Iris Melanoma: Management and Prognosis

Antonio Longo,Antonio Basile,Rosario Caltabiano,Matteo Fallico,Corrado Spatola,Silvia Varricchio,Rocco Luca Emanuele Liardo,Floriana Arena,Andrea Russo,Giuseppe Broggi,Teresio Avitabile,Daniela Russo,Vincenza Bonfiglio,Roberto Milazzotto,Michele Reibaldi,Francesco Pignatelli,Pietro Valerio Foti

doi:10.3390/app10248766

Abstract

Iris melanomas represent 2–5% of uveal melanomas. Iris melanomas vary in their size, shape, degree of pigmentation and clinical behavior. The main local clinical complications of iris melanomas are tumor vascularization, ectropion uvea, pupillary distortion, pigment dispersion, sector cataract, chronic uveitis, hyphema and glaucoma with irreversible optic nerve damage. The most effective treatment for iris nevus and melanoma remains debatable; treatment modalities have been proposed depending on the local status as well as the age and general condition of the patient. A melanocytic iris nevus is usually observed until documented progression is identified. In this case, radiotherapy or surgical resection is generally performed. Cataract, glaucoma and limbal stem cell deficiency are usually secondary to radiotherapy, while incomplete tumor excisions, which could lead to recurrence, hemorrhage, vitreous loss, dislocated lens, iridocyclitis, macular edema, retinal detachment, glaucoma and cataract, are related to surgical resection. In some cases, a combination of radiotherapy and surgery is used. Conservative treatment is an efficient alternative to enucleation and allows good local tumor control.

Highlights

Introduction and General OverviewUveal melanoma is the most common primary intraocular tumor [1] and arises from the malignant proliferation of uveal melanocytes [2]
Jakobiec and Silbert believe that ectropion uvea, vascularity, pupil distortion, involvement of the angle and glaucoma could be evident in other types of lesions [23]
In order to monitor growth and to describe the dimensions of the basal and antero-posterior diameter, as well as the margins, of iris melanoma, there is agreement in the literature regarding the use of sequential photographs with a slit lamp and ultrasound biomicroscope, obviously before dilation

Summary

Introduction and General Overview

Uveal melanoma is the most common primary intraocular tumor [1] and arises from the malignant proliferation of uveal melanocytes [2]. Jakobiec and Silbert believe that ectropion uvea, vascularity, pupil distortion, involvement of the angle and glaucoma could be evident in other types of lesions (nevus or melanoma) [23] They propose only the involvement of the ciliary body as the most indicative aspect of malignancy, while Shields is of the opinion that documented growth is the most important sign to diagnose iris melanoma [22]. After the evaluation of 1611 eyes, Shields et al constructed an ABCDEF guide to remember the risk factors that are predictive of iris nevus growth to melanoma, where A is age (young), B is blood (past episodes of hyphema), C is the clock hour (a tumor location from 4 o’clock to 9 o’clock), D is a diffuse configuration, E is ectropion and F is a feathery margin These key clinical features help to identify iris melanoma at a time in which therapy could be life-saving [22]. For a precise patient diagnosis, the following lesions in the iris need to be differentiated: congenital heterochromia, congenital ectropion iridis, Cogan–Reese syndrome (iridocorneal endothelial syndrome, which usually affects one eye of young to middle-aged women), siderosis, hemosiderosis, pigmentary glaucoma, melanocytomalytic glaucoma [21], Koeppe or Busacca nodules characteristic of sarcoidosis, Lisch nodules in neurofibromatosis, iris nevi, iris cysts, iris metastases, leiomyoma, juvenile xanthogranuloma and ciliary body melanoma with anterior extension [7]

Imaging Studies

Tumor Staging and Histological Findings

Surgical Excision

Proton Beam and Plaque Radiotherapy

Metastatic Spread

Findings

Conclusions