Congenital Intraocular Teratoma

Abstract

The clinical and histopathologic findings of an intraocularly located congenital teratoma are reported. In addition to routine histologic stainings, the tissue types present and the fate of the neuroectodermal elements of the disorganized eye were analyzed by immunohistochemistry. An otherwise healthy baby girl was born with a large greenish mass replacing the left eye without invading the orbit. The enucleated eye showed a firm polycystic intraocular tumor filling the intraocular space. Normal sclera and a massively distended cornea enclosed the globe. Fairly normal choroid and a disorganized ciliary muscle were present, but the ciliary body and iris had not formed. The tumor was surrounded by maldeveloped remnants of the optic vesicle and consisted of derivatives of all three germinal layers such as adnexal glands, brain, choroid plexi, intestinal and respiratory epithelium, cartilage, adipose tissue, as well as smooth and skeletal muscle. The clinical history with presentation at birth, female sex of the patient, and both macroscopic and microscopic findings are typical of a benign orbital teratoma, but the intraocular location is unique. Lack of truly medulloepitheliomatous elements in this case and the absence of derivatives from three germinal layers in all previously reported teratoid medulloepitheliomas of the ciliary body exclude the latter diagnosis. The teratoma may have arisen in the orbit with subsequent entrapment within the developing eye when the embryonic fissure closed.