eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Melanoma, Ciliary Body

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 in Private Practice, Charles A Garcia, MD, PA; Christopher M Bardorf, MD, MS, Ophthalmology, Children's Eye Physicians, Denver, CO; Dean Eliott, MD, Associate Professor, Department of Ophthalmology, Division of Vitreoretinal Surgery, Kresge Eye Institute, Wayne State University
Contributor Information and Disclosures

Updated: Jan 10, 2007

Introduction

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

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.

More on Melanoma, Ciliary Body

Overview: Melanoma, Ciliary Body
Differential Diagnoses & Workup: Melanoma, Ciliary Body
Treatment & Medication: Melanoma, Ciliary Body
Follow-up: Melanoma, Ciliary Body
Multimedia: Melanoma, Ciliary Body
References
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Further Reading

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Keywords

ciliary body melanoma, ciliary body malignant melanoma, uveal melanoma, intraocular tumor, intraocular melanoma, uvea, iris, ciliary body, choroid

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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 in Private Practice, 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.

Christopher M Bardorf, MD, MS, Ophthalmology, Children's Eye Physicians, Denver, CO
Christopher M Bardorf, MD, MS is a member of the following medical societies: American Medical Association
Disclosure: Nothing to disclose.

Dean Eliott, MD, Associate Professor, Department of Ophthalmology, Division of Vitreoretinal Surgery, Kresge Eye Institute, Wayne State University
Dean Eliott, MD is a member of the following medical societies: American Academy of Ophthalmology and American Medical Association
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
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

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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