J Korean Ophthalmol Soc.  1999 Oct;40(10):2942-2947.

Medulloepithelioma of the Ciliary Body

Affiliations
  • 1Department of Ophthalmology, College of Medicine, The Catholic University of Korea, St.Mary`s.
  • 2Department of Clinical Pathology, College of Medicine, The Catholic University of Korea.

Abstract

Medulloepithelioma is a rare primary congenital intraocular tumor that usually arises from the primitive medullary epithelium that is destined to form the nonpigmented ciliary epithelium of the ciliary body, but medul-loepitheliomas of retina and optic nerve also occur. We found a dark brownish mass protruding toward the iris and cataract in a 11-year-old girl who had developed gradual loss of vision in her right eye. So we did a simple ciliary body mass excision through the full-thick-ness scleral window and then a partial vitrectomy, cryotheraphy and lens aspiration. Microscopically, the ciliary body mass was outlined by distended neuroepithelial tubles and cords that resembled the primitive medullary epithelium. Stains for mucopolysaccharides disclosed abundant pools of hydronidase-sensitive mucopolysaccharides [hyaluronic acid] around the neuroepithelial tubles. So we histologically diagnosed medulloepithelioma of ciliary body. Although medulloepitheliomas are generally slow growing and relatively benign with regard to survival when they are localized to the eye, they appear to be relentlessly progressive and potentially lethal once extrascleral extention to the orbit occurs. We carefully followed up an 11-year-old girl from whom medulloepithelioma had been completely removed.

Keyword

Medulloepithelioma; Primitive medullary epithelium

MeSH Terms

Cataract
Child
Ciliary Body*
Coloring Agents
Epithelium
Female
Glycosaminoglycans
Humans
Iris
Neuroectodermal Tumors, Primitive*
Optic Nerve
Orbit
Retina
Vitrectomy
Coloring Agents
Glycosaminoglycans
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