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Applied Sciences
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Ocular Melanoma: Current Concepts
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Ocular Melanoma: Current Concepts

A special issue of Applied Sciences (ISSN 2076-3417). This special issue belongs to the section "Applied Biosciences and Bioengineering".

Deadline for manuscript submissions: closed (28 February 2021) | Viewed by 22361

Special Issue Editors

Prof. Dr. Rosario Caltabiano

E-Mail Website
Guest Editor
Department G.F. Ingrassia – Section of Anatomic Pathology, University of Catania, via Santa Sofia no. 87, Catania (CT), Italy
Interests: dermatopathology; uveal melanoma; neuropathology; immunohistochemistry; FISH
Special Issues, Collections and Topics in MDPI journals
Dr. Andrea Russo

E-Mail Website
Guest Editor
Department of Ophthalmology, University of Catania, 95100 Catania, Italy
Interests: ocular oncology; uveal melanoma; vitreoretinal surgery; cataract surgery; medical retina
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Uveal melanoma (UM) is the most common neoplasm of the eye that develops in adults, displaying a high propensity for metastasis. UM can appear in the choroid, ciliary body, or iris of the eye, and can also rarely arise in melanocytes in the conjunctiva; melanoma of the conjunctiva accounts for ~2%–3% of all eye neoplasms.

Diagnosis is based principally on clinical examination of the tumor with biomicroscopy and indirect ophthalmoscopy and confirmed by diagnostic techniques such as ultrasonography, fundus fluorescein angiography, and optical coherence tomography.

Magnetic resonance and diffusion-weighted imaging (DWI) has been recently proven as a biomarker for the prediction and early detection of tumor response to eye-preserving therapies in ocular melanoma.

For most patients, the primary tumor is effectively controlled with surgical or radio-therapeutic approaches. Unfortunately, about 50% of patients will later present with metastases, usually affecting the liver. The time between treatment of the primary tumor and metastasis ranges from simultaneous presentation to decades later.

The most frequently mutated genes that are considered to be drivers in UM development and progression are BAP1, EIF1AX, GNA11, GNAQ, and SF3B1. In many cases, mutations in these genes appear in a mutually exclusive manner, have different risk of metastasis, and are consequently of prognostic importance.

This Special Issue will cover clinical, diagnostic, recent therapy advancements, and genetic and molecular aspects of UM. Manuscripts that include clinicopathological studies, in vitro studies, and in vivo studies with animals are welcome. A limited number of relevant case reports will also be accepted.

Prof. Dr. Rosario Caltabiano
Prof. Dr. Andrea Russo
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Applied Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Published Papers (6 papers)

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Editorial

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3 pages, 182 KiB  
Editorial
Ocular Melanoma: Current Concepts
by Rosario Caltabiano and Andrea Russo
Appl. Sci. 2021, 11(12), 5678; https://doi.org/10.3390/app11125678 - 19 Jun 2021
Viewed by 1102
Abstract
Uveal melanoma (UM) is the most frequent intraocular malignancy in adults, affecting the iris, choroid, and ciliary bodies [...] Full article
(This article belongs to the Special Issue Ocular Melanoma: Current Concepts)

Research

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20 pages, 6324 KiB  
Article
MR Imaging–Pathologic Correlation of Uveal Melanomas Undergoing Secondary Enucleation after Proton Beam Radiotherapy
by Pietro Valerio Foti, Corrado Inì, Mario Travali, Renato Farina, Stefano Palmucci, Corrado Spatola, Rocco Luca Emanuele Liardo, Roberto Milazzotto, Luigi Raffaele, Vincenzo Salamone, Rosario Caltabiano, Giuseppe Broggi, Lidia Puzzo, Andrea Russo, Michele Reibaldi, Antonio Longo, Paolo Vigneri, Massimo Venturini and Antonio Basile
Appl. Sci. 2021, 11(9), 4310; https://doi.org/10.3390/app11094310 - 10 May 2021
Cited by 7 | Viewed by 2027
Abstract
Background: Currently, radiotherapy represents the most widely employed therapeutic option in patients with uveal melanoma. Although the effects of proton beam radiotherapy on uveal melanoma end ocular tissues have been histologically documented, their appearance at MR imaging is still poorly understood. The purpose [...] Read more.
Background: Currently, radiotherapy represents the most widely employed therapeutic option in patients with uveal melanoma. Although the effects of proton beam radiotherapy on uveal melanoma end ocular tissues have been histologically documented, their appearance at MR imaging is still poorly understood. The purpose of our study was to elucidate the magnetic resonance (MR) semiotics of radiotherapy-induced changes to neoplastic tissues and ocular structures in patients with uveal melanoma undergoing secondary enucleation after proton beam radiotherapy. Methods: Nine patients with uveal melanoma who had undergone proton beam radiotherapy, MR imaging, and subsequent secondary enucleation were retrospectively selected. The histopathologic findings evaluated for irradiated tumors were necrosis, fibrosis, and viable tumor, while the histopathologic findings evaluated for extratumoral ocular/periocular tissues were radiation-related intraocular inflammation, vitreous hemorrhage, optic nerve degeneration, iris neovascularization, and periocular fibrotic adhesions. On MR images, the appearance of the abovementioned histologic features was assessed on conventional and diffusion-weighted sequences. Results: T2-weighted sequences performed better in detecting radiation-induced necrosis, fibrosis, optic nerve degeneration, and periocular fibrotic adhesions. T1-weighted sequences were preferable for identifying cataracts, vitreous hemorrhage, and inflammatory complications. Contrast-enhanced T1-weighted sequences were irreplaceable in assessing iris neovascularization, and in confirming inflammatory complications. Conclusions: In the light of their increasing role in the multidisciplinary management of patients with uveal melanoma, radiologists should be aware of the MR appearance of the effects of radiotherapy on neoplastic and ocular tissue, in order to improve the accuracy of follow-up MR examinations. Full article
(This article belongs to the Special Issue Ocular Melanoma: Current Concepts)
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Review

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8 pages, 755 KiB  
Review
Radiotherapy of Conjunctival Melanoma: Role and Challenges of Brachytherapy, Photon-Beam and Protontherapy
by Corrado Spatola, Rocco Luca Emanuele Liardo, Roberto Milazzotto, Luigi Raffaele, Vincenzo Salamone, Antonio Basile, Pietro Valerio Foti, Stefano Palmucci, Giuseppe Antonio Pablo Cirrone, Giacomo Cuttone, Andrea Russo, Teresio Avitabile, Michele Reibaldi, Antonio Longo, Giuseppe Broggi, Vincenza Bonfiglio, Rosario Caltabiano, Stefano Pergolizzi and Floriana Arena
Appl. Sci. 2020, 10(24), 9071; https://doi.org/10.3390/app10249071 - 18 Dec 2020
Cited by 9 | Viewed by 4872
Abstract
Conjunctival melanoma is a rare neoplasia, whose therapeutic management is generally of ophthalmological relevance, through radical surgical resection. The high incidence of local relapses after surgery, has made it necessary to combine various types of adjuvant treatments, which in some cases take on [...] Read more.
Conjunctival melanoma is a rare neoplasia, whose therapeutic management is generally of ophthalmological relevance, through radical surgical resection. The high incidence of local relapses after surgery, has made it necessary to combine various types of adjuvant treatments, which in some cases take on the role of radical treatments. Among these non-surgical treatments, those involving the application of ionizing radiation are becoming particularly important. In this review, we discuss the role of episcleral brachytherapy, external photon-beam radiotherapy, also made through stereotactic or radiosurgical modality, and of proton-beam radiotherapy. We try also to take stock of the benefits of the different irradiation modalities and the application difficulties of each. Full article
(This article belongs to the Special Issue Ocular Melanoma: Current Concepts)
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10 pages, 609 KiB  
Review
Iris Melanoma: Management and Prognosis
by Andrea Russo, Teresio Avitabile, Michele Reibaldi, Vincenza Bonfiglio, Francesco Pignatelli, Matteo Fallico, Rosario Caltabiano, Giuseppe Broggi, Daniela Russo, Silvia Varricchio, Corrado Spatola, Antonio Basile, Rocco Luca Emanuele Liardo, Roberto Milazzotto, Floriana Arena, Pietro Valerio Foti and Antonio Longo
Appl. Sci. 2020, 10(24), 8766; https://doi.org/10.3390/app10248766 - 08 Dec 2020
Cited by 7 | Viewed by 4046
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Ocular Melanoma: Current Concepts)
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10 pages, 4300 KiB  
Review
Histopathology and Genetic Biomarkers of Choroidal Melanoma
by Giuseppe Broggi, Andrea Russo, Michele Reibaldi, Daniela Russo, Silvia Varricchio, Vincenza Bonfiglio, Corrado Spatola, Cristina Barbagallo, Pietro Valerio Foti, Teresio Avitabile, Antonio Longo and Rosario Caltabiano
Appl. Sci. 2020, 10(22), 8081; https://doi.org/10.3390/app10228081 - 15 Nov 2020
Cited by 27 | Viewed by 4981
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Ocular Melanoma: Current Concepts)
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27 pages, 1824 KiB  
Review
Diabetic Retinopathy and Ocular Melanoma: How Far We Are?
by Eliana B. Souto, Joana R. Campos, Raquel Da Ana, Joana F. Fangueiro, Carlos Martins-Gomes, Alessandra Durazzo, Massimo Lucarini, Elena Sánchez López, Marta Espina, Maria Luisa García, Amélia M. Silva, Fernando Mendonça, Antonello Santini and Selma B. Souto
Appl. Sci. 2020, 10(8), 2777; https://doi.org/10.3390/app10082777 - 16 Apr 2020
Cited by 1 | Viewed by 4554
Abstract
Diabetic retinopathy causes vascular damage to retinal neurons, presenting characteristics of chronic inflammation. The development of new therapies capable of combating vision loss involves knowledge of inflammatory retinal changes. Studies in animal models and patients with diabetes have shown a high expression of [...] Read more.
Diabetic retinopathy causes vascular damage to retinal neurons, presenting characteristics of chronic inflammation. The development of new therapies capable of combating vision loss involves knowledge of inflammatory retinal changes. Studies in animal models and patients with diabetes have shown a high expression of the inflammatory molecules that are involved in the progression of diabetic retinopathy. Uveal melanoma is an eye tumour that remains highly deadly, because despite the correct treatment, it still causes metastasis in about 50% of patients. This type of tumour has the ability to produce and store melanin, which may result in resistance to therapy. Over time there has been development of new therapies for this disease, such as radiotherapy and surgical resection. In this review, we discuss diabetic retinopathy and ocular melanoma, their relationship with angiogenesis and the current anti-angiogenic therapies for their treatment. Full article
(This article belongs to the Special Issue Ocular Melanoma: Current Concepts)
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