TL;DR: Intraocular medulloepithelioma is a nonhereditary neoplasm of childhood arising from primitive medullary epithelium that appears as a grey-white ciliary body lesion with intratumoral cysts and Histopathology shows primitive neuroepithelial cells arranged as cords closely resembling the primitive retina.
Abstract: Intraocular medulloepithelioma is a nonhereditary neoplasm of childhood arising from primitive medullary epithelium. It most often involves the ciliary body. Most patients present between 2 and 10 years of age with loss of vision, pain, leucocoria, or conjunctival congestion. The mass appears as a grey-white ciliary body lesion with intratumoral cysts. Presence of a neoplastic cyclitic membrane with extension to retrolental region is characteristic. Secondary manifestations like cataract and neovascular glaucoma may be present in up to 50% and 60% patients, respectively. These could be the first signs for which, unfortunately, about 50% patients undergo surgery before recognition of the hidden tumor. Systemic correlation with pleuropulmonary blastoma (DICER1 gene) has been documented in 5% cases. Histopathology shows primitive neuroepithelial cells arranged as cords closely resembling the primitive retina. Histopathologically, the tumor is classified as teratoid (containing heteroplastic elements) and nonteratoid (containing medullary epithelial elements), each of which are further subclassified as benign or malignant. Retinoblastoma-like and sarcoma-like areas may be seen within the tissue. The treatment modality depends on tumor size and extent of invasion. For small localized tumors (≤3-4 clock hours), conservative treatments with cryotherapy, plaque radiotherapy, or partial lamellar sclerouvectomy (PLSU) have been used. Plaque brachytherapy is generally preferred for best tumor control. Advanced and extensive tumors require enucleation. Rare use of intra-arterial and intravitreal chemotherapy has been employed. Systemic prognosis is favorable, but those with extraocular extension and orbital involvement show risk for local recurrence and metastatic disease, which can lead to death.
TL;DR: In 1908 Fuchs, in his paper entitled "Proliferations and Tumors of the Epithelium of the Ciliary Body," described four instances collected from the literature of a tumor which he called diktyoma.
Abstract: In 1908 Fuchs, 1 in his paper entitled "Proliferations and Tumors of the Epithelium of the Ciliary Body," described four instances collected from the literature of a tumor which he called diktyoma. Since that time several additional cases have been reported by various authors, but still the total number does not exceed eight. This case is reported because of the great rarity of the disease and the advanced stage of involvement of the eyeball. REPORT OF CASE W. Y. H., a Chinese farmer aged 28, came to the St. Michel Hospital in Peiping on Dec. 11, 1934, complaining of loss of vision of the left eye. The patient was referred to the ophthalmologic clinic of the Peiping Union Medical College for consultation. There he gave the following history: Ten months prior to consultation the vision of the left eye began to fail, and in the course of two months it
TL;DR: A 34-month-old girl treated for anterior uveitis, a superiorly subluxated cataract, rubeosis iridis and secondary glaucoma of the right eye of 3 months duration was diagnosed as having medulloepithelioma.
TL;DR: A 22-month-old girl was found to have a pigmented mass of the ciliary body of the right eye and five months later the globe was enucleated because of enlargement of the tumor and the appearance of brown pigment on the adjacent epibulbar surface.
TL;DR: The patient was a girl, aged 16, whose parents had observed a new growth in the right eye since birth to have increased slowly and steadily, and who entered the Eye Department of Rigshospitalet, Copenhagen in August 1947.
Abstract: The patient was a girl, aged 16. From birth her parents had observed a new growth in the right eye. It is stated to have increased slowly and steadily. During the last 4 years the patient has been examined every 6 months by Dr. A . Nordsted, ophthalmologist of Aalborg. During this period the size of the tumour and the vision remained unchanged. Apart from the reduced vision the patient has exhibited no symptoms, particularly not of earlier inflammation or glaucoma. In August 1947 the patient entered the Eye Department of Rigshospitalet, Copenhagen (387/47). O b i . exam. revealed completely normal conditions except for the right eye. Vision of right eye 6/60, not improved with glass. Tension of right eye %-1O/X = 27 mm., The right eye is pale, the cornea natural except for a narrow marginal rim of corneal degeneration at 2-3 o'clock. Corresponding to the iris in the area at 12-5 o'clock there is a tumour consisting of an upper reddish lobe and a lower lobe with a slightly brownish pigment (see Fig. 1). The surface of the tumour is somewhat irregular, slightly papillomatous and richly vascularized. On the nasal side the tumour reaches as far as the posterior