Isolated choroidal melanocytosis: clinical update on 37 cases

Purpose Isolated choroidal melanocytosis is a congenital melanocytic hyperpigmentation involving the choroid that is not associated with iridic or scleral features of ocular melanocytosis. The purpose of this work was to describe the clinical features and course of a relatively large series of patients with this disorder. Methods A retrospective clinical study of 37 patients with isolated choroidal melanocytosis encountered in a single practice 1986–2018 was done. All lesions were 5 mm or larger in the largest basal diameter, homogeneously melanotic, and completely flat by conventional ocular ultrasonography. Results The 37 patients ranged in age from 2 weeks to 87 years (mean 31.5 years, median 18 years) at initial diagnosis of the melanotic choroidal lesion. Arc length largest basal diameter of the melanotic choroidal lesion ranged from 5.5 to 37 mm (mean 14.6 mm, median 13 mm). The lesion extended beneath the fovea in 18 eyes and to the optic disc margin in 6 eyes. Ten of the lesions straddled the ocular equator, but the center point of all of the lesions was posterior to the equator. The retina was fully attached and appeared normal over the melanotic choroidal lesion in each of these eyes. None of the melanotic choroidal lesions exhibited clumps of orange pigment or drusen on its surface. The lesion was unilateral and unifocal in 36 of the 37 patients. One patient had bilateral choroidal melanocytosis that was isolated in one eye but associated with partial iris melanocytosis in the fellow eye. Three adult patients had a choroidal melanoma localized to the patch of choroidal melanocytosis at baseline. One other adult patient had a choroidal melanoma in the fellow eye at baseline. One pediatric patient had viable unilateral non-familial retinoblastoma in the fellow eye and two adult patients had a classic choroidal nevus in the fellow eye. None of the flat patches of choroidal melanocytosis that were monitored periodically after initial diagnosis expanded appreciably during follow-up ranging from 4.9 months to 15.2 years (mean 5.0 years, median 2.3 years). Conclusions Isolated choroidal melanocytosis is a distinct clinical entity that must be distinguished from broad-based choroidal nevus, choroidal melanocytoma, small choroidal malignant melanoma, acquired bilateral patchy-streaky choroidal melanocytic fundopathy associated with disorders such as cutaneous vitiligo and Waardenburg syndrome, acquired bilateral zonal choroidal melanocytic fundopathy, and diffuse uveal melanocytic proliferation associated with systemic cancer. This disorder appears to predispose affected eyes to development of choroidal melanoma arising from the hypermelanotic patch.

Multifocal Intraretinal Tumour Deposits following Treatment of an Ipsilateral Choroidal Melanoma

<b><i>Background/Aims:</i></b> The aim of this study was to report a patient who, following plaque brachytherapy for a choroidal melanoma, developed two separate retinal foci of malignant melanoma in the same eye, and suggest possible mechanisms that might explain such an occurrence. <b><i>Methods:</i></b> We conducted a retrospective case report. <b><i>Results:</i></b> A 79-year-old Caucasian male developed a left inferotemporal choroidal melanoma in 2016 and subsequently underwent ruthenium-106 plaque brachytherapy under the care of the Scottish Ocular Oncology Service. Serial B-scan ultrasonography demonstrated a post-treatment reduction in tumour height to &#x3c;2 mm. In 2018, 27 months after the treatment, the patient complained of a “black spot” in the centre of his left eye vision. His visual acuity had reduced to counting fingers from 6/24 (corrected Snellen visual acuity). Several new areas of pigmentation and elevation were observed, and uveal melanoma recurrence was initially suspected. Given the multifocal nature of the presentation, the multi-disciplinary team recommended enucleation. Histological examination of the enucleated eye revealed three discrete foci of malignant melanoma – the previously treated choroidal malignant melanoma, and two solitary deposits of malignant melanoma within the retina. <b><i>Conclusion:</i></b> Retinoinvasive melanoma is a rare subtype of uveal melanoma, characterised by transvitreal melanoma invasion of the retina at a site non-contiguous with the uveal tumour, which may explain the clinical and pathological findings of the reported case.

Approach to the patient in the diagnosis of Choroidal Malignant Melanoma. Question and Answer

The most valuable diagnostic method in intraocular tumors is indirect ophthalmoscopy. USG is very important in determining the thickness of the lesion and documenting post-treatment changes. However, in today's conditions, we have important auxiliary examination opportunities. OCT is especially important in detecting macular edema and fluid. In choroidal melanomas, thickening and edema of the macula may be observed before treatment due to increased VEGF levels in the eye. OCT is important in detecting such changes. Since FAF shows the amount of lipofuscin in the lesion, it reveals the activity of the lesion. Hyperotofluorescence of> 50% of the lesion surface, in other words, the diffuse pattern is a risk factor for growth and metastasis. On fluorescein angiography, choroidal melanoma shows focal hyperfluorescence starting from the late venous phase and gradually increasing.

Discriminator and Diagnostic Features for Choroidal Malignant Melanoma and Choroidal Nevus

Choroidal nevus and choroidal malignant melanoma, especially when they are small in size, may have similar ophthalmoscopic appearance. Additionally, though rarely, choroidal nevi may convert to malignant melanomas over several years. Early detection of a small choroidal malignant melanomas presents the risk of growth, and metastasis is extremely critical for the preservation of both vision and eye. Currently, the usage of the mnemonic TFSOM-HHD has been suggested to find small ocular melanomas using helpful hints daily. In this review, we aim to provide the main discriminator and diagnostic features for choroidal malignant melanoma and choroidal nevus.

Experiences of two different modalities in the management of choroidal melanoma in the Asian Indian population

Background: Choroidal malignant melanoma is a rare intraocular cancer in Asian Indian population. There is a paucity of data from our population comparing iodine 125 (I-125) brachytherapy and enucleation. Aim: This study aims to compare two groups of choroidal melanoma patients treated with either I-125 brachytherapy or enucleation in the Asian Indian population. Objectives: To evaluate the long-term morbidity and mortality rates in choroidal melanoma patients treated with either I-125 brachytherapy or enucleation. Methods: It is a retrospective study involving forty four eyes, diagnosed with choroidal melanoma from May 2008 to February 2015. All were evaluated preoperatively for metastasis. Twenty-two underwent globe salvaging I-125 brachytherapy surgery whereas remaining 22 underwent globe destructive primary enucleation procedure. Results: At a mean follow-up 39 months, 2 of 22 cases (9%) died in the brachytherapy group. One was secondary to metastasis in the liver while the other case died of a natural cause. In the enucleation group also, there were two deaths (9%) secondary to metastasis. The metastasis free survival was 95% in the brachytherapy group and 91% in the enucleation group, which was not statistically significant (P = 0.3577). Kaplan–Meier survival at 3 years and 5 years was 95% and 89% for brachytherapy group and 94% and 89% for enucleation group. Conclusion: This study found no difference in the long-term survival of choroidal melanoma patients treated with either brachytherapy or enucleation in the Asian Indian eyes.