A 40 year old female presents with an enlarging intraocular mass in the ciliary body area. The eye is enucleated.
Figure 1: macro of the lesion
Figure 2-lesion with sclera and choroid interface
Figure 3-lesion with ciliary body/iris/sclera interface
Figure 4-micro correlate of macro Figure 3
Figure 5 Histology of lesional cells
Figure 6-Smooth muscle actin immuno
Figure 7-Desmin immuno
Figure 8-Progesterone receptor immuno
Figure 9-Transmission EM image
Figure 10-Transmission EM image
1. What do the macro images indicate?
2. What does the micro and immuno indicate?
3. What do the transmission EMs show?
1. The macro shows a solid white lesion with some ‘cystic’ change posteriorly. It is in direct contact with the sclera and undermines the choroid and ciliary body. Therefore it is suprauveal and does not arise within the choroid.
2. The micro shows a spindle cell lesion with paranuclear vacuoles, with syncitial eosinophilic cytoplasm. The immuno indicates smooth muscle differentiation with progesterone receptor positivity.
3. The EMs shows typical focal densities of smooth muscle actin in the cytoplasm (Fig 9) and the other figure shows a skenoid fibre (Fig 10).
4. Mesectodermal leiomyoma. These typically occur in women-20-40y in the supra-uveal space and cam mimic melanoma, except that careful inspection of the lesion at fundoscopy shows intact choroidal vessels stretched over the lesion. The ciliary body smooth muscle is predominantly derived from the neural crest (except for the ciliary body artery smooth muscle which is mesoderm-derived). Why the lesion arises in the supra-uveal space is a mystery. It could be that there are ectopic ciliary body muscle fibres that give origin to the lesion. The typical cytology is shown in the figures, with the typical immuno-phenotype. The progesterone receptor status may indicate some hormonal influences. Skenoid fibres are seen in some cases. See References below.
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