Prevalence of ocular and oculodermal melanocytosis in Spanish population with uveal melanoma

Abstract

Purpose The aim of this study was to determine the prevalence of ocular and oculodermal melanocytosis (ODM) among patients with uveal melanoma (UM) in a Spanish population. Methods Retrospective review of the medical records of patients with ODM among patients with UM. Results Ten (11 eyes) of 400 patients (2.7%) with UM associated had ODM. The mean age at diagnosis of UM among patients with ODM was 62 years. One patient had bilateral tumours. UM was diagnosed during a routineexamination in two cases. All tumours were medium (7/11) or large (4/11) in size, with a mean maximum base of 13 mm and height of 7 mm. No patient had extraocular extension or metastatic disease at diagnosis. Enucleation was done in five cases and I-125-brachytherapy in six. The mean follow-up was 43 months. One patient died because of metastasis 2 years after enucleation; one patient is currently on treatment of systemic metastasis 11 years after. Conclusions ODM is more frequent in spanish population with UM than in American population. Despite the risk of UM in ODM, it is often diagnosed late when a conservative treatment is not indicated.

Primary bilateral uveal melanoma: a population‐based study and systematic review

“…Table provides an overview of the 46 cases of primary bilateral UM reported from eight countries described in the English language literature between 1950 and 2016 . Case reports comprised the largest percentage of studies (72.0%), and the largest case series described eight patients .…”

Genetic and epigenetic insights into uveal melanoma

“…Earlier studies have pointed towards the risk of ultraviolet light exposure in UM but current views in ocular biology consider them inconclusive. There are certain other phenotypes such as fair skin, light iris color, ancestry from northern latitudes and ocular/oculodermal melanocytosis which are associated with UM incidence . Risk factors for metastases comprise invasion of the ciliary body, extraocular extension and tumor size (largest basal diameter and thickness).…”

“…There are certain other phenotypes such as fair skin, light iris color, ancestry from northern latitudes and ocular/oculodermal melanocytosis which are associated with UM incidence. [14][15][16][17] Risk factors for metastases comprise invasion of the ciliary body, extraocular extension and tumor size (largest basal diameter and thickness). Histologically, prognostic factors such as infiltration of vortex veins, cell type (spindle vs epithelioid cells), mean vascular density and the presence of vasculogenic mimicry (= vessel-like extracellular matrix pattern, first described in UM by Maniotis et al in 1999 18 ) can be evaluated.…”

Pigmentary disorders of the eyes and skin

“…Oculodermal melanocytosis is usually a benign condition but patients have a 10% chance of developing glaucoma 98 and possibly an increased risk of associated melanoma, 99 which has been documented to occur in the skin 100,101 ; the uveal tract (which includes the iris, ciliary body, and choroid) [102][103][104][105][106][107] ; the central nervous system 108,109 ; and the orbit. 110 The clinical presentation of cutaneous melanoma developing within Nevus of Ota can be subtle.…”