• Clinical science

Uveal melanoma

Summary

Uveal melanoma is the most common primary malignant tumor of the eye. It develops from choroidal melanocytes and is therefore often pigmented. The tumor can arise from the choroid, iris, or ciliary body. Symptoms, which depend on the location of the tumor, begin when the tumor extends to the optical axis or if there is concomitant retinal detachment or interference with the function of the ciliary body or pupil. The condition is diagnosed through fundoscopy and ultrasonography. Treatment usually consists of radiation therapy or surgical removal of the tumor in advanced disease. With the exception of iris melanoma, uveal melanoma is typically diagnosed late because of the lack of early symptoms. Approximately half of patients go on to develop distant metastases via hematogenous spread, most commonly to the liver, which are then fatal.

Epidemiology

  • Incidence: 6–10/1,000,000 per year
  • Peak incidence: 50–70 years
  • More common in individuals of Northern European descent and those with light eye and/or skin color
  • Location:

References:[1][2][3]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

References:[4]

Clinical features

References:[5]

Diagnostics

References:[6][5][7]

Differential diagnoses

References:[5]

The differential diagnoses listed here are not exhaustive.

Treatment

  • Observation: for small, asymptomatic tumors
  • Radiation therapy
    • Brachytherapy
      • Iodine-125 (gamma rays)
      • Ruthenium-106 (beta rays)
    • Charged-particle radiation therapy
      • Indicated in circumpapillary lesions (i.e., lesions encircling the optic nerve)
  • Enucleation : in advanced findings

References:[2][8][5]

Complications

References:[5]

We list the most important complications. The selection is not exhaustive.

Prognosis

References:[4]