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  

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

Background

Ciliary body melanoma is a subtype of uveal melanoma. Uveal melanomas are the most common primary intraocular malignancies and the second most common type of primary malignant melanoma in the body. They can be classified as anterior uveal melanomas when the tumor arises in the iris and as posterior uveal melanomas when it arises in either the choroid or the ciliary body. Intraocular melanomas can involve 2 uveal structures simultaneously, such as in ciliochoroidal melanoma.

The ocular tissue where these tumors arise, the uvea, is a densely pigmented layer that lies for the most part between the sclera and the retina. The uvea is subdivided into iris, ciliary body, and choroid. The ciliary body is located between the iris and the ora serrata. It has a specialized function in the uveal tract; it produces aqueous humor, facilitates trabecular outflow, intervenes in alteration of the shape of the crystalline lens during accommodation, and secretes hyaluronic acid into the vitreous.

Ciliary body melanoma is an infrequent tumor. It is encountered less commonly than choroidal melanoma, with a ratio of occurrence of 1 in 10 to that in the choroid. Nevertheless, uveal melanomas are the most common primary malignant tumor in the ciliary body.

Pathophysiology

Primary ciliary body melanoma arises from melanocytes in the uveal tract. Although uveal melanomas may grow de novo, most develop from a preexisting melanocytic nevus. Three distinct cell types are recognized in uveal melanomas, as follows: spindle A, spindle B, and epithelioid. Presence of the latter type of cell is associated with more aggressive behavior and carries a poorer prognosis for the patient's survival.

Local growth of ciliary body melanoma produces signs and symptoms as it pathologically involves adjacent structures. Separation and disruption of the overlying ciliary epithelium decreases its production of aqueous humor with consequent ocular hypotension. Growth of the melanoma into the lens may produce its subluxation, lenticular astigmatism, or cataract. Erosion of the tumor into blood vessels in adjacent tissues, or areas of necrosis within the tumor, can lead to hyphema or vitreous hemorrhage. Ciliary body melanomas can push the iris diaphragm anteriorly, or they can infiltrate the trabecular meshwork, producing acute angle closure.

Melanoma in the ciliary body poses a serious threat to life. It usually remains hidden behind the iris diaphragm, growing undetected for longer periods of time than melanoma in the iris or choroid. Patients who die from ciliary body melanoma die because of distant metastasis rather than local spread. Its metastatic potential depends on the phenotype of the tumor cells, and it frequently disseminates before diagnosis. If the melanoma does not show extraocular extension, it only can disseminate hematogenously, since there are no lymphatic vessels in the eye. It has a tendency to spread preferentially to the liver. Other frequent sites of metastasis are lung, bone, skin, and CNS. Less frequently, ciliary body melanoma can grow transsclerally, through emissary channels, and metastasize locally into the orbit and conjunctiva.

Frequency

United States

Incidence of primary intraocular melanoma is 4.3-6 cases per 1 million population. Ciliary body melanoma represents about one tenth of all intraocular melanomas. A higher incidence of uveal melanoma has been reported in the southern latitudes of the United States than in the northern latitudes. This difference may be the effect of more sunlight exposure in the lower latitudes or a tendency of older Americans to retire in the South.

International

Incidence of intraocular melanoma is much greater in countries with people of northern European descent than elsewhere in the world. In Denmark and other Scandinavian countries, the annual incidence is about 7.5 cases per 1 million population.

Mortality/Morbidity

An overall mortality rate of approximately 30-50% occurs from ciliary body melanoma within 10 years from diagnosis and treatment. Most often, it is related to the development of distant metastasis. Peak incidence of metastasis occurs during the first year after diagnosis of the primary intraocular melanoma; however, these can appear for the first time years later.

• For uncertain reasons, ciliary body and anterior choroidal melanomas have a worse prognosis for patient survival than posterior choroidal melanomas. Delayed diagnosis is probably partly responsible.
• No effective treatment exists yet for metastatic uveal melanoma. 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.
• Ciliary body melanomas cause partial or total visual loss in the affected eye, from direct pathological involvement of ocular structures or as a result of the treatment used.

Race

Uveal melanoma, including that in the ciliary body, is mostly a disease of white people, particularly of northern European descent and is rarely seen in nonwhite races. Most cases (97.8%) occur in the white population. Incidence of ocular melanoma among blacks is extremely rare. Hispanics and Asians are thought to have an intermediate risk compared to whites and blacks.

Sex

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

Age

Uveal melanoma shows a peak incidence at 55 years. In Asians, a tendency exists for the condition to occur at a younger age. A recent study in Europe reported that 1.4% of uveal melanomas occur in people younger than 20 years. Uveal melanoma is generally exceptional in children.

CLINICAL

Section 3 of 10  

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• Clinical
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History

Ciliary body melanomas can remain asymptomatic until they grow enough to affect neighboring ocular structures. Patients may present with the following symptoms:

• Blurred vision consequent to growth of the melanoma into the crystalline lens, leading to lenticular astigmatism or cataract; they also may block the visual axis directly or via an intraocular hemorrhage.
• Floaters can be reported when areas of necrosis within the tumor or adjacent structures produce vitreous hemorrhage or hyphema.
• Painless visual field loss may be present as the melanoma grows centrally and posteriorly.
• Severe ocular pain occasionally can be associated with ciliary body melanoma, secondary to high intraocular pressure because of 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 ciliary body metastasis.

Physical

A preliminary report from the COMS showed that clinical diagnosis has an accuracy rate of 99.7%. The premanagement evaluation of ciliary body melanoma should include a thorough physical examination, with particular attention to the hepatic abdominal region and the skin and subcutaneous tissues, which are frequent sites of metastatic spread.

• Patients can present with painless visual loss or inflammation and pain from a complicated tumor, but many patients have no symptoms, and melanomas are discovered on routine ocular examination.
• An early sign of an occult ciliary body melanoma is a sentinel vessel, which is one or more dilated episcleral blood vessels feeding the metabolically active tumor and is visible through the conjunctiva overlying it. This finding should prompt the physician to dilate and carefully examine the anterior-posterior segment.
• Another early physical sign of an occult ciliary body melanoma is unexplained unilateral low intraocular pressure, as compared to the healthy fellow eye. A difference of 5 mm Hg or more may be the only initially detectable external sign of a tumor affecting the ciliary body.
• Posterior uveal melanomas can grow into the sclera (mainly through emissary channels) and extrasclerally. If located anteriorly enough, it may appear on examination as a small subconjunctival area of abnormal hyperpigmentation.
• On occasion, melanomas in the ciliary body can grow anteriorly into the anterior chamber pushing the iris root centrally, becoming visible on biomicroscopy.
• • Most ciliary body melanomas can be observed as a darkly pigmented mass posterior to the pupil. Nevertheless, pigmentation ranges from inapparent to dark brown.
  • These tumors may have a diffuse, nodular, or mixed pattern.
  • Most commonly, they are solitary, dome-shaped, sessile tumors, although multicentric melanomas have been described.
  • They usually are solid but can be cystic.
  • Some ciliary body melanomas with diffuse growth patterns can extend around the circumference of the ciliary body for 360°; they are known as ring melanomas and have a greater tendency to metastasize and grow extrasclerally. They can cause such secondary effects as cataract; lens subluxation; hyphema; orbital involvement via extrascleral extension; and, rarely, corneal involvement.
• Tumor-induced glaucoma may be produced by obstruction of outflow pathways by pigment cells (pigment dispersion syndrome), melanin-laden macrophages (melanomalytic glaucoma), or tumor cells. Additional mechanisms of glaucoma include rubeosis iridis, angle closure, and direct invasion of angle structures.
• Location of the melanoma in the ciliary body makes diagnosis difficult because pupillary dilation and indirect ophthalmoscopy or a 3-mirror contact lens is needed to visualize the lesion.
• Transillumination is helpful in localizing the tumor. Its accuracy is dependent on melanin content, dark pigmentation, and if a hemorrhage is present in the tumor.

Causes

A strong predisposition exists for ciliary body melanomas to occur in white patients, particularly in those with light-colored irides. Some evidence suggests that increased sunlight exposure contributes to the development of ciliary body melanoma, but this is not well established.

• Predisposing conditions for uveal melanoma include the following:
• • Uveal nevus
  • Congenital ocular melanocytosis
  • Xeroderma pigmentosum
  • Dysplastic nevus syndrome
  • Family history of uveal melanoma
• Other diagnostic considerations: Ciliary body melanomas must be distinguished from benign and malignant tumors, cysts, and other abnormal masses in the ciliary body.

DIFFERENTIALS

Section 4 of 10  

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• References

Other Problems to be Considered

Melanocytic nevus
Melanocytoma
Metastatic tumors
Medulloepithelioma (diktyoma)
Adenoma
Adenocarcinoma
Fuchs adenoma (senile hyperplasia of the ciliary body)
Hemangioma
Lymphoid tumor
Hemangiopericytoma
Neurofibroma
Glioneuroma
Astrocytoma
Neurilemoma
Rhabdomyosarcoma
Iridociliary epithelial cysts
Sarcoid nodules
Tubercular granuloma
Intraocular foreign body granuloma

WORKUP

Section 5 of 10  

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

Lab Studies

• Because the most common site of metastasis is the liver, obtain liver enzyme levels in any patient with ciliary body melanoma.
• • The most sensitive hepatic function tests are serum levels of gamma-glutamyl transpeptidase, lactic dehydrogenase, and glutamic-oxaloacetic transaminase.
  • If any of these serum levels are abnormal, ultrasonographic and CT studies of the liver are indicated. Unfortunately, both imaging modalities have low sensitivity for micrometastasis (smaller than 1-2 cm in diameter).

Imaging Studies

• A-scan ultrasound of the eye: In tumors more than 3 mm thick, standardized ultrasonography has a diagnostic accuracy of over 95%, and it is helpful in distinguishing melanomas from ciliary body cysts.
• B-scan ultrasound of the eye: It is especially useful in patients with media opacity and to estimate tumor size. Routinely evaluate patients with unilateral cataracts using ultrasonography to rule out the possibility of a retrolental mass. Ultrasonography also can be used to evaluate extraocular extension. The ciliary body is a difficult ocular region to evaluate with B-scan. Immersion technique and comparison with the contralateral healthy eye often are needed to detect a small mass in the ciliary body. Intraocular melanomas have several distinctive features, as follows:
• • 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 (acoustic hollowing)
• Ultrasound biomicroscopy (UBM) has high resolution for ciliary body abnormalities, including melanomas. It can help differentiate tumors of the ciliary body from those of choroidal origin and help define the anterior border. It also is helpful in assessing angle closure and localized narrowing.
• Fluorescein angiography and indocyanine green angiography are more useful in the diagnosis of choroidal melanomas.
• CT scan of the globe and orbit is more expensive than ultrasound and currently is not as sensitive. It requires intravenous contrast media. CT scan is useful to see extraocular extension and to help differentiate between detachment and a solid tumor.
• MRI of the globe and orbit is more expensive than CT scan and is not as sensitive as ultrasound. Intravenous gadolinium highlights the melanoma in the ciliary body. Pigmented melanomas are seen as high-density images in T1 and low-density images in T2.
• Obtain a chest x-ray in patients with ciliary body melanomas for the possibility of lung metastasis.

Procedures

• Fine-needle biopsy can be used in difficult diagnostic cases, particularly in cases of amelanotic melanomas.
• • In experienced hands, this technique has an accuracy of more than 95% in tumors larger than 3 mm; however, false-negative and false-positive results may be obtained.
  • Its risk of local spread is very small, an advantage over incisional biopsy.
  • The most common complication is intralesional hemorrhage.

Histologic Findings

Histologic evaluation of the tumor after enucleation can confirm the diagnosis and evaluate prognosis. Three distinct cell types are recognized in uveal melanomas, spindle A, spindle B, and epithelioid. Spindle A cells have elongated nuclei and uncommonly have mitotic figures. Spindle B cells have an elongated profile but are slightly larger than spindle A cells and have prominent nucleoli. They are found more commonly than spindle A cells. Epithelioid melanoma cells are highly anaplastic, poorly cohesive, and have considerable morphological variation. They tend to resemble epithelial cells and to contain frequent mitotic figures.

The most commonly used histological classification of uveal melanomas is the modified Callender classification. It divides uveal melanocytic tumors into several groups, as follows: spindle cell nevi, spindle cell melanomas, necrotic melanomas, epithelioid cell melanomas, and mixed cell melanomas. The latter 2 groups 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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Medical Care

Choice of management of ciliary body melanoma remains controversial in many respects. Although enucleation has been the treatment of choice in the past, the results of the COMS show that vision-sparing approaches may offer similar degrees of ocular and metastasis tumor control. Particularly, in many patients at the time of diagnosis, it is clear that posterior uveal melanomas have already spread through micrometastasis.

Although undetected metastatic spread at the time of diagnosis and treatment of ciliary body 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, management of the intraocular melanomas becomes palliative. Systemic chemotherapy is the primary treatment. Many modalities and combinations of chemotherapeutic and immunotherapeutic agents exist; however, 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.

Surgical Care

Multiple modes of treatment are available for ciliary body and other uveal melanomas. In determining an approach, multiple factors need to be considered, such as visual acuity of the affected eye, visual acuity of the contralateral eye, intraocular pressure, ocular structures involved, size of the tumor, age and general health of the patient, 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 mm in elevation can be observed until growth is documented. Sequential measurements of the tumor dimensions with ultrasound scanning are necessary.
• Enucleation is the classic approach to posterior ciliary body melanomas. It often has been the preferred treatment of advanced and complicated tumors, which compromise visual function, and when other therapies have failed. Because of potential release of malignant cells into the bloodstream and orbital soft tissues during the surgical procedure, keep manipulation of the globe to a minimum. Some physicians advocate for preenucleation radiation therapy to hypothetically reduce local and hematogenous dissemination. The theoretical advantage of enucleation over vision-sparing treatments is a decreased risk of metastatic spread. It remains unproven whether it truly improves the patient's prognosis for life.
• External beam irradiation with either protons or helium ions is a frequently used alternative method to treat medium-size tumors ( <10 mm in height and 15 mm in diameter). Radiopaque tantalum rings usually are sutured to the sclera to serve as reference markers for alignment of the radiation beam. By causing irradiation-induced vessel damage, the tumor necroses and regresses. Treatment may be complicated with radiation cataract, dry eye syndrome, radiation retinopathy, and rubeosis iridis. It seems that patients treated with this method have a survival rate comparable to those treated by enucleation. About 15% of eyes ultimately require enucleation, often because of neovascular glaucoma or local recurrence.
• Plaque brachytherapy is a widely accepted alternative to enucleation for medium-size posterior uveal melanomas ( <10 mm in height and 15 mm in diameter). It has similar indications and success rates to external beam irradiation. Plaques containing radioactive isotopes of iodine-125 are attached temporarily to the sclera and limbus underlying the melanoma. Other radioactive agents used in the past include iridium, cobalt, palladium, ruthenium, and other isotopes. A computerized calculation is used to determine the dose and duration of plaque application for a radiation delivery of approximately 40,000 cGy to the base and 8000 cGy to the apex of the tumor. Local recurrence, usually requiring enucleation, occurs at a rate of about 12%. This procedure can cause complications, including radiation retinopathy, but at a reduced rate compared with external beam irradiation.
• Block excision, or sclerouvectomy, is an alternative treatment method for ciliary body melanomas covering less than 4 clock hours of the circumference. Its goal is to salvage the eye, with most of these patients retaining some useful vision. It consists of a full-thickness excision with in-block removal of the ciliary body, cornea, iris, and sclera, with a 2- to 3-mm margin of healthy tissue around the tumor, followed by grafting banked sclera and cornea to close the defect. Retinal detachment, vitreous hemorrhage, and cataract are common complications. These risks are improved by a modified approach, lamellar sclerouvectomy, which uses a scleral flap and minimizes altering the retina and vitreous. In a small proportion of cases (about 15%), local reappearance of the melanoma requires subsequent enucleation.
• Laser photocoagulation and transpupillary thermotherapy are used in selected small choroidal melanomas, but it usually is not useful for ciliary body melanomas.
• Orbital exenteration is a radical treatment reserved for cases with extensive orbital extension. The usefulness of such disfiguring surgery is not established. Patients with such advanced melanomas are likely to have extensive distant metastases and poor prognosis for survival, with or without orbital exenteration surgery.

Consultations

• Oncology
• Radiation oncology

FOLLOW-UP

Section 7 of 10  

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• Follow-up
• Miscellaneous
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Further Outpatient Care

• Irrespective of the treatment modality chosen, patients with ciliary body melanomas need to be monitored carefully for many years. This is particularly true for small ciliary body tumors, when the diagnosis is not clearly established. Close observation and measurement of the tumor dimensions with any of the diagnostic tools mentioned earlier is critical.
• Repeat examinations are usually performed about every 3 months initially, and if no changes are seen, follow-up care is completed every 6 months. If growth of the lesion is detected, consider further treatment.
• Ciliary body 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 the tumor's growth.
• Because of the possibility of intraocular or extraocular tumor recurrence, repeat examinations and imaging tests are performed after all treatment modalities.
• Follow-up care in patients with treated ciliary body melanomas should include thorough physical examinations, liver function tests, and imaging of the 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 predisposition to intraocular melanoma.

Complications

• The tumor or the treatment can cause a variety of ocular complications that severely affect vision, as discussed in Pathophysiology and Treatment.

Prognosis

• Visual prognosis is guarded for melanomas in the ciliary body.
• • Ciliary body melanomas have an overall mortality of about 30-50% within 10 years from initial diagnosis and treatment.
  • Deaths are mostly secondary to distant metastases.
  • Melanomas in the ciliary body have worse prognosis for survival than those in other uveal locations. Possible reasons include delayed diagnosis as the tumor is hidden behind the iris diaphragm and an easier regional path to hematogenous spread, perhaps facilitated by the continuous contractions of the ciliary-iris muscles.
  • An additional factor found to correlate with worse survival prognosis of ciliary body melanomas is their increased vascularization.

MISCELLANEOUS

Section 8 of 10  

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• Miscellaneous
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• References

Medical/Legal Pitfalls

• Three circumstances put ciliary body melanoma in a special category regarding medical legal liability, as follows:
• • Diagnosis of ciliary body melanoma often is delayed because of its insidious development.
  • Ciliary body melanoma is rare, and it tends to be last among the differential diagnoses.
  • Ciliary body melanoma is a disease with a high mortality rate, usually irrespective of the chosen treatment modality.

MULTIMEDIA

Section 9 of 10  

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• References

Media file 1:  Transpupillary photograph of ciliary body melanoma.
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Media file 2:  Transpupillary photograph of ciliary body melanoma.
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Media file 3:  Fundus photograph of a large ciliary body melanoma.
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REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
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• References

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• Rummelt V, Folberg R, Rummelt C, et al. Microcirculation architecture of melanocytic nevi and malignant melanomas of the ciliary body and choroid. A comparative histopathologic and ultrastructural study. Ophthalmology. Apr 1994;101(4):718-27. [Medline].
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Article Last Updated: Jan 10, 2007