Primary uveal malignant melanoma

Last revised by Tariq Walizai on 18 Jun 2024

Malignant uveal melanomas, also referred to as choroidal melanomas, are the most common primary tumor of the adult eye 3

Malignant melanoma of the uvea is the most common primary intraocular malignancy and is predominantly seen in the White population 5. The incidence of these tumors increases with age, with only 2% of tumors found in patients younger than 20 years of age 6

Clinical presentation depends on the location. When the tumor is located on the iris, it may be apparent to the patient or friends and family as a brown nodule. Choroidal or ciliary body tumors are only identified incidentally on fundoscopy or as a result of retinal detachment or advanced disease.

Uveal melanomas arise from the ciliary body or the choroid. Three histological types are recognized 5

  • mixed melanoma: containing both spindle B cell and epithelioid cells

  • epithelioid melanoma

  • necrotic melanoma

Ultrasound is an excellent modality for examining the globe and can visualize small plaque-like or discoid uveal melanoma with a thickness of less than 3 mm, which are usually difficult to assess on CT or MRI. Features include 4

  • solid low to medium echotexture

  • internal vascularity

  • collar button shape

    • this is the classic shape but not always the case

    • represents tumor having broken through Bruch's membrane

    • tumors may be irregular, lobulated or domed

Other features sometimes present include retinal detachment, posterior scleral bowing, choroidal excavation and vitreous or subretinal seeding. Additionally, larger tumors are often significantly sound attenuating 4.

CT is able to identify most uveal melanomas which appear as elevated, hyperdense sharply marginated lenticular or mushroom-shaped lesions, which enhance with the administration of contrast.

MRI is the modality of choice for assessment of malignant uveal melanomas 5,6:

  • T1/PD

    • uveal melanomas are seen as moderately high signal mass lesions (melanin and hemorrhage are responsible for the high signal)

    • associated exudative retinal detachment is moderately high signal

  • T2

    • moderately low signal mass lesion

    • associated exudative retinal detachment is moderately high signal

    • hemorrhagic subretinal fluid has a variable signal pattern

  • T1 C+ (Gd)

    • tumors enhance

    • fat suppression useful in detecting extraocular extension

Treatment for uveal malignant melanoma depends on tumor size and clinician preference. Options include photocoagulation, transpupillary thermotherapy (TTT), brachytherapy, hadrontherapy 11,12, local excision, or enucleation 5,6.

Poor prognostic factors for systemic disease include 5:

  • older age: >60 years of age

  • larger tumors

  • anterior location within the globe

  • epithelioid cells (i.e. mixed or epithelioid histology)

  • extraocular extension, which usually occurs along the ciliary vessels and nerves, and less commonly along the optic nerve 9

Systemic metastases may be widespread (liver > lung > bone > kidney > brain > breast) 6. They can metastasize late to the liver, with delays of 10 to 15 year from the presentation. 

Overall survival depends on tumor size, extraocular spread, and metastases. Even small (<10 mm diameter, <3 mm thickness) tumors still carry a 10-15% 5-year mortality 6.

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