AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

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 US. 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 Bruch's membrane, optic nerve extension, lack of pigmentation, and histological characteristics such as mitotic activity and cell type.Although metastases from the primaryintraocularmelanomacanfirst 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

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

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 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, which are dilated episcleral blood vessels visible through the conjunctiva, which 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 development of choroidal melanoma.
• Predisposing diseases for uveal melanomas include family history of uveal melanoma, uveal nevus, congenital ocular melanocytosis, dysplastic nevus syndrome, and xeroderma pigmentosum.

DIFFERENTIALS

Section 4 of 10  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Benign and malignant tumors, cysts and other abnormal masses in the choroid, retina and pigment epithelium must be distinguished from choroidal melanomas.
Melanocytic nevus
Melanocytoma
Metastatic tumors
Medulloepithelioma (diktyoma)
Choroidal osteoma
Adenoma
Adenocarcinoma
Combined hamartoma of the retina and pigment epithelium
Congenital hypertrophy and reactive hyperplasia of the retinal pigment epithelium
Retinal cavernous hemangioma
Presumed acquired retinal hemangioma
Lymphoid tumor
Hemangiopericytoma
Leiomyoma
Neurofibroma
Glioneuroma
Astrocytoma
Neurilemmoma
Rhabdomyosarcoma
Posterior uveitis
Sarcoid nodules
Tubercular granuloma

WORKUP

Section 5 of 10  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• The most common site of choroidal melanoma metastasis is the liver.
• • Liver enzyme levels are indicated in any patient with uveal melanoma. The most sensitive tests of hepatic function are serum levels of alkaline phosphatase, glutamic-oxaloacetic transaminase, lactic dehydrogenase, and gamma-glutamyl transpeptidase.
  • These test results are negative at the time of diagnosis in most patients with choroidal melanoma. If any of these laboratory test results are abnormal, order ultrasonographic and CT scan studies of the liver. Unfortunately both imaging modalities have low sensitivity for metastasis smaller than 10-20 mm in diameter.

Imaging Studies

• A-scan ultrasound of the eye is a very useful tool in tumors more than 2-3 mm thick. Choroidal melanoma characteristically shows an initial prominent spike, followed by low-to-medium internal reflectivity with diminishing amplitude and a significant echo. Vascular pulsations can be seen as fine oscillations of the internal spiking pattern within the tumor. Standardized ultrasonography has a diagnostic accuracy of more than 95%. Performing sequential A-scans, with accurate dimension measurements, in cases of diagnostic uncertainty is important.
• B-scan ultrasound of the eye is a routine test used in the evaluation of any posterior segment mass. It is especially needed in patients with media opacity. For choroidal melanomas, B-scan is used to help establish the diagnosis, to evaluate possible extraocular extension, and to estimate tumor size for periodic observation and plan therapeutic intervention. Intraocular melanomas have several distinctive features, to include the following:
• • Low-to-medium reflectivity
  • Excavation of underlying uveal tissue
  • Shadowing of subjacent soft tissues
  • Internal vascularity
  • An acoustic quiet zone at the base of the tumor called acoustic hollowing
• Ultrasound biomicroscopy (UBM) uses high-frequency waves, with excellent resolution for anterior ocular abnormalities. It can differentiate very anterior choroidal melanomas from those of ciliary body origin and helps define the tumor's anterior border. It is also helpful in assessing angle-closure glaucoma.
• Fluorescein angiography and indocyanine green angiography do not show pathognomonic signs of choroidal melanoma but can help point to its diagnosis.
• Small choroidal melanomas may show fluorescein angiographic changes similar to some choroidal nevi, as follows: ranging from normal angiography to hypofluorescence secondary to blockage of background fluorescence.
• Larger melanomas may show a patchy pattern of early hypofluorescence and hyperfluorescence followed by late intense staining.
• Some choroidal melanomas demonstrate intrinsic vascularization, visible throughout the angiogram. The angiographic sign called "double circulation" pattern refers to simultaneous fluorescence of retinal and choroidal circulation within the tumor. When it occurs, it is fairly distinctive of choroidal melanomas.
• CT scan of the globe and orbit is more expensive than ultrasound and currently is not as sensitive. It is useful to see extraocular extension and may help differentiate between choroidal or retinal detachment and a solid tumor. CT scan requires intravenous injection of contrast material. Choroidal melanoma shows enhancement with contrast, where as exudation does not. CT scan also is very sensitive detecting calcium, a feature of some tumors that are different than uveal melanoma (characteristically choroidal osteoma).
• MRI of the globe and orbit is more expensive than CT scan. Use of surface coil imaging and gadolinium as a contrast material greatly improves its resolution, but it still remains less sensitive than ultrasound. Pigmented melanomas are seen as a high-density image in T1 and low-density image in T2. MRI also can be used to determine extrascleral extension of the melanoma and distinguish surrounding fluid from the tumor.
• Obtain a chest x-ray in patients with choroidal melanomas to rule out possible lung metastases.

Procedures

• Fine needle and incisional biopsy usually are not required but sometimes are helpful in difficult diagnostic cases, particularly to distinguish amelanotic melanomas from metastatic tumors, and if results from other ancillary tests are equivocal.
• • In experienced hands, both biopsy techniques have an accuracy of more than 95% in tumors larger than 3 mm.
  • Incisional biopsy is more invasive and may have more associated complications, but it has less false-negative and false-positive results. The most common complication for tumor biopsy is intralesional or perilesional hemorrhage.
  • Risk of spread of cancerous cells in the case of needle biopsy is small for choroidal melanoma (unlike retinoblastoma). Follow biopsy by prompt treatment to avoid extrascleral extension.

Histologic Findings

Histologic evaluation of the tumor after enucleation can confirm the diagnosis and evaluate prognosis. Three distinct cell types are recognized to occur in uveal melanomas, as follows: spindle A, spindle B, and epithelioid.

Spindle A cells have elongated nuclei and uncommonly have mitotic figures. Spindle B cells have a prominent nucleolus. They are found more commonly and also have an elongated profile but are slightly larger than spindle A cells. Epithelioid melanoma cells are highly anaplastic, poorly cohesive, and have considerable morphological variation. They tend to resemble epithelial cells and contain frequent mitotic figures.

The most commonly used histologic classification of uveal melanomas is the modified Callender classification. It divides uveal melanocytic tumors in several groups, as follows: spindle cell nevi, spindle cell melanomas, necrotic melanomas, epithelioid cell melanomas, and mixed cell melanomas. The latter two carry the poorest survival prognosis.

Evaluation of vascular supply of the tumor, age at presentation, presence of extrascleral extension, tumor size, tumor cell types, mitotic rate, nucleolar area, and quantification of nucleolar organizer regions have been used for prognostic purposes.

TREATMENT

Section 6 of 10  

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• Workup
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• Follow-up
• Miscellaneous
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• References

Medical Care

Several modes of treatment are available for choroidal melanomas. Multiple factors are taken into account when deciding one approach over other approaches, such as visual acuity of the affected eye, visual acuity of the contralateral eye, size of the tumor, age and general health of the patient, ocular structures involved, and presence of metastases.

• Observation may be acceptable for posterior uveal tumors where diagnosis is not well established. In particular, tumors of less than 2-2.5 mm in elevation and 10 mm in diameter can be observed until growth is documented.
• Photography and sequential ultrasonography for precise measuring of the tumor's dimensions is usually necessary.

Surgical Care

• Enucleation is the classic approach to choroidal melanomas and has been the preferred treatment for large (basal diameter >15 mm and height >10 mm) and complicated tumors, which compromise visual function, and where other therapies tend to fail.
  • Because of potential release of malignant cells into the bloodstream and orbital soft tissues during the surgical procedure, manipulation of the globe should be kept to a minimum.
  • Particular care has to be taken to avoid perforation of the globe during surgery. If transscleral extension is found, the tumor should be removed in one piece, followed by cryotherapy of the involved orbital soft tissues.
  • The theoretical advantage of enucleation over globe-sparing treatments is a reduced risk of metastatic spread. However, the Collaborative Ocular Melanoma Study (COMS), where medium-sized tumors were treated with either iodine 125 brachytherapy or enucleation, found that the mortality rates following brachytherapy did not differ from the mortality rates following enucleation for up to 12 years after treatment. Some investigators have advocated preenucleation radiation of the eye as a way to improve survival. However, the COMS demonstrated neither a positive effect nor a negative effect on the 10-year mortality rates among patients whose eyes containing large choroidal melanomas were randomized to treatment with enucleation alone or enucleation preceded by external radiation.
• Plaque brachytherapy is a widely accepted alternative to enucleation for medium size posterior uveal melanomas ( <10 mm in height and <15 mm in diameter).
  • Plaques containing various radioactive isotopes, such as iridium, cobalt, ruthenium, and other isotopes have been used. The most common material currently used in modern plaques is iodine 125, because of its lower energy emission (lack of alpha and beta rays), good tissue penetration, and its commercial availability. Radiation from this source causes tumor destruction through damage of DNA in cancerous cells and tumor vessels, with consequent tumor necrosis and regression. However, it is not devoid of complications. In a recent publication, Detorakis et al found that after iodine 125 brachytherapy for choroidal melanoma, iris and anterior chamber angle neovascularization developed in 23% of eyes.
  • A computerized calculation is used to determine the dose and duration of plaque application for a radiation delivery of approximately 400 Gy to the base and 80-100 Gy to the apex of the tumor, at 50-125 cGy/h.
  • The basal size of the melanoma is estimated preoperatively and confirmed during surgery. Appropriately sized plaques are sutured temporarily to the sclera and limbus underlying the melanoma. A margin of 2 mm over the largest tumor basal dimension is adequate. Intraoperative techniques such as transillumination or ultrasound are used to ensure proper plaque placement under the tumor.
  • Postoperative imaging confirmation of correct plaque localization is required. Radioactive plaques are left in place for 3-7 days. The goal of successful treatment is to achieve arrest of tumor growth or regression in size.
  • Local recurrence, usually requiring enucleation, occurs at a rate of about 12-16%. Plaque brachytherapy can cause complications, including cataract, rubeosis, scleral necrosis, keratopathy, radiation retinopathy, and optic neuropathy but at a reduced rate compared with external beam irradiation.
• External beam irradiation using charged particles, either protons or helium ions, is a frequently used alternative method to treat medium size choroidal melanomas ( <10 mm in height and <15 mm in diameter), although it has been used for larger tumors. It has similar indications and success rates to plaque brachytherapy.
• • After conjunctival incision and localization of the melanoma with transillumination, radiopaque tantalum rings usually are sutured to the sclera to serve as reference markers for alignment of the radiation beam. A collimated beam delivers about 70 Gy, divided usually in 5 sessions.
  • Vital ocular structures are avoided through careful positioning of the head and eye. Irradiation causes damage of DNA in cancerous cells and tumor vessels, in a similar way to plaque brachytherapy, with consequent tumor necrosis and regression. Treatment may be complicated with exudative retinal detachment, radiation cataract, dry eye syndrome, epithelial keratopathy, rubeosis, radiation retinopathy, and optic neuropathy.
  • Patients treated with this method seem to have a survival rate comparable to those treated by enucleation. Treatment is successful when it achieves arrest of tumor growth or regression in size. About 10-15% of eyes ultimately require enucleation, often because of neovascular glaucoma or local recurrence.
• Block excision, or sclerouvectomy, is an alternative treatment method for choroidal melanomas. It is reserved for small tumors covering less than a one third of the globe's circumference.
• • The goal of block excision is to salvage the eye, with most of these patients retaining some useful vision. It consists of full-thickness excision with in-block removal of tumor, choroid, retina, and sclera.
  • About 3 mm margin of healthy tissue around the melanoma is included, followed by closure with a graft of banked sclera. Surround treatment with cryotherapy or laser usually is added.
  • Most common complications are vitreous hemorrhage, retinal detachment, residual tumor, and cataract. Risks are improved by a modified approach, lamellar sclerouvectomy, which uses a partial-thickness scleral flap and minimizes altering the retina and vitreous. In a percentage of cases (about 15-20%), local reappearance of the melanoma requires subsequent treatment, usually enucleation.
• Laser photocoagulation and transpupillary thermotherapy are used in selected small choroidal melanomas, when they are located away from the fovea and are less than 3 mm in thickness.
• Orbital exenteration is a radical treatment reserved for cases with widespread orbital extension. Patients with such advanced melanomas are likely to have extensive distant metastases and poor prognosis for survival, with or without orbital exenteration surgery. The usefulness of such disfiguring surgery is not established and should only be considered in rare cases where marked discomfort is associated with massive orbital spread of the melanoma.
• Choice of treatment of choroidal melanoma remains controversial in many respects. Although enucleation has been the treatment of choice in the past, it appears that vision-sparing approaches might offer similar degrees of ocular and metastatic tumor control. Particularly, because it is clear that in many patients at the time of diagnosis, posterior uveal melanomas already have spread through micrometastasis.
• A multicenter randomized trial conducted by the Collaborative Ocular Melanoma Study (COMS) Group regarding conservative management revealed that patient survival after treatment of medium-size melanoma is similar when comparing plaque radiotherapy versus enucleation.
• Although undetected metastatic spread at the time of diagnosis and treatment of choroidal melanoma is a major concern in every patient, adjuvant systemic treatment currently is not advocated. This consensus comes from treatment trials with intraocular melanomas and extrapolation of the experience with cutaneous melanoma, where adjuvant treatment has shown no benefit.
• In cases where distant metastases are found during the initial systemic workup, treatment of the intraocular melanomas becomes palliative. Systemic chemotherapy is the primary treatment.
• Many modalities and combinations of chemotherapeutic and immunotherapeutic agents exist, but for the most part results continue to be disappointing. This is an area of intense medical research with ever-increasing degrees of biological sophistication being applied to new clinical trials.

Consultations

• Oncology
• Radiation oncology

FOLLOW-UP

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Outpatient Care

• Irrespective of the treatment modality chosen, patients with choroidal melanomas need to be observed carefully and for many years.
• • This is particularly true for small tumors, when the diagnosis is not established clearly. Close observation and measurement of the tumors' dimensions with any of the diagnostic tools mentioned earlier (see Lab Studies) is critical.
  • Repeat examinations usually are performed about every 3 months initially, and if no changes are seen, follow-up care is performed every 6 months. If growth of the lesion is detected, consider further treatment. Choroidal melanomas may show size regression starting several months after being treated with external beam irradiation or plaque brachytherapy. The goal of successful treatment is not necessarily reduction in size but long-term arrest of tumor's growth.
  • Repeat examinations and imaging tests are performed after all treatment modalities because of the possibility of intraocular or extraocular tumor recurrence.
  • Follow-up care in patients with treated choroidal melanomas should include thorough physical examinations, liver function tests, and imaging of lungs, repeated about every 6-12 months. Early detection of distant metastases may affect management and survival.

Deterrence/Prevention

• Patients with choroidal nevi, family history of uveal melanoma, congenital ocular melanocytosis, dysplastic nevus syndrome, and other predisposing conditions of uveal melanoma may benefit from annual careful ophthalmologic examinations.
• Limiting excessive ocular sunlight exposure through sunglasses or other means may have a theoretical preventive effect in patients with a predisposition to intraocular melanoma.

Prognosis

• Visual prognosis is guarded for choroidal melanomas. The tumor or the treatment can cause a variety of ocular complications that severely affect vision, as discussed in Pathophysiology and Treatment.
• Choroidal melanomas have an overall mortality of about 30-50% within 10 years from initial diagnosis and treatment. Deaths are mostly secondary to distant metastases. Characteristics of the tumor that have been found to correlate with decreased survival include larger size, anterior location, transscleral extension, growth through the Bruch membrane, optic nerve extension, lack of pigmentation, increased vascularization, and histological features such as mitotic activity and cell type.

MISCELLANEOUS

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Two circumstances put choroidal melanoma in a special category regarding medical legal liability, as follows:
• • Other choroidal masses can present with very similar characteristics to choroidal melanoma, making it a challenging diagnosis.
  • Choroidal melanoma is a disease with a high mortality rate, usually irrespective of the chosen treatment modality.

MULTIMEDIA

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Color photograph of a dome-shaped choroidal melanoma.
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Media file 2:  Early fluorescein angiogram of choroidal melanoma in Image 1 showing intrinsic vascularity.
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Media file 3:  Late fluorescein angiogram of choroidal melanoma in Image 1 showing early diffuse staining.
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Media file 4:  B-scan showing acoustic hollowing and uveal excavation in posterior choroidal melanoma seen in Image 1.
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Media file 5:  A-scan of choroidal melanoma seen in Image 1 showing low-to-medium internal reflectivity.
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Media file 6:  B-scan showing acoustic hollowing in intraorbital extension of a posterior choroidal melanoma.
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Media file 7:  T2-weighted MRI showing a small anterior choroidal melanoma in the left eye.
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Media file 8:  Transpupillary photograph showing a posterior choroidal melanoma.
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Media file 9:  Photograph showing an enucleated eye with advanced choroidal melanoma with transscleral extension.
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Media file 10:  Histologic section of an enucleated eye showing a large dome-shaped choroidal melanoma.
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Media file 11:  Histologic section of an enucleated eye showing a medium-sized mushroom-shaped choroidal melanoma with associated exudative retinal detachment.
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Media file 12:  Choroidal melanoma. Histologic section showing spindle A cells in a uveal nevus.
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Media file 13:  Choroidal melanoma. Histologic section showing spindle B cells in a uveal melanoma.
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Media file 14:  Choroidal melanoma. Histologic section showing epithelioid cells in a uveal melanoma.
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Media file 15:  Photograph showing a skin metastasis of a posterior choroidal melanoma.
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Media file 16:  Intraoperative photograph showing placement of a radioactive plaque for posterior choroidal melanoma.
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REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

• Augsburger JJ, Peyster RG, Markoe AM, et al. Computed tomography of posterior uveal melanomas. Arch Ophthalmol. Nov 1987;105(11):1512-6. [Medline].
• Augsburger JJ, Correa ZM, Freire J, Brady LW. Long-term survival in choroidal and ciliary body melanoma after enucleation versus plaque radiation therapy. Ophthalmology. Sep 1998;105(9):1670-8. [Medline].
• Augsburger JJ, Gamel JW, Sardi VF. Enucleation vs cobalt plaque radiotherapy for malignant melanomas of the choroid and ciliary body. Arch Ophthalmol. May 1986;104(5):655-61. [Medline].
• Augsburger JJ, Correa ZM, Freire J, Brady LW. Long-term survival in choroidal and ciliary body melanoma after enucleation versus plaque radiation therapy. Ophthalmology. Sep 1998;105(9):1670-8. [Medline].
• Cham MC, Pavlin CJ. Ultrasound detection of posterior scleral bowing in young patients with choroidal melanoma. Can J Ophthalmol. Aug 2000;35(5):263-6. [Medline].
• Chang M, Shields JA, Wachtel DL. Adenoma of the pigment epithelium of the ciliary body simulating a malignant melanoma. Am J Ophthalmol. Jul 1979;88(1):40-4. [Medline].
• Char DH, Kroll S, Quivey JM, Castro J. Long term visual outcome of radiated uveal melanomas in eyes eligible for randomisation to enucleation versus brachytherapy. Br J Ophthalmol. Feb 1996;80(2):117-24. [Medline].
• Char DH, Kroll SM, Miller T, Castro J, Quivey J. Irradiated uveal melanomas: cytopathologic correlation with prognosis. Am J Ophthalmol. Oct 1996;122(4):509-13. [Medline].
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Article Last Updated: Sep 25, 2006