eMedicine Specialties > Ophthalmology > Choroid

Melanoma, Choroidal

Author: Enrique Garcia-Valenzuela, MD, PhD, Clinical Assistant Professor, Department of Ophthalmology, University of Illinois Eye and Ear Infirmary; Consulting Staff, Vitreo-Retinal Surgery, Midwest Retina Consultants, SC, Parkside Center
Coauthor(s): Mauricio E Pons, MD, Associate Physician, Charles A Garcia, MD, PA; James E Puklin, MD, Professor, Department of Ophthalmology, Vitreoretinal Service, Kresge Eye Institute, Wayne State University; Cathleen A Davidson, MSc, Consulting Staff, Department of Obstetrics and Gynecology, Laboratory of Assisted Reproductive Technologies, University of Michigan
Contributor Information and Disclosures

Updated: Jun 24, 2009

Introduction

Background

Choroidal melanoma is the most common primary malignant intraocular tumor and the second most common type of primary malignant melanoma in the body. It is nevertheless an infrequently found tumor.

Choroidal melanoma is a subtype of uveal melanoma. Uveal melanomas can be classified in anterior uveal melanomas, when the tumor arises in the iris, and in posterior uveal melanomas, when it arises in either the choroid or the ciliary body. Intraocular melanomas simultaneously can involve more than one uveal structure.

The ocular tissue where these tumors arise, the uvea, is a densely pigmented layer that forms part of the wall of the eye. The uvea is subdivided into iris, ciliary body, and choroid. The choroid underlies the retina and its pigment epithelium throughout the ocular fundus. The main function of the uvea is to provide oxygen and other nourishment to the highly metabolically demanding retinal photoreceptors. It is primarily a vascular tissue, with fenestrated capillaries and stroma containing melanocytes.

Pathophysiology

Primary choroidal melanoma arises from melanocytes within the choroid. Most choroidal melanomas are believed to develop from preexisting melanocytic nevi. De novo growth of choroidal melanomas also occurs. Three distinct cell types are recognized in choroidal and other uveal melanomas, as follows: spindle A, spindle B, and epithelioid. The latter cell type usually has the most aggressive behavior and carries a poorer prognosis for the patient's long-term survival.

Choroidal melanomas may have variable coloration, ranging from darkly pigmented to purely amelanotic. They typically are domed-shaped. As they enlarge, if they break through the Bruch membrane, they can assume a mushroom configuration. Other shapes found for these tumors are bilobular, multilobular, and diffuse. The latter is characterized by lateral growth throughout the choroid with minimal elevation. It occurs in about 5% of cases. Rarely, choroidal melanomas may arise in a multicentric distribution in one or both eyes.

Choroidal melanomas affect the retinal pigment epithelium as they push against it and deprive it from normal choroidal circulation. Overlying retinal pigment epithelium usually develops areas of atrophy, drusen, and localized pigment epithelial detachments. Areas of phagocytic activity, digesting cellular debris from melanocytes, give the pigment epithelium patches of coloration change. Macrophages within these typically orange areas contain melanin and lipofuscin. These changes can lead to choroidal neovascularization over the tumor, with consequent subretinal exudation, hemorrhage, and fibrous plaque formation.

Growth of choroidal melanomas can occur silently until it produces enough visual loss through various mechanisms. The tumor's disruption of choroidal circulation and consequent ischemia typically causes degeneration of retinal photoreceptors and other retinal neurons. The retina overlying the tumor can separate into cystoid spaces and larger schisis cavities. There may be associated cystoid macular edema.

In general, the farther away the tumor's origin is located from the optic nerve and fovea, the larger size it can reach before the patient notices a visual field defect. Exudation of fluid into the subretinal space with consequent retinal detachment may enlarge the field loss. This exudation can lead to total retinal detachment. Rarely, choroidal melanomas can impinge into underlying posterior ciliary nerves, causing severe ocular pain. Other signs and symptoms can result if the tumor grows anteriorly, pathologically involving the ciliary body, trabecular meshwork, and lens, with consequent ocular hypotension or hypertension and cataract. Large choroidal melanomas can induce iris rubeosis. Erosion of the melanoma into blood vessels in adjacent tissues, or areas of necrosis within the tumor, can lead to vitreous hemorrhage or hyphema.

Choroidal melanomas ultimately cause death, practically always secondary to distant metastases rather than local spread. Its metastatic potential depends on the histopathologic aggressiveness of the tumor cells. Unfortunately, not infrequently it metastasizes before diagnosis. If the melanoma does not show extraocular extension, it can only spread hematogenously, because there are no lymphatic vessels in the eye. It most often metastasizes to the liver. Other organs of dissemination are lung, bone, skin, and CNS. Less frequently, choroidal melanoma can grow transsclerally, through emissary channels, and metastasize locally into the orbit or rarely the conjunctiva. Choroidal melanoma almost never extends through the optic nerve, and, when it happens, it is usually in juxtapapillary tumors or in diffuse choroidal melanomas.

Frequency

United States

Incidence of primary choroidal melanoma is about 6 cases per 1 million population. Perhaps because of increased sunlight exposure, there appears to be a higher incidence of uveal melanoma in the southern latitudes of the United States. Alternatively, this might be the effect of a tendency of older Americans to retire in the South.

International

Incidence of choroidal melanoma is much higher in countries with large numbers of people of northern European descent than elsewhere in the world. In Denmark and other Scandinavian countries, incidence is about 7.5 cases per million per year.

Mortality/Morbidity

  • About 30-50% of patients with choroidal melanoma will die within 10 years from diagnosis and treatment. It is usually secondary to distant metastases, and the risk is greatest in larger tumors.
    • For large melanomas, the Collaborative Ocular Melanoma Study found the 10-year rates of death secondary to metastasis were 45% and 40% in pre-enucleation radiation and in the enucleation alone treatment groups, respectively. There appears to be no survival benefit attributable to pre-enucleation radiation. The maximum diameter of the base of the tumor and older age were the primary predictors of time to death in patients with melanoma metastasis.
    • Previous publications have found several tumor features to correlate with increased mortality, including larger size, anterior location, transscleral extension, growth through the Bruch membrane, optic nerve extension, lack of pigmentation, and histological characteristics, such as mitotic activity and cell type. Although metastases from the primary intraocular melanoma can first be detected years later, their highest incidence is in the first year after diagnosis. As yet, no effective treatment exists for metastatic uveal melanoma.
  • Choroidal melanoma normally leads to partial or total visual loss in the affected eye. This is the result of either tumor destruction of ocular structures or consequence to the treatment used. Patients with small- to medium-sized choroidal melanomas may be able to preserve very good central vision, even after treatment.

Race

Choroidal melanoma and other uveal melanomas most often affect whites of northern European descent. Incidence of choroidal melanoma among blacks is extremely rare. Hispanics and Asians are thought to have a small but intermediate risk compared to whites and blacks.

Sex

Choroidal melanoma is found slightly more frequently in men for all age groups, except from 20-39 years, when a small predilection exists for women.

Age

Incidence of choroidal melanoma is highest around age 55 years. In Asians, although it is a very infrequent tumor, reports indicate a peak incidence at a somewhat younger age. Choroidal melanoma is exceptional in children.

Clinical

History

Choroidal melanomas remain asymptomatic for prolonged periods of time, when they can be found incidentally during ophthalmoscopy. In general, the more anterior their origin, the longer the delay of any symptoms. Choroidal melanoma might present with the following symptoms:

  • Blurred visual acuity consequent to various mechanisms, including growth of the melanoma into the subfoveal retina, cystoid macular edema, retinal detachment, vitreous hemorrhage, cataract, and blockage of the visual axis directly by the tumor.
  • A paracentral scotoma may occur as the tumor affects the perifoveal retina.
  • Painless and progressive visual field loss may occur as peripheral melanoma grows or exudates subretinal fluid.
  • Floaters can be reported when areas of necrosis within the tumor or adjacent structures produce vitreous hemorrhage or hyphema.
  • Occasionally, choroidal melanomas may produce severe ocular pain when they impinge into posterior ciliary nerves. They also can cause pain secondary to high intraocular pressure from acute angle-closure glaucoma.
  • History of weight loss, marked fatigue, cough, or change in bowel or bladder habits should prompt consideration of primary nonocular malignancy with choroidal metastasis.

Physical

Patients with choroidal melanoma may present with painless visual loss or, occasionally, inflammation and pain from a complicated tumor. However, many patients have no symptoms, and melanomas are discovered on routine ophthalmologic examination.

  • Small choroidal melanomas are typically a nodular, domed-shaped, and well-circumscribed mass under the retinal pigment epithelium. As they grow, they may adopt more irregular configurations, such as bilobular, multilobular, or mushroom shapes.
  • An unusual tumor type that may be found is diffuse choroidal melanoma, characterized by lateral growth throughout the choroid with minimal elevation. Diffuse melanomas are more difficult to diagnose and often cause significant exudative retinal detachment.
  • Choroidal melanomas may have variable coloration, ranging from amelanotic to darkly pigmented. Some tumors are partially pigmented. If lightly colored, the abnormal vascularization of the tumor usually can be seen ophthalmoscopically. Overlying the choroidal melanoma there are usually retinal pigment epithelial changes (eg, drusen), patches of atrophy, and orange discoloration. The latter traditionally has been regarded as strongly indicative of malignancy. It is now well known that orange changes in the pigment epithelium can be seen over benign lesions as well.
  • Sometimes, a choroidal melanoma may remain undetected underneath a large exudative retinal detachment, a subretinal hemorrhage, or a vitreous hemorrhage.
  • Infrequent presentations of advanced choroidal melanoma are a painful blind eye with cataract and proptosis from tumor transscleral orbital extension.
  • Anterior choroidal melanomas might show sentinel vessels (dilated episcleral blood vessels visible through the conjunctiva) that feed the metabolically active tumor.
  • Transscleral growth of an anterior choroidal melanoma (mainly through emissary channels) may appear on examination as a small subconjunctival area of abnormal hyperpigmentation.
  • Transillumination can be used to find the borders of the tumor, especially if it is surrounded by exudative retinal detachment. Its precision is dependent on even tumor pigmentation and if associated hemorrhage is present.
  • Overall accuracy of the clinical diagnosis of choroidal melanoma, using modern diagnostic tools, was shown to be 99.7% in a preliminary report from the Collaborative Ocular Melanoma Study. Thorough evaluation of choroidal melanoma should include a complete physical examination, with particular attention to the hepatic-abdominal region and the skin and subcutaneous tissues, which are frequent sites of metastatic spread.

Causes

  • A particular predisposition exists for choroidal melanomas to occur in people with light-colored irides. Evidence points to sunlight exposure as a likely contributor to the development of choroidal melanoma.
  • Predisposing diseases for uveal melanomas include a family history of uveal melanoma, uveal nevus, congenital ocular melanocytosis, dysplastic nevus syndrome, and xeroderma pigmentosum.

More on Melanoma, Choroidal

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References
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Further Reading

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Keywords

choroidal melanoma, malignant choroidal melanoma, intraocular tumor, intraocular melanoma, malignant tumor, malignant melanoma, uveal melanoma, primary malignant melanoma, anterior choroidal melanoma, posterior choroidal melanoma, anterior uveal melanoma, posterior uveal melanoma, uvea, iris, choroid, ciliary body, uveal structure

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Contributor Information and Disclosures

Author

Enrique Garcia-Valenzuela, MD, PhD, Clinical Assistant Professor, Department of Ophthalmology, University of Illinois Eye and Ear Infirmary; Consulting Staff, Vitreo-Retinal Surgery, Midwest Retina Consultants, SC, Parkside Center
Enrique Garcia-Valenzuela, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, Retina Society, and Society for Neuroscience
Disclosure: Nothing to disclose.

Coauthor(s)

Mauricio E Pons, MD, Associate Physician, Charles A Garcia, MD, PA
Mauricio E Pons, MD is a member of the following medical societies: American Academy of Ophthalmology and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

James E Puklin, MD, Professor, Department of Ophthalmology, Vitreoretinal Service, Kresge Eye Institute, Wayne State University
Disclosure: Nothing to disclose.

Cathleen A Davidson, MSc, Consulting Staff, Department of Obstetrics and Gynecology, Laboratory of Assisted Reproductive Technologies, University of Michigan
Disclosure: Nothing to disclose.

Medical Editor

Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas
Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians & Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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