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Case Study: March 2009
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
Questions
1. What do the macro images indicate?
2. What does the micro and immuno indicate?
3. What do the transmission EMs show?
4. Diagnosis?
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.
References
1. Blodi FC. Leimyoma of the ciliary body. Am J Ophthamol 1950; 33:939-42.
2. Shields JA, Shields CL, Eagle RC, De Potter P. Observations on seven cases of intraocular leiomyoma. The 1993 Byron Demorest Lecture. Arch Ophthalmol 1994; 112:521-28.
3. Biswas J, Krishna Kumar S, Gopal L, Bhende MP. Leiomyoma of the ciliary body extending to the anterior chamber: clinicopathologic and ultrasound biomicroscopic correlation. Surv Ophthalmol 2000; 44:336-42.
4. Schlotzer-Schrehardt U, Junemann A, Naumann G.O.H. Mitochondria-rich epithelioid leiomyoma of the ciliary body. Arch Ophthalmol 2002; 120:77-82.
5. Park S.H, Lee J, Chae Y.S, Kim C.H. Recurrent mesectodermal leiomyoma of the ciliary body: a case report. J Korean Med Sci 2003; 18:614-7.
6. Jeon Y.K, Cha H.J, Kim N.R, Kim C.J, Chi J.G. Leiomyoma in the posterior choroid: a case report. J Korean Med Sci 2002; 17: 429-33.
7. Jakobiec F.A, Font R.L, Tso M.O, Zimmerman L.E. Mesectodermal leiomyoma of the ciliary body: a tumor of presumed neural crest origin. Cancer 1977; 39: 2102-13.
8. Jakobiec FA, Witschel H, Zimmerman L.E. Choroidal leiomyoma of vascular origin. Am J Ophthalmol 1976; 82: 205-212.
9. Shields CL, Shields JA, Varenhorst MP. Transcleral leiomyoma. Ophthalmology 1991; 98(1): 84-97.
10. Park SW, Kim HJ, Chin HS, Tae KS, Han JY. Mesectodermal leiomyoma of the ciliary body. Am J Neuroradiology 2003; 24: 1765-1768.
11. Campbell RJ, Min KW, Bolling JP. Skenoid fibres in mesectodermal leiomyoma of the ciliary body. Ultrastruct Pathol 1997; 21(6): 559-567.
12. Lai CT, Tai MC, Liang CM, Lee HS. Unusual uveal tract tumour: mesectodermal leiomyoma of the ciliary body. Pathol Int 2004; 54(5): 337-342.
13. Meyer SL, Fine BS, Font RL, Zimmerman LE. Leiomyoma of the ciliary body. Am J Ophthalmol 1968; 66: 1061-1068.
14. Croxatto JO, Malbran E. Unusual ciliary body tumour: mesectodermal leiomyoma. Ophthalmology 1982; 89: 1208-1212.
