Abstract
Choroidal melanoma (CM), despite its rarity, is the most frequent intraocular malignancy. Over time, several histological variants of CM have been distinguished, including spindle A and B cell, fascicular, epithelioid and necrotic type. However, they have been progressively abandoned as having no prognostic value and currently, the American Joint Committee of Cancer (AJCC) classification identifies three CM cell types: spindle, epithelioid and mixed cell type. Other rare histological variants of CM include: (i) diffuse melanoma; (ii) clear cell; and (iii) balloon cell melanoma. Immunohistochemically, CMs are stained with Human Melanoma Black 45 (HMB45) antigen, S-100 protein, Melan-A (also known as melanoma antigen recognized by T cells 1/MART-1), melanocyte inducing transcription factor (MITF), tyrosinase, vimentin, and Sex determining region Y-Box 10 (SOX10). Several genetic and histopathological prognostic factors of CM have been reported in the literature, including epithelioid cell type, TNM staging, extraocular extension, monosomy 3 and 6p gain and loss of BAP-1 gene. The aim of this review was to summarize the histopathological, immunohistochemical and genetic features of CM, establishing “the state of the art” and providing colleagues with practical tools to promptly deal with patients affected by this rare malignant neoplasm.
Highlights
Despite being generally considered a rare neoplasm, uveal melanoma (UM) represents the most common primary ocular malignant lesion in adults [1]
[10]; (3) prognosis balloon cell melanoma characterized of cells with abundant cytoplasm containing multiple vacuoles that stain for lipids
Choroidal melanoma (CM) are positive for Human Melanoma Black 45 (HMB45) antigen, S-100 protein, Melan-A, melanocyte inducing transcription
Summary
Despite being generally considered a rare neoplasm, uveal melanoma (UM) represents the most common primary ocular malignant lesion in adults [1]. UM more frequently affects the choroid, the ciliary bodies and the iris [1,2,3]. Sci. 2020, 10, 8081 a better outcome than those affecting choroid or ciliary body, probably because iris melanomas are often visible from the outside of the eye and early diagnosed. Since the choroid is the most common site of onset, we will use the term “choroidal melanoma” (CM) to indicate the entire spectrum of melanomas arising from the uveal tract. Have traditionally been associated with a higher occurrence of CM: (i) presence of choroidal nevus,. CM is characterized by poor prognosis with distant metastases (liver is the most affected site) occurring in about 50% of patients within 10–15 years from diagnosis [1,2,3]
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