Ocular Melanoma

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Original article contributed by: Grant M. Comer M.D.
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Assigned editor:
Review: Not reviewed

Disease Entity[edit | edit source]

Disease[edit | edit source]

Ocular melanoma (iris, ciliary body, choroid)

Etiology[edit | edit source]


Risk Factors[edit | edit source]

  • Age
  • Caucasian race of Northern European ancestry
  • Choroidal nevus
  • Nevus of Ota

General Pathology[edit | edit source]

Gross pathology[edit | edit source]

The majority of ocular melanomas are oval shaped nodules despite the classically described collar-button shape. Alternatively, melanomas rarely present as a diffuse pattern with minimal height. Pigmentation varies from amelanotic to black and affects intraocular tissues containing melanin such as the choroid, iris, and ciliary body.

Histology[edit | edit source]

The modified Callender system describes cell types as: spindle cell, mixed cell (containing epitheliod), and necrotic. Other findings consistent with melanoma include: large nucleolar size, higher mitotic rate, aneuploidy, vascularity, lymphocyte infiltration, and overlying retinal degeneration.

Pathophysiology[edit | edit source]

Ocular melanoma can cause significant ocular morbidity including: serous retinal detachment, choroidal neovascularization, and melanomalytic glaucoma.

Primary prevention[edit | edit source]

There are no known prevention measures since the basic etiology remains unknown.

Diagnosis[edit | edit source]

History[edit | edit source]

Patients with ocular melanoma are often asymptomatic or report nonspecific symptoms including photopsias or vision loss if the tumor or exudative detachment encroach on the fovea. Most patients are middle aged or older and Caucasian.

Physical examination[edit | edit source]

The examination includes detailed ophthalmoscopy and ultrasound. Fluorescein angiography can be useful in evaluating lesions that simulate ocular melanoma but is not usually diagnostic for melanoma.

Signs[edit | edit source]

The classic description of a choroidal melanoma is a pigmented collar button-shaped tumor often associated with a serous retinal detachment; however, melanomas can vary from amelanotic to dark brown and from fusiform to collar-button. High risk characteristics suggestive of a melanoma include orange lipofuscin pigment, height greater than 3 mm, and subretinal fluid.

Symptoms[edit | edit source]

  • Asymptomatic
  • Photopsia
  • Blurred vision (central or peripheral)

Clinical diagnosis[edit | edit source]

Ocular melanoma is typically diagnosed clinically by appearance on ophthalmoscopy and ultrasound. Biopsy is generally reserved for equivocal cases.

Diagnostic procedures[edit | edit source]

Standardized Echography (Ultrasound )[edit | edit source]

A-scan (time amplitude-mode)[edit | edit source]

8 MHz transducer: A-scan echography provides a one-dimensional assessment of the distance between ocular structures and relative reflectivity within the structures. Tumor height is measured as the distance between the spikes representing the tumor surface and the inner- sclera.

B-scan (brightness mode)[edit | edit source]

10 MHz transducer: B-scan echography is a two-dimensional representation of the posterior segment. This mode is valuable in detecting the tumors and assessing basal measurements.

Characteristics of ocular melanoma using Standardized Echography include: collar button (mushroom) shape, low to medium internal reflectivity, regular internal structure, and internal vascularity. (Ossoinig, 1979)

Other resources for anterior segment assessment[edit | edit source]

  • Ultrasound biomicroscopy (UBM)
  • 20 Mhz B-scan
  • Anterior segment optical coherency tomography

Laboratory test[edit | edit source]

No laboratory tests are helpful in diagnosing ocular melanoma.

Differential diagnosis[edit | edit source]

  • Nevus
  • Choroidal hemangioma
  • Metastatic carcinoma
  • Disciform lesions
  • Choroidal hemorrhage
  • Choroidal osteoma
  • Posterior nodular scleritis
  • Leiomyoma
  • Ampulla of vortex vein
  • Melanocytoma
  • Congenital hypertrophy of the retinal pigment epithelium

Management[edit | edit source]

General treatment[edit | edit source]

Management options include observation, laser photocoagulation, transpupillary thermotherapy, radiotherapy, local resection, or enucleation. 

Medical therapy[edit | edit source]

Chemotherapy and immunotherapy are not used for local control of ocular melanoma.

Medical follow up[edit | edit source]

Patients are followed periodically after treatment with follow-up clinical examinations and ultrasound to ensure appropriate resolution of the tumor.

Surgery[edit | edit source]

Ocular melanoma is generally treated surgically using one of the approaches described in 3.1 with radiotherapy and enucleation used most frequently.

Surgical follow up[edit | edit source]

Specific follow-up depends on the individual clinician; however, most ocular oncologists will follow the patients periodically to monitor for treatment failure, complications, and ensure appropriate metastatic screening.

Complications[edit | edit source]

Complications universal to all treatment modalities include local treatment failure. Treatment specific complications include radiation retinopathy and neuropathy, neovascular glaucoma, cataract, hemorrhage, and diplopia for radiotherapy. Infection and tissue dehiscence are occasionally observed after enucleation.

Prognosis[edit | edit source]

Local treatment rates are high after radiotherapy and enucleation. However, metastatic disease was diagnosed at a 25% and 34% rate at 5-years and 10-years respectively in the Collaborative Ocular Melanoma Study (COMS) with the liver affected 89% of the time. The mortality rate after diagnosis of metastatic disease was 80% at 1 year and 92% at 2 years. (Diener-West, 2005)

References[edit | edit source]

  1. Diener-West M, Reynolds SM, Agugliaro DJ, et al. Development of metastatic disease after enrollment in the COMS trials for treatment of choroidal melanoma: Collaborative Ocular Melanoma Study Group Report No 26. Archives of Ophtalmology. 123(12):1639-43.2005.
  2. Ossoinig, KC. Standardized echography: basic principles, clinical applications, and results. International Ophthalmology Clinics. 19:127-210. 1979.