Dysplastic Nevi are a Risk Factor for Uveal Melanoma

Hammer, Helga; Oláh, Judit; Tóth-Molnár, Edit

doi:10.1177/112067219600600423

Cited by 45 publications

(28 citation statements)

References 11 publications

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“…Patients with atypical cutaneous nevi are 4.36 to 10.4 times more likely to develop ocular melanoma than the average population. 26,27 Iris nevus is a risk factor for uveal melanoma in general and it is a particular risk for iris melanoma, although the rate of transformation of iris nevus into melanoma is not clearly understood. 28 In a study of 175 patients with suspicious iris nevus by Territo et al, 29 8 (5%) lesions showed clinical evidence of growth into iris melanoma at a mean follow-up of 5 years.…”

Section: Host Susceptibility Factorsmentioning

confidence: 99%

Uveal melanoma: relatively rare but deadly cancer

Kaliki

Shields

2016

Eye

469

533

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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. Tumors are located either in iris (4%), ciliary body (6%), or choroid (90%). 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. Currently, the most widely used first-line treatment options for this malignancy are resection, radiation therapy, and enucleation. There are two main types of radiation therapy: plaque brachytherapy (iodine-125, ruthenium-106, or palladium-103, or cobalt-60) and teletherapy (proton beam, helium ion, or stereotactic radiosurgery using cyber knife, gamma knife, or linear accelerator). The alternative to radiation is enucleation. Although these therapies achieve satisfactory local disease control, long-term survival rate for patients with uveal melanoma remains guarded, with risk for liver metastasis. There have been advances in early diagnosis over the past few years, and with the hope survival rates could improve as smaller tumors are treated. As in many other cancer indications, both early detection and early treatment could be critical for a positive long-term survival outcome in uveal melanoma. These observations call attention to an unmet medical need for the early treatment of small melanocytic lesions or small melanomas in the eye to achieve local disease control and vision preservation with the possibility to prevent metastases and improve overall patient survival.

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Section: Host Susceptibility Factorsmentioning

confidence: 99%

Uveal melanoma: relatively rare but deadly cancer

Kaliki

Shields

2016

Eye

469

533

View full text Add to dashboard Cite

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“…Certain skin conditions, such as dysplastic nevus syndrome and nevus of Ota, are also associated with uveal melanoma. 2 It has been theorized by some investigators that exposure to ultraviolet radiation increases the risk of this neoplasia, but this has not been definitively proven. Whereas certain somatic mutations are associated with neoplastic growth and distant metastasis, the malignancy is not inherited in a traditional genetic fashion, although it is believed that individuals who have germline breast cancer 1 (BRCA1)-associated protein 1 (BAP1) mutations are at higher risk for uveal and cutaneous melanoma as well as mesothelioma and renal cancers.…”

Section: Background and Epidemiologymentioning

confidence: 99%

Uveal melanoma: From diagnosis to treatment and the science in between

Chattopadhyay

Kim

Gombos

et al. 2016

Cancer

310

258

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Melanomas of the choroid, ciliary body, and iris of the eye are collectively known as uveal melanomas. These cancers represent 5% of all melanoma diagnoses in the United States, and their age-adjusted risk is 5 per 1 million population. These less frequent melanomas are dissimilar to their more common cutaneous melanoma relative, with differing risk factors, primary treatment, anatomic spread, molecular changes, and responses to systemic therapy. Once uveal melanoma becomes metastatic, therapy options are limited and are often extrapolated from cutaneous melanoma therapies despite the routine exclusion of patients with uveal melanoma from clinical trials. Clinical trials directed at uveal melanoma have been completed or are in progress, and data from these well designed investigations will help guide future directions in this orphan disease. Cancer 2016;122:2299-312. V C 2016 American Cancer Society.KEYWORDS: breast cancer 1-associated protein 1 (BAP1), choroidal melanoma, diagnosis, guanine nucleotide binding protein a11 (GNA11), guanine nucleotide-binding protein Q polypeptide (GNAQ), ocular melanoma, review, science, treatment, uveal melanoma. BACKGROUND AND EPIDEMIOLOGYUveal melanoma is the most common primary intraocular malignancy. The uveal tract is the pigmented layer of the globe encompassing the iris, ciliary body, and choroid (Fig. 1). The terms choroidal melanoma and ocular melanoma are alternative terms for this cancer, because most of the uveal tract is choroidal. However, the term ocular melanoma should be avoided, because it implies the inclusion of conjunctival and adnexal melanomas, which behave and are managed like cutaneous rather than uveal primaries. Approximately 1500 new cases of uveal melanoma are diagnosed in the United States each year, most commonly arising in the choroid followed by the ciliary body. Iris melanomas are the least common location for uveal melanoma (Fig. 2).1 Although the disease has no sex preference, it is more common in middle-aged Caucasians (median age at presentation, 58 years). Risk factors include the presence of a choroidal nevus, which can be observed in 7% to 8% of the Caucasian population. Certain skin conditions, such as dysplastic nevus syndrome and nevus of Ota, are also associated with uveal melanoma.2 It has been theorized by some investigators that exposure to ultraviolet radiation increases the risk of this neoplasia, but this has not been definitively proven. Whereas certain somatic mutations are associated with neoplastic growth and distant metastasis, the malignancy is not inherited in a traditional genetic fashion, although it is believed that individuals who have germline breast cancer 1 (BRCA1)-associated protein 1 (BAP1) mutations are at higher risk for uveal and cutaneous melanoma as well as mesothelioma and renal cancers.

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“…The occurrence of ocular melanoma and cutaneous melanoma in the same patient, the incidence of cutaneous melanoma and ocular melanoma in the same family, the appearance of ocular melanoma and cutaneous melanoma in patients with atypical mole syndrome and dysplastic nevi as risk factors for uveal melanoma are documented [29, 30, 31, 32, 33]. …”

Section: Discussionmentioning

confidence: 99%

Ocular Melanoma: Epidemiology, Clinical Presentation and Relationship with Dysplastic Nevi

Richtig

Langmann²,

Müllner³

et al. 2004

Ophthalmologica

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Purpose: Ocular melanoma is a rare entity compared to cutaneous malignant melanoma. We examined the frequency of the tumor in a defined geographic region, its clinical presentation and its relationship with dysplastic nevi in 136 patients. Methods: 136 patients (64 men and 72 women; mean age 61.7 years, range 20–92 years) with ocular melanoma were treated at the University Hospital of Graz between June 1996 and December 2001. 129 had primary uveal melanoma in one eye (117 choroidal melanomas, 11 melanomas of the ciliary body and 1 of the iris), 2 patients had uveal melanoma in both eyes, 4 patients had conjunctival melanoma and 1 patient had a melanoma of the lacrimal sac. Epidemiology, history, potential risk factors, clinical presentation and relationship with dysplastic (= atypical) nevi were documented. Results: 48 patients (35.3%) showed more than five dysplastic nevi, compared to only 1.2% in the general population (χ² test: p < 0.001). 5 (3.7%) had additional cutaneous melanoma and 7 (5.1%) had a family history of melanoma. The lifelong risk for the occurrence of an additional primary cutaneous melanoma was 2.9%, which is significantly higher than the usual estimate of 1% for the general population. Conclusions: Patients with primary ocular melanoma have an increased risk to develop cutaneous melanoma and should therefore be examined regularly by dermatologists.

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Dysplastic Nevi are a Risk Factor for Uveal Melanoma

Cited by 45 publications

References 11 publications

Uveal melanoma: relatively rare but deadly cancer

Uveal melanoma: relatively rare but deadly cancer

Uveal melanoma: From diagnosis to treatment and the science in between

Ocular Melanoma: Epidemiology, Clinical Presentation and Relationship with Dysplastic Nevi

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