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Year : 2002  |  Volume : 50  |  Issue : 2  |  Page : 138-40

Medulloepithelioma of the ciliary body

Glaucoma Division, Department of Ophthalmology, Oasseem Medical College, King Saud University, P.O. Box 225, Buraidah 81999, Saudi Arabia

Correspondence Address:
A al-Torbak
Glaucoma Division, Department of Ophthalmology, Oasseem Medical College, King Saud University, P.O. Box 225, Buraidah 81999
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

PMID: 12194573

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A rare case of medulloepithelioma of the ciliary body is described. The tumour necessitated enucleation of the eye; histopathological diagnosis was benign nonteratoid medulloepithelioma of the ciliary body

Keywords: Ciliary body tumours, nonteratoid, medulloepithelioma, congenital tumours, diktyoma

How to cite this article:
al-Torbak A, Abboud EB, al-Sharif A, el-Okda MO. Medulloepithelioma of the ciliary body. Indian J Ophthalmol 2002;50:138

How to cite this URL:
al-Torbak A, Abboud EB, al-Sharif A, el-Okda MO. Medulloepithelioma of the ciliary body. Indian J Ophthalmol [serial online] 2002 [cited 2022 Sep 28];50:138. Available from: https://www.ijo.in/text.asp?2002/50/2/138/14804

Medulloepithelioma of the ciliary body is a rare embryonic tumour arising from the primitive medullary epithelium, prior to its differentiation into various derivatives.[1],[2] The tumour usually occurs in the first decade of life, with an average delay of about 2 years between onset of clinical symptoms and the final diagnosis.[1],[2] We report a case of medulloepithelioma of the ciliary body in a young Arab boy.

  Case report Top

A 6-year-old Kuwaiti boy was referred to King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia, for evaluation and management of a ciliary body mass in the right eye, with a clinical diagnosis of melanoma versus medulloepithelioma. Past medical and ocular history were unremarkable.

Ophthalmic examination revealed best corrected visual acuity 6/18 right eye and 6/9 left eye. Intraocular pressure by applanation tonometery was 36 and 16 mmHg in the right and left eye, respectively. Left eye was normal. The right eye had a quiet conjunctiva and clear cornea with iridocorneal touch extending from 6:00 to 11:30 o'clock position. The anterior chamber was of moderate depth with mild inflammatory activity. There was rubeosis iridis and the angle was totally closed for 360ø. Vitreous was clear and retina intact. A grayish lobulated mass arose from the temporal part of the ciliary body, protruded through the pupil, pushed the iris anteriorly, and subluxated the lens with some cataractous changes [Figure - 1].

Systemic examination was normal and complete metastatic work-up was negative. Ultrasonography using 20 MHz probe revealed a well circumscribed, 4.6 mm dome-shaped lesion originating from the temporal ciliary body. The lesion was highly reflective and echogenically had a nonhomogeneous internal structure with small cystic cavities and there was no evidence of extrascleral extension [Figure - 2]. Computed tomographic findings confirmed the intraocular mass, with no evidence of calcification or extension outside the globe or to the optic nerve.

Diagnosis of medulloepithelioma was made based on the clinical findings, ultrasonography, and the age of the patient. After discussing therapeutic options with the parents, enucleation was carried out.

The enucleated right globe measured 25 mm horizontally, 25 mm vertically and 26 mm anterioposteriorly with irregular pupil and transilluminated truly except temporally where ciliary body area was swollen by a grayish lobulated mass. On gross sectioning the mass was mucoidal non-infiltrative and measured 9 mm in the antero-posterior plane and 3 mm in thickness. The mass anteriorly displaced the adjacent iris occluding the trabecular meshwork [Figure - 3] and extended through the pupil to the anterior chamber, touching the cornea. The tumour indented the adjacent edge of the lens and appeared to laterally displace the lens. The anterior chamber angle was occluded by iridocorneal adhesions. Rubeosis iridis was present and the iris appeared elongated and reduced in thickness. Vitreous grossly appeared free of any seeding.

Microscopically the mass was made up of proliferated neuroepithelial plump cells in clusters with frequent primitive tubules as large empty clefts surrounded by peripheral multilayers of columnar cells forming rosette-like structures [Figure - 4]. Much of the bulk consisted of these tumour cells intervened by abundant eosinophilic and myxoid material that stained positive with alcian blue. The cells contained relatively large nuclei with distinct prominent nucleoli and occasional mitotic figures (one per high power field) especially in the region of the ciliary body. Particularly, the solid posterior parts of the lesion stained positively to neuron specific enolase, vimentin and S-100, contrary to the more anterior parts bordering the iris.

The posterior globe showed no tumour involvement, but the retinal ganglion cells in the macula appeared decreased and the lamina cribrosa was bowed posteriorly without significant cupping or atrophy of the optic nerve head.

  Discussion Top

Intraocular medulloepithelioma characteristically is a disease of childhood and usually becomes clinically apparent during the first decade of life at a mean age of 4 years.[1],[2] However, there are documented cases where it first becomes symptomatic in adulthood.[3],[4] The most common features of medulloepithelioma are loss of vision, pain, visible iris or ciliary body cystic mass, and leukocoria.[1],[2] Cysts within the tumour is a well-known clinical feature[1],[2] and one study reported presence of clear cysts in 60% of patients.[5] Iris neovascularisation with neovascular glaucoma is a common finding[1],[6] and has been reported in up to 60% instances.[5] Neovascular glaucoma in a child with normal posterior fundus is highly suggestive of occult ciliary body medulloepithelioma. Diagnosis of intraocular medulloepithelioma is best made by clinical recognition of a ciliary body mass with the aforementioned clinical characteristics.[5] Ultrasonographic examination may be beneficial, as it sometimes demonstrates the cystic nature of the tumour.[2] Radiological investigations (computed tomographic or magnetic resonance imaging scan) usually do not help in establishing the diagnosis, but may visualise extent of the tumour and may be useful for monitoring tumour recurrence.[2],[5] A definitive diagnosis is often established on histological examination.

Broughton and Zimmerman[1] categorized medulloepithomas into teratoid and nonteratoid types, each of which can be further subclassified as benign or malignant. The teratoid variety contains heterologous elements such as brain, cartilage, and skeletal muscle, whereas the nonteratoid type consists of a proliferation of fairly well differentiated cells of the non-pigmented ciliary epithelium. The histologic criteria for malignancy consist of (1) areas of poorly differentiated neuroblastic cells; (2) increased mitotic activity or nuclear pleomorphism; (3) sarcomatous areas; and (4) invasion of other ocular tissue with or without extraocular extension.[1] Based on the above criteria, our case was classified as benign nonteratoid medulloepithelioma of the ciliary body. The predominant neuroepithelial rosette-like structures, cellular morphology, lack of invasiveness and immunohistochemical positivity to neuron specific enolase, vimentin and S-100 confirm the benign nature of the neoplasm.[7]

There is no established treatment for intraocular medulloepithelioma. Smaller anterior tumours can be observed carefully and may be suitable for local resection if they show signs of enlargement.[2],[5],[6] However, there is a high risk of subsequent enucleation after local resection; hence primary enucleation is recommended in most cases.[2],[5],[6]

In summary, medulloepithelioma should be considered in the differential diagnosis of a ciliary body mass especially in a child. The presence of cysts within the tumour and iris neovascularisation are useful clinical findings that suggest the diagnosis of medulloepithelioma. We performed primary enucleation in our patient, which is recommended in most cases.

  References Top

Broughton WL, Zimmerman LE. A clinicopathologic study of 56 cases of intraocular medulloepitheliomas. Am J Ophthalmol 1978:85;407-18.  Back to cited text no. 1
Shields JA. Shields CL. Tumors of the nonpigmented ciliary epithelium. In: Shields JA, Shields CL, eds. Intraocular Tumors. A Text and Atlas. Philadelphia: Saunders. 1996;461-87.  Back to cited text no. 2
Floyd BB, Minckler DS, Valentin L. Intraocular medulloepitheliomas in a 79-year-old man. Ophthalmology 1982;89:1088-94.  Back to cited text no. 3
Husain SE, Husain N, Boniuk M, Font RL. Malignnat non teratoid medullopeithelioma of the ciliary body in an adult. Ophthalmology 1998;105:596-99.  Back to cited text no. 4
Shields JA, Eagle RC Jr, Shields CL, De Potter P. Congenital neoplasms of the nonpigmented ciliary epithelium (medullo-epithelioma). Ophthalmology 1996;103:1998-2006.  Back to cited text no. 5
Canning CR, McCartney AC, Hungerford J. Medulloepithelioma (diktyoma) [review]. Br J Ophthalmol 1988;72:764-67.  Back to cited text no. 6
Kivela T, Tarkkanen A. Recurrent medulloepithelioma of the ciliary body. Ophthalmology 1988;95:1565-75.  Back to cited text no. 7


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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