Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis

doi:10.1016/S0161-6420(98)92205-9

Ophthalmology

Volume 105, Issue 1, January 1998, Pages 195-198

https://doi.org/10.1016/S0161-6420(98)92205-9 Get rights and content

Objective:

In the white population, an association between oculo(dermal) melanocytosis (ODM) and uveal melanoma is well recognized. However, the lifetime prevalence of uveal melanoma in the ODM population is not known. This study was designed to determine the lifetime prevalence of uveal melanoma among patients with ocular melanocytosis.

Design:

Fifty-six white patients manifesting ODM with uveal melanoma formed the basis of the study.

Main Outcome Measures:

Published prevalence rates of ODM and uveal melanoma were used for calculations using Bayes' theorem.

Results:

The lifetime prevalence of uveal melanoma in white patients with ODM is estimated to be 2.6 × 10⁻³. The median age at diagnosis of uveal melanoma in the ODM population was similar to a randomly selected population (60.5 years and 62.5 years, respectively). In the vast majority of patients (90%) with ODM-associated uveal melanoma, the uveal melanoma was diagnosed between the ages of 31 years and 80 years.

Conclusions:

One of about 400 patients with ODM followed for life is estimated to develop uveal melanoma. Excessive melanocytes in the uveal tract in ODM may provide the biologic basis for susceptibility to the development of uveal melanoma. Patients with ODM should be monitored ophthalmoscopically, especially during the susceptible period, for the development of uveal melanoma. The authors suggest that a national registry of ODM patients be created and prospective data collected to better assess the risk of developing uveal melanoma.

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Citation Excerpt :
The nevus of Ota (oculodermal melanocytosis) is a congenital pigmented condition of the periocular area, which includes the skin, sclera, uvea and orbit. It was estimated that the lifetime risk for UM development in this condition was 1:400 (Singh et al., 1998), which is clearly above chance alone, and once UM develops, these patients have an increased risk for metastases (Shields et al., 2013). Several cases of cutaneous melanoma have been reported in relation to a nevus of Ota (Patel et al., 1998), including detrimental melanoma with orbital invasion (Radhadevi et al., 2013), but it has not been concluded that there is a true increased risk.
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A recent breakthrough in the treatment of patients with metastatic cutaneous melanoma was the development of immunotherapy. While immunotherapy is currently sparsely effective in intraocular tumours such as UM, the similarities between CoM and cutaneous melanoma (including in their immunological tumour micro environment) provide hope for the application of immunotherapy in CoM, and preliminary clinical data are indeed emerging to support this use.
This review aims to provide a comprehensive overview of the current knowledge regarding CoM, with a focus on the genetic and immunologic understanding. We elaborate on the distinct position of CoM in contrast to other types of melanoma, and explain how new insights in the pathophysiology of this disease guide the development of new, personalized, treatments.
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: Supported by Eye Tumor Research Foundation, Philadelphia, Pennsylvania.

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Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis

Objective:

Design:

Main Outcome Measures:

Results:

Conclusions:

Ophthalmology

Am J Ophthalmol

Am J Ophthalmol

Ophthalmology

Ophthalmology

Ophthalmology

Surv Ophthalmol

Ocular and dermal melanocytosis

Arch Ophthalmol

Nevus fusco-ceruleus opthalmomaxillaris

Tokyo Med J

Malignant melanoma of the choroid in association with oculodermal melanocytosis: a case report

Indian J Ophthalmol

Histogenesis of malignant melanomas of the uvea. III. The relationship of congenital ocular melanocytosis and neurofibromatosis to uveal melanomas

Arch Ophthalmol

Ocular and oculodermal melanocytosis associated with uveal melanoma

Ophthalmology