eMedicine Specialties > Ophthalmology > Iris & Ciliary Body

Melanoma, Ciliary Body: Treatment & Medication

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

Treatment

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

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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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