Prognosis of uveal melanoma based on race in 8100 patients: The 2015 Doyne Lecture

Shields, Carol L.; Kaliki, Swathi; Cohen, Murray; Shields, Patrick W.; Furuta, Minoru; Shields, Jerry A.

doi:10.1038/eye.2015.51

Cited by 111 publications

(132 citation statements)

References 14 publications

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“…Of the cases registered, 97.8% occurred in the white population [5]. There is a strong difference in the incidence for different ethnic groups: the annual age-adjusted incidence is 0.31 for Afro-Americans, 0.38 for Asians, 1.67 for Hispanics, and 6.02 for non-Hispanic whites [6], yet prognosis does not differ for ethnic groups [7]. The European Cancer Registry-based study on survival and care of cancer patients (EUROCARE) for the years 1983–1994 reported similar incidence rates with a characteristic increase from south to north, from <2 per million in Spain and Southern Italy to >8 per million in Norway and Denmark [8].…”

Section: Clinical Features Of Uveal Melanomamentioning

confidence: 99%

The biology of uveal melanoma

Amaro

Gangemi

Piaggio

et al. 2017

Cancer Metastasis Rev

184

193

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Uveal melanoma (UM), a rare cancer of the eye, is distinct from cutaneous melanoma by its etiology, the mutation frequency and profile, and its clinical behavior including resistance to targeted therapy and immune checkpoint blockers. Primary disease is efficiently controlled by surgery or radiation therapy, but about half of UMs develop distant metastasis mostly to the liver. Survival of patients with metastasis is below 1 year and has not improved in decades. Recent years have brought a deep understanding of UM biology characterized by initiating mutations in the G proteins GNAQ and GNA11. Cytogenetic alterations, in particular monosomy of chromosome 3 and amplification of the long arm of chromosome 8, and mutation of the BRCA1-associated protein 1, BAP1, a tumor suppressor gene, or the splicing factor SF3B1 determine UM metastasis. Cytogenetic and molecular profiling allow for a very precise prognostication that is still not matched by efficacious adjuvant therapies. G protein signaling has been shown to activate the YAP/TAZ pathway independent of HIPPO, and conventional signaling via the mitogen-activated kinase pathway probably also contributes to UM development and progression. Several lines of evidence indicate that inflammation and macrophages play a pro-tumor role in UM and in its hepatic metastases. UM cells benefit from the immune privilege in the eye and may adopt several mechanisms involved in this privilege for tumor escape that act even after leaving the niche. Here, we review the current knowledge of the biology of UM and discuss recent approaches to UM treatment.

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Section: Clinical Features Of Uveal Melanomamentioning

confidence: 99%

The biology of uveal melanoma

Amaro

Gangemi

Piaggio

et al. 2017

Cancer Metastasis Rev

184

193

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“…No conclusive results have been published on the association between iris colour, skin colour and the development of CoM. Pigmentation of the tumours themselves was investigated as well: amelanotic cutaneous melanoma has a significantly worse survival compared with melanotic cutaneous melanoma,13 and one analysis found that low tumour pigmentation was similarly associated with a worse clinical outcome in UM 14. Low tumour pigmentation has been associated with a worse prognosis in CoM,15 but this was investigated in a limited number of patients as the disease is so rare.…”

Section: Introductionmentioning

confidence: 99%

Lack of tumour pigmentation in conjunctival melanoma is associated with light iris colour and worse prognosis

et al. 2018

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Lightly pigmented tumours occurred especially in individuals with lightly coloured irises. While iris colour or skin colour was not significantly related to clinical outcome, a low tumour pigmentation was related to a worse outcome in patients with conjunctival melanoma. The amount and type of melanin in conjunctival melanocytes may be involved in the pathogenesis and behaviour of selected conjunctival melanoma.

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“…In the Collaborative Ocular Melanoma Study (COMS), as well as a more recently published series of 8,100 patients with UM, 97%-98% of patients were Caucasian, with Asian patients representing < 1% of patients in both cohorts. (8,9) Aside from significant epidemiological heterogeneity, there is also marked biological diversity in UM. In recent years, the genetic abnormalities in UMs have been found to be distinct from those in cutaneous melanomas.…”

mentioning

confidence: 99%

Clinical spectrum, treatment and outcomes of uveal melanoma in a tertiary centre

Wong

Sundar

Chee

et al. 2019

smedj

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Uveal melanoma (UM) is an aggressive intraocular malignancy, and despite advances in the local methods of treatment of primary UM, the survival rates have not improved. (1) This is thought to be due to the tumour's proclivity for early occult micro-metastasis, which is undetectable at the time of diagnosis and treatment of the primary tumour. (2,3) At present, there are no effective chemotherapeutic agents for metastatic UM, (3) with death rates from melanoma-related metastasis at 80% at one year and 92% at two years. (4) The most common primary intraocular tumour in Caucasian adults is UM. The reported age-standardised incidence rate in the United States and Europe is 5.1 per million (5) and 2.6-7.5 per million, respectively. In contrast, the age-standardised incidence rate in South Korea and Japan is 0.6 per million (6) and 0.2 per million, (7) respectively. The literature on the clinicopathologic spectrum of UM is largely derived from a Western setting. In the Collaborative Ocular Melanoma Study (COMS), as well as a more recently published series of 8,100 patients with UM, 97%-98% of patients were Caucasian, with Asian patients representing < 1% of patients in both cohorts. (8,9) Aside from significant epidemiological heterogeneity, there is also marked biological diversity in UM. In recent years, the genetic abnormalities in UMs have been found to be distinct from those in cutaneous melanomas. In contrast to cutaneous melanomas, UMs lack mutations in B rapidly accelerated fibrosarcoma (BRAF), neuroblastoma rapidly accelerated sarcoma (NRAS) and Kit genes. (2,10) Instead, mutations in guanine nucleotide-binding protein subunit alpha-Q (GNAQ) and guanine nucleotide-binding protein subunit alpha-11 (GNA11) are early events in UM tumorigenesis. BRCA1 associated protein-1 (BAP1) is implicated in tumour progression and metastasis. (11) The cytogenetic profiles in UMs based on normal disomy 3, 6, 8, or partial/complete monosomy 3, 6p gain/loss, 6q gain/loss, 8p gain/loss, and 8p gain/loss have also been found to be predictive of the risk of melanoma-related metastasis. (12) Our study reports the clinical characteristics, treatment patterns and outcomes of patients with UM treated in a tertiary centre in Southeast Asia. METHODSPatients with a diagnosis of UM made by clinical and/or pathologic analysis and who were followed up in our centre between 2002 and 2017 were retrospectively reviewed. Information on the patients' baseline demographics, clinical presentation, treatment and survival outcomes was collected. Approval from our Institutional Review Board was obtained.Choroidal melanoma (CM) was classified as small, medium or large based on the criteria established by the COMS. A melanoma INTRODUCTION We aimed to describe the clinical characteristics, diagnostic challenges, treatment patterns and outcomes of uveal melanoma (UM) in a tertiary care centre.METHODS This is a retrospective case series of 11 consecutive patients with UM who were managed in a tertiary referral centre between 2002 and 2017. Epidemiological, ...

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Prognosis of uveal melanoma based on race in 8100 patients: The 2015 Doyne Lecture

Cited by 111 publications

References 14 publications

The biology of uveal melanoma

The biology of uveal melanoma

Lack of tumour pigmentation in conjunctival melanoma is associated with light iris colour and worse prognosis

Clinical spectrum, treatment and outcomes of uveal melanoma in a tertiary centre

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