity and mortality. […] Uveal melanoma is a rare condition but is the most prevalent primary intra-ocular malignant tumor in adults. Cutaneous melanoma, on the other hand, is significantly more common. While the frequency of cutaneous melanoma has increased in the last decades worldwide, the incidence of uveal melanoma has remained stable. […] The uvea is the second most common site for melanoma and accounts for 5% of all primary melanoma cases, with UM representing the most frequently diagnosed primary intraocular malignant tumor in adults. […] UM has an annual incidence of six people per million, whereas CM has an annual incidence of 12.2 to 48.1 people per 100,000 persons. […] While the worldwide incidence of CM has been steadily increasing in recent decades across all continents at a more rapid rate compared to any other type of cancer, the incidence of UM has remained stable.
  • #9 Incidence and mortality of uveal melanoma in Australia (1982–2014) | British Journal of Ophthalmology
    https://bjo.bmj.com/content/107/3/406
    Aims We aimed to estimate the incidence and mortality of uveal melanoma (UM) in Australia from 1982 to 2014. […] From 1982 to 2014, there were 5087 cases of ocular melanoma in Australia, of which 4617 were classified as UM. The average age-standardised incidence rate of UM was 7.6 (95% CI 7.3 to 7.9) per million. […] In conclusion, we found that the incidence of UM peaked in the 1990s. Although treatment for primary UM has improved in the last 30 years, overall survival did not change significantly in the last 30 years. […] Uveal melanomas (UMs) are the most common eye-related malignancies in adults. […] Given that the last report of the incidence of UM in the Australian population was released in the early 2000s, the aims of this work are to review the recent trends in the incidence and mortality of UM in Australia from 1982 to 2014 and to provide an updated estimate against which future changes in prevention strategies and treatment practices of UM can be measured.
  • #10 Incidence and mortality of uveal melanoma in Australia (1982–2014) | British Journal of Ophthalmology
    https://bjo.bmj.com/content/107/3/406
    The average ASR of UM was 7.6 (95% CI 7.3 to 7.9) per million. […] While there was no overall change in incidence from 1982 to 2014 (AAPC 0.0%; 95%CI 0.9% to 1.0%), JoinPoint analysis of UM revealed an increase in cases from 1982 to 1993 (APC 2.5%; 95%CI 0.0% to 5.0%), followed by a significant (p0.05) decrease from 1993 to 2014 (APC 1.2%; 95%CI 2.0% to 0.4%). […] The indirect age-standardised incidence ratio of UM when compared by country of birth differed from Australian-born cases. […] The overall 5-year disease-specific survival of UM in Australia remained stable from 1982 to 2011, at an average of 81%.
  • #11 Risk Factors for Eye Cancer | American Cancer Society
    https://www.cancer.org/cancer/types/eye-cancer/causes-risks-prevention/risk-factors.html
    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 syndrome are at increased risk of skin melanoma. They also seem to have a higher risk of developing melanoma of the eye. […] 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.
  • #12 Epidemiology and management of uveal melanoma | Harvard Medical School Department of Ophthalmology
    https://eye.hms.harvard.edu/publications/epidemiology-and-management-uveal-melanoma
    Uveal melanoma is the most common primary intraocular malignancy in adults. The disease overwhelmingly affects white populations. Other risk factors include fair skin, light iris color, ancestry from northern latitudes, and ocular/oculodermal melanocytosis. […] However, there are still no effective therapies against metastatic uveal melanoma, which is almost always fatal. Continued advances in understanding of the molecular mechanisms of uveal melanoma will facilitate the identification of prognostic markers and therapeutic targets.
  • #13 Choroidal and Ciliary Body Melanoma – EyeWiki
    https://eyewiki.org/Choroidal_and_Ciliary_Body_Melanoma
    Those with pre-existing choroidal nevi are also at risk where the incidence of malignant transformation for each lesion is 1/5000 to 1/8845. […] The size of the choroidal melanomas is the most important clinical feature in determining disease prognosis. Size is classified into three categories according to a modification of the criteria of the Collaborative Ocular Melanoma Study (COMS): small, medium and large melanomas. Treatment strategies may vary depending on this classification. […] The goals of treating uveal melanoma is to achieve the following: Preservation of useful vision in the affected eye, Tumor destruction, Prevention of metastasis and recurrence. […] Documented growth of a melanocytic choroidal tumor is suggestive that the lesion is a choroidal melanoma. Growth over a short time (2 years) is strongly suggestive of melanoma whereas growth over a long period (10 years) is still consistent with a benign lesion.
  • #14 Uveal Melanoma: What You Need to Know
    https://www.curemelanoma.org/about-melanoma/types/uveal-melanoma
    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. […] It’s important to note that many people who develop uveal melanoma do not have any known risk factors, and the exact cause of the disease is not fully understood. […] 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. […] The treatment of uveal melanoma depends on several factors, including the size and location of the tumor, as well as the patient’s overall health.
  • #15 Uveal Melanoma Study – NCI
    https://www.cancer.gov/ccg/research/genome-sequencing/tcga/studied-cancers/uveal-melanoma-study
    Uveal melanoma is rare, it is the most common eye cancer in adults. In the United States, approximately 1,700 people are diagnosed each year. When uveal melanoma becomes metastatic, almost all patients die within one year. Individuals who are Caucasian, older, have fair skin that tans easily, or have light eye color tend to be more at risk of uveal melanoma. […] Uveal melanoma is molecularly distinct from cutaneous melanoma, with: lower somatic mutation density, no ultraviolet radiation mutational signature, a discrete set of significantly mutated genes. […] Specific gene expression patterns and molecular pathways associate with differential time to metastasis. […] Distinct global DNA methylation profiles, copy number alterations, and cellular pathway activity profiles can distinguish certain subtypes of the cancer.
  • #16 Ambient ultraviolet radiation and ocular melanoma incidence in the United States, 2000−2019 | Eye
    https://www.nature.com/articles/s41433-024-02959-9
    In this large U.S. cancer registry study with a broad range of ambient UVR exposure, we did not find an increased risk of ambient UVR with total ocular melanoma. However, ambient UVR was associated with increased risk of ciliary body/iris melanoma among non-Hispanic White individuals. […] We found some evidence of an association between ambient UVR and reduced risk of choroidal melanoma among non-Hispanic White and Hispanic White individuals. […] Our study may strengthen an etiological role of UVR in ciliary body/iris melanoma. However, this harmful association was not consistent with Vajdic et al. that found no association between several surrogates (e.g., latitude, occupational and recreational sun exposures, lifetime modelled ambient UVR, and eye protection) of UVR and risks of iris melanoma. […] Our findings of an inverse association for UVR and melanoma of the choroid were largely consistent with other cancer registry studies examining associations between latitude and risks of uveal melanoma, of which choroidal melanoma was predominant.
  • #17 Uveal melanoma: epidemiology, etiology, and treatment of primary disease
    https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/
    Despite excellent rates of local disease control with surgery or radiotherapy, up to 50% of patients will ultimately develop metastatic disease, with the most common initial sites being the liver (60.5%), the lung (24.4%), skin/soft tissue (10.9%), and bone (8.4%).25 The overall rate of survival from initial diagnosis is 69% at 5 years, 55% at 15 years, and 51% at 25 years; however, following the development of metastatic disease, median overall survival is approximately 13.4 months, with only 8% surviving 2 years.2628 […] Various predictors of metastatic disease and survival have been studied, including clinical staging systems, gene-cytogenetic findings, and gene-expression panels. The American Joint Committee on Cancer (AJCC) UM T1T4 staging system is based on tumor characteristics of base diameter, thickness, ciliary body involvement, and distance of extraocular extension. Survival decreases rapidly with increasing stage. Estimates of death at 5, 10, and 20 years are 4%, 8%, and 11% for T1, 8%, 13%, and 24% for T2, 19%, 27%, and 36% for T3, and 30%, 43%, and 51% for T4 lesions, respectively.29 When compared with AJCC stage I disease, the rate of metastasis/death is three times greater for stage II and nine to ten times greater for stage III disease.30
  • #18 OMF | Ocular Melanoma Foundation – Surveillance
    https://ocularmelanoma.org/surveillance
    At the time of diagnosis, metastatic disease will only be seen in about 3% of patients due to the micrometastatic nature of OM. […] Because, of the 50% of patients who develop metastatic disease, more than 90% of patients will develop liver metastases, the majority of surveillance techniques are focused on the liver. […] These liver-focused surveillance techniques include: Liver function tests, Abdominal ultrasound, Abdominal magnetic resonance imaging (MRI), CT scan, PET scan. […] Anecdotally with the OMF forums, the most common surveillance regimen is getting scans (typically CT or MRI) every 6 months.
  • #19 Uveal melanoma – Wikipedia
    https://en.wikipedia.org/wiki/Uveal_melanoma
    Uveal melanomas are the most common primary intraocular tumor in adults. Uveal melanoma is classified as a rare cancer with 5.1 cases per million people per year. The incidence has remained stable for several years. […] There are about 2500 patients with UM diagnosed annually in the US. […] Currently, there is no consensus regarding type or frequency of scans following diagnosis and treatment of the primary eye tumor. Of the 50% of patients who develop metastatic disease, more than 90% of patients will develop liver metastases. As such, the majority of surveillance techniques are focused on the liver. These include abdominal magnetic resonance imaging (MRI), abdominal ultrasound and liver function tests. The scientific community is currently working to develop guidelines, but until then, each patient must take into consideration their individual clinical situation and discuss appropriate surveillance with their doctors.
  • #20 Uveal melanoma: epidemiology, etiology, and treatment of primary disease
    https://pmc.ncbi.nlm.nih.gov/articles/PMC5298817/
    There is no consensus as to what radiological or serum surveillance tests, if any, are appropriate after management of local disease.108,109 Various surveillance imaging modalities have been used, including chest X-ray, abdominal ultrasound, magnetic resonance imaging, computed tomography, and fludeoxyglucose positron-emission tomography imaging.110,111 Compared to liver function tests alone, biannual magnetic resonance imaging has greater predictive value for metastasis detection and may be a more effective, radiation-free alternative to other imaging modalities, as it also detects small lesions that may be missed on ultrasonography, computed tomography, and positron-emission tomography imaging.112115 In the absence of data, consideration of routine imaging, including imaging of the liver, and examination at intervals between 3 and 12 months based upon predicted risk of recurrence is reasonable in an effort to identify disease when resection of metastases or institution of therapy for metastatic disease may be possible.
  • #21 Ocular Melanoma | Treatment & Management | Point of Care
    https://www.statpearls.com/point-of-care/26124
    Molecular prognostic testing stratifies patients based on risk and guides surveillance for metastatic disease. Clinicians categorize patients into low, medium, or high risk. […] Patients with low-risk tumors undergo systemic imaging annually. […] Patients categorized as medium risk have an intermediate likelihood of developing metastatic disease. They may have the following characteristics. […] Patients in the high-risk category have the greatest likelihood of metastasis. […] Patients with high-risk tumors undergo systemic imaging every 3 to 6 months for the first 5 years, followed by every 6 to 12 months for years 6 to 12, and then as clinically indicated. MRI is the preferred diagnostic study for systemic imaging.
  • #22 Consensus statement for metastatic surveillance of uveal melanoma in Scotland | Eye
    https://www.nature.com/articles/s41433-022-02198-w
    Ophthalmic treatments are successful in managing uveal melanomas achieving good local control. However, a large number still metastasise, primarily to the liver, resulting in mortality. There is no consensus across the world on the mode, frequency, duration or utility of regular liver surveillance for metastasis and there are no published protocols. […] The consensus statement recommends a risk-stratified approach to surveillance for these patients dividing them into low to medium-risk and high-risk groups defining the mode and duration of surveillance for each. […] The GDG concluded that some of the evidence in the literature appeared to suggest that offering surveillance to all patients may be futile. However, there was a consensus supporting the concept of conducting surveillance with an emphasis on liver screening.
  • #23 Consensus statement for metastatic surveillance of uveal melanoma in Scotland | Eye
    https://www.nature.com/articles/s41433-022-02198-w
    The consensus statement supplements the UK-wide Uveal Melanoma National Guidelines and allows a more uniform consensus-based approach to surveillance in Scotland. It has been adopted nationally by all health care providers in Scotland as a guideline and is available to patients on a publicly accessible website. […] The SCSG believes that an effective strategy is to target the high-risk uveal melanoma patients in Scotland with the more sensitive imaging modalities for surveillance of liver metastases. These recommendations bring about greater clarity and transparency to this difficult issue and serve as a template for a discussion and combined decision-making with the patient.
  • #24 Active surveillance (watchful waiting) for eye cancer | Canadian Cancer Society
    https://cancer.ca/en/cancer-information/cancer-types/eye/treatment/active-surveillance
    If you have eye cancer but dont have any symptoms, you may be offered active surveillance (watchful waiting). This means that your healthcare team watches your cancer closely rather than giving treatment right away. They will use tests and exams to check if the tumour starts to grow. Treatment is given when you develop symptoms or the cancer changes. […] There is no evidence so far that people wont live as long when they get active surveillance compared to other treatments. And there is no evidence that active surveillance has other negative effects if or when you start treatment. […] You may be offered active surveillance if: the tumour is small, is not causing any symptoms and hasnt spread outside of the eye; the tumour is growing very slowly; the tumour is in the only eye with useful vision; you are older or very ill, which may make it hard to cope with treatment.
  • #25 Trends in incidence, survival, and management of uveal melanoma: a pop | OPTH
    https://www.dovepress.com/trends-in-incidence-survival-and-management-of-uveal-melanoma-a-popula-peer-reviewed-fulltext-article-OPTH
    Multivariate analysis identified male sex (odds ratio [OR] 1.1, CI =1.01.3), age 50 years (OR 4.0, CI =3.44.6), distant metastases (OR 8.6, CI =4.715), and primary surgical treatment (OR 2.6, CI =2.03.3) as independently associated with increased mortality, P0.005. […] Conversely, patients identified as Hispanic (OR 0.6, CI =0.50.8) and patients receiving radiation treatment (OR 0.5, CI =0.40.7) were independently associated with reduced mortality, P0.005. […] UM remains a rare form of melanoma that occurs primarily in Caucasian patients older than 50 years. […] More than two-thirds of UM patients are curatively treated with primary radiotherapy as opposed to surgery, which has resulted in a significant improvement in both overall survival and cancer-specific survival. […] Despite this shift in management strategy, the mean 5-year cancer-specific survival rate remained relatively unchanged during the study period.
  • #26 Eye cancer incidence statistics | Cancer Research UK
    https://www.cancerresearchuk.org/health-professional/cancer-statistics/statistics-by-cancer-type/eye-cancer/incidence
    Eye cancer is not among the 20 most common cancers in the UK, accounting for less than 1% of all new cancer cases (2017-2019). […] For eye cancer, there are few established risk factors therefore differences between countries largely reflect differences in diagnosis and data recording. […] Eye cancer European age-standardised (AS) incidence rates for females and males combined increased by 30% in the UK between 1993-1995 and 2017-2019. […] For eye cancer there are few established risk factors, therefore increasing incidence in the 1980s and 1990s may largely reflect improvements in diagnosis and data recording. […] Eye cancer incidence rates are projected to rise by 50% in the UK between 2023-2025 and 2038-2040, to 3 cases per 100,000 people on average each year by 2038-2040.
  • #27 Epidemiology of uveal melanoma in Brazil | International Journal of Retina and Vitreous | Full Text
    https://journalretinavitreous.biomedcentral.com/articles/10.1186/s40942-020-00261-w
    Uveal melanoma was mainly diagnosed in Southeast Brazil due to the highest concentration of citizens allied to the presence of the most important tertiary cancer centers with trained staff and equipment to provide diagnosis and management of the disease. […] The Law of 60 days was enforced in 2012 by the Brazilian Federal Government as a result of the long delays between the diagnosis and the initiation of cancer treatment nationwide. […] Unfortunately, after the implementation of the 60 days law, there was no difference in terms of diagnosis, staging and treatment, with the persistence of general difficulties in healthcare access such as long queues for patients who need assistance and delays in scheduling medical appointments. […] Epidemiological data is critical for planning early treatment strategies and allocating medical resources in order to establish an efficient, democratic and homogeneous public healthcare.