Case Study: December 2006

A 40-year-old white-Caucasian male presented to the ophthalmologists with a loss of vision in the left eye. A mass was noted in the eye. The eye was enucleated. There was no history of trauma or eye drop use.

At the cut-up bench, flat, diffuse pigmentation was seen at an episcleral level and a mass was noted arising from the choroid.
Figure 1 macroscopic image of the external aspect of the enucleation.
Figure 2-macro image showing a higher power of the episcleral brown pigment.
Figure 3-macro showing the cut surface of the episcleral pigment (arrowed). CB=ciliary body.
Figure 4-macro cut surface showing a substantial tumour.
Figure 5 and Figure 6-micro low and higher power images respectively of the episcleral brown pigmented lesion.
Figure 7-micro image of the ciliary body
Figure 8-micro image of the iris
Figure 9-micro image of the choroid
Figure 10-micro image of the tumour.

1. What is the intraocular mass?

2. What is the flat brown episcleral pigmentation? What do the iris, ciliary body and choroid show?

3. What is the relationship between the lesion in Q1 and Q2?

Click to show answers

1. Choroidal, uveal type malignant melanoma.

2. The episclera, iris, ciliary body and choroid show a diffuse distribution of heavily pigmented bland melanocytes, indicative of congenital melanocytosis. No skin changes were present in this case, and so this is ocular melanocytosis. Note that the episcleral melanocytes are dendritic.

3. Ocular and oculo-dermal melanocytosis is said to be a risk factor for the development of melanoma in the uvea, orbit, meninges and central nervous system. The uveal melanoma usually arises from the ciliary body and the choroid. Iris melanoma is rare in this setting.

Some ocular oncology centres will follow up these patients for life, given their elevated risk of developing melanoma.

Some references:

Gonder JR, Shields JA, Albert DM, Augsburger JJ, Lavin PT.Uveal malignant melanoma associated with ocular and oculodermal melanocytosis.
Ophthalmology. 1982 Aug;89(8):953-60.

Singh AD, De Potter P, Fijal BA, Shields CL, Shields JA, Elston RC. Lifetime prevalence of uveal melanoma in white patients with oculo(dermal) melanocytosis.
Ophthalmology. 1998 Jan;105(1):195-8.

Gonder JR, Shields JA, Shakin JL, Albert DM. Bilateral ocular melanocytosis with malignant melanoma of the choroid.
Br J Ophthalmol. 1981 Dec;65(12):843-5.

Cu-Unjieng AB, Shields CL, Shields JA, Eagle RC Jr. Iris melanoma in congenital ocular melanocytosis. Cornea 1995; 14: 206-209.