Uveal Melanoma: A Review of the Literature and Personal Experiences

Background: Uveal melanoma is the most common primary intraocular malignancy in adults, often resulting in painless vision loss. We report a case of necrotic uveal melanoma presenting with orbital inflammation mimicking orbital cellulitis and present a comprehensive review of the literature and tabulation of reported cases. Summary: Our review found 44 published reports of spontaneously necrotic uveal melanoma involving 55 patients. Of these reports, 26 patients (47%) presented with orbital cellulitis. Presenting symptoms of necrotic uveal melanoma with orbital cellulitis included proptosis (82.8%), pain (80.7%), vision loss (61.5%), and restricted extraocular movements (46.2%). Key Messages: Uveal melanoma can rarely mimic orbital cellulitis. Autoinfarction and tumor necrosis causes secondary orbital inflammation. Intraocular malignancy must remain in the differential for patients with orbital inflammation and vision loss.

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Case Report with Review of the Literature: Uveal Melanoma in a Patient with Carney Complex – Another Rare Component of the Syndrome?

Ocular Oncology and Pathology ◽

10.1159/000506205 ◽

2020 ◽

Vol 6 (5) ◽

pp. 311-317

Author(s):

Diva R. Salomão ◽

Cristiane M. Ida ◽

Patricia T. Greipp ◽

J. Aidan Carney

Keyword(s):

Case Report ◽

Uveal Melanoma ◽

Ciliary Body ◽

Carney Complex ◽

Review Of The Literature ◽

Poor Vision ◽

Monosomy 3 ◽

Molecular Studies ◽

Complex Genome

A 74-year-old woman with Carney complex (CNC) and complaints of poor vision was found, on ophthalmic examination, to have a pigmented tumor involving the peripheral choroid and ciliary body in her right eye. The eye was enucleated and showed a ciliochoroidal melanoma with marked pleomorphism. The tumor did not recur or metastasize after almost 10 years of follow-up, and the patient died of unrelated causes. Molecular studies revealed a complex genome with multiple whole-chromosome losses including monosomy of chromosomes 1, 2 (including loss of CNC2at 2p16), 14, 17 (including loss of a copy of PRAKA1 at 17q24.2), 18, 19, 21, 22, and X. No monosomy 3 was observed. This is only the second case of uveal melanoma in a patient with CNC, raising the possibility that this might represent a rare component of this syndrome.

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Lentigo Maligna: Melanoma In Situ on Chronically Sun-Damaged Skin

Archives of Pathology & Laboratory Medicine ◽

10.5858/2011-0051-rair.1 ◽

2011 ◽

Vol 135 (7) ◽

pp. 838-841

Author(s):

Jon A. Reed ◽

Christopher R. Shea

Keyword(s):

Review Of The Literature ◽

Lentigo Maligna ◽

Superficial Spreading ◽

Anatomic Site ◽

Classification Schemes ◽

Melanoma In Situ ◽

Personal Experiences ◽

Lentigo Maligna Melanoma ◽

Histologic Subtypes

Abstract Context.—Cutaneous primary invasive malignant melanoma often is classified by its histologic appearance. Major recognized histologic subtypes of melanoma include superficial spreading, lentigo maligna melanoma, nodular, and acral lentiginous. More recently, it has been shown that most primary invasive melanomas harbor nonrandom genetic or biochemical aberrations that correlate with anatomic site or with the amount of cutaneous exposure to sunlight. It also is generally accepted that most primary invasive melanomas are preceded by an intraepidermal atypical melanocytic proliferation that lacks invasive capability (melanoma in situ). Objective.—To focus on lentigo maligna, the preinvasive/in situ form of melanoma located on chronically sun-damaged skin. Data Sources.—Review of the literature and the authors' personal experiences. Conclusions.—A better understanding of the earliest stage of melanoma progression, including the contribution of chronic exposure to ultraviolet radiation, may lead to improved classification schemes that direct more effective targeted or personalized therapies for patients.

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