Choroidal melanoma - External and Internal Eye
Choroidal melanoma is rarely hereditary. A very small increase in risk of ocular melanoma has been described in patients with BRCA2 gene mutations. The most important risk factors include age, light eye color, fair skin, and chronic sun exposure. Other risk factors include ocular melanocytic conditions such as oculodermal melanocytosis (nevus of Ota) and melanosis oculi. Choroidal nevus, a common benign tumor found on the fundus, may rarely transform to melanoma.
Patients often present without any symptoms, and the choroidal melanoma is found incidentally on routine eye examinations. Patients sometimes complain of blurred vision, floaters, photopsia, or visual field defects.
Survival rates are generally poor, particularly if metastasis is found. The Collaborative Ocular Melanoma Study (COMS), a large multicenter randomized controlled trial, showed improved survival with younger age and smaller maximum basal tumor diameter. Five-year rates of death with histopathologically confirmed metastasis were 1%, 10%, and 33% for patients found to have, respectively, small (tumors 4-8 mm in largest basal diameter and 1-2.4 mm in thickness), medium-sized (6-12 mm base diameter and 2.5-8 mm thick), and large (greater than 12 mm in largest basal diameter and 8 mm in thickness) ocular melanomas without metastasis at baseline. Chromosomal 3 monosomy also correlates strongly with metastatic disease and mortality.
C69.30 – Malignant neoplasm of unspecified choroid
255021005 – Malignant melanoma of choroid
- Choroidal nevus
- Choroidal hemangioma
- Metastasis to choroid
- Combined hemartoma of the retina and retinal pigment epithelium (RPE)
- Congenital hypertrophy of the RPE (CHRPE)
- Bilateral diffuse melanocytic proliferation
- Choroidal osteoma
- Metastatic carcinoma
- Hemorrhagic detachment of choroid or RPE (see age-related macular degeneration)