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A. Characteristics navigator

  1. Posterior uveal melanoma
  2. Peak incidence in early 60s
  3. White : Black 15:1
  4. 1200 new cases per year in USA (incidence is 6 cases per million)

B. Symptomsnavigator

  1. Usually asymptomatic
  2. Pain occurs if secondary glaucoma is present
  3. Visual loss: field defect; floaters or flashers

C. Examinationnavigator

  1. Conjunctiva - ocular melanocytosis (increased pigment) appears to be a risk factor
  2. Sclera
    1. "Sentinel Vessel" - dilated episcleral vessel in region of tumor
    2. Dark epibulbar mass with tumor / extension
  3. Iris
    1. Tumor extension
    2. Neovascularization
    3. Ciliary body melanoma may erode through iris root
  4. Other
    1. Glaucoma may be secondarily induced
    2. Cataract or lens subluxation if mass pressing lens
    3. Solid uveal mass
  5. Fundus
    1. Lipofuscin - orange pigment on tumor surface
    2. Retinal detachment - hemorrhage, abnormal vessels
    3. Cystoid macular edema, disc swelling
    4. Tumor invasion of retina - erupt through Bruch's membrane in mushroom shape

D. Diagnosisnavigator

  1. Transillumination
  2. Ultrasonography - used to measure height of tumor
  3. CT/MRI - less accurate than ultrasound
  4. Fluorescein Angiography - limited use, mostly in diagnosing lesions simulating melanoma
  5. Fine Needle Aspiration - role uncertain, interpretation difficult, tumor seeding reported

E. Differential Diagnosisnavigator

  1. Choroidal Nevus - usually flatter than true neoplasm
  2. Disciform macular scar
  3. Choroidal hemangioma
  4. Congenital hypertrophy of the RPE
  5. Bilateral Uveal Melanocytic Proliferation [2]
    1. Bilateral progressive cataracts
    2. Iris masses
    3. Choroidal melanocytic proliferation
    4. Overlying retinal detachment
    5. Paraneoplastic syndrome typically associated with abdominal cancer

F. Treatmentnavigator

  1. Enucleation
  2. Radioactive plaque sewn onto eye over tumor
  3. Proton beam irradation
  4. Observation - small tumors, seriously ill patients

G. Metastasisnavigator

  1. Liver is predominant site; also lung and subcutaneous tissue
  2. Median duration to onset of metastases after treatment is ~7 years
  3. Patients with metastatic disease usually survive less than one year
  4. Monitor liver enzymes, chest radiography, generaly physical exam for metastatic disease

References navigator

  1. De Potter P, Shields CL, Eagle RC Jr, et al. 1996. Arch Ophthalmol. 114(5):608 abstract
  2. D'Amico DJ and Dryja T. 2002. NEJM. 346(3):189 (Case Record) abstract