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Year : 1985  |  Volume : 33  |  Issue : 3  |  Page : 183-186

Medulloepithelioma of the ciliary body

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, A.I.I.M.S. New Delhi, India

Correspondence Address:
A Panda
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, A.I.I.M.S. New Delhi
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Source of Support: None, Conflict of Interest: None

PMID: 3841866

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How to cite this article:
Panda A, Dayal Y, Mohan M. Medulloepithelioma of the ciliary body. Indian J Ophthalmol 1985;33:183-6

How to cite this URL:
Panda A, Dayal Y, Mohan M. Medulloepithelioma of the ciliary body. Indian J Ophthalmol [serial online] 1985 [cited 2023 Dec 10];33:183-6. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1985/33/3/183/30818

Medulloepithelioma of the eye is a very rare embryonal tumor generally arising from the pars ciliaris retinae. Occasionally it may originate in the area of the optic nerve. clinically, it may present as a tumor in the anterior chamber or retinal detachment or glaucoma[1]. This paper is to document four cases of medulloepithelioma diagnosed histo­pathologically within a period of 1971­-1983.

  Material and methods Top

Four histologically proven cases of Medulloepithelioma are the subjects of this study. The detailed history, clinical findings, histopathological findings and follow up are as evident from [Table 1].

  Histopathology Top

Biopsy - The piece removed at biopsy was small and was mainly composed of cells arranged in a tubular and cribriform pattern [Figure - 2]. In view of the clinical data and histological findings, a diagnosis of pleomor­phic adenoma of the lacrimal gland was considered.

Treatment - As the growth was very exten­sive but without involvement of bony struc­tures and the skin, subtotal exenteration of the eyeball was performed.

Histopathology (Exenterated specimen)

Gross -- The exenterated specimen was comprised of an enlarged eyeball alongwith eyelids and periorbital tissues. There was large grey white fleshy tumor with areas of haemorrhage and necrosis in side and outside the eyeball. The normal morphology of eyeball structures was completely lost except for the scleral wall [Figure - 3].

  Microscopic Top

The tumor was seen just underneath the oonjunctiva and the periocular tissue. The choroid was partially detached, the pale eosinophilic exudate with cholesterol cleft was seen in suprachoroidal space. The retina could not be made out. The tumor was occupying the eye ball cavity. The anterior segment structure could not be distinctly identified.

The tumor was highly cellular and showed variegated appearance. In some areas, there was multilayered columnar cells form­ing convolutions with well defined margin and some of them containing melanin granu­les while in some areas there was dense collection of round cells with scanty cyto­plasm without any specific arrangement [Figure - 4]. In other areas single layer of columnar epithelial cells enclosing tubular lamina of varying sizes with an eosinophilic exudate similar to the previous biopsy were seen. Foci of glial cells were also seen in loose and fibrillary background [Figure - 5].

Mitotic figures were seen mostly in the highly cellular areas along with areas of haemorrhage. Similar morphology was seen in the intra-ocular as well as extraocular part of the tumor.

  Discussion Top

Tumors of the pars ciliaries retinae are interesting and rare. Three types of tumors are known to arise from this area (1) Benign epithelioma (2) Medulloepithelioma embryo­nal type (Diktyoma) and (3) Medulloepithe­lioma adult type which is the rarest one.

Generally medulloepithelioma occurs in childhood and is rare beyond the age of 9 years[1]. In our cases, the symptoms started before the age of 4 years in two. Case No. 3 had the problem at the age of 7 years, the disease was diagnosed and the enucleation was performed at the age of 13 years. He had a recurrence after 2 years of enucleation but the cause of recurrence and the cervical lymphadenopathy, remained inconclusive as the patient was lost from follow up. Similarly case No. 4, though had the symptoms at the age of 4 years the disease was detected at the age of 18 years.

The general mode of presentations of this tumor being a mass in the anterior chamber, glaucoma or retinal detachment but certain other mode of presentation have also been reported in the literature in the form of panophthalmitis, perforated eye ball, painful blind eye and progressive deterioration of distant vision[1],[2],[3],[4],[5],[6]. Our cases also had various modes of presentations.

The fourth case was unusual with respect of age, long duration of history and different mode of presentation. The presentation was in confusing nature in such a way that even at biopsy the diagnosis of pleomorphit adenoma was suggested. It was only after exenteration, true nature of the lesion could be recognized.

The tumor may be benign or malignant. but not much comments have been given or its malignant potential. The present cast demonstrated all the criterias of malignancy mentioned by Zimmerman(9) such as areas of poorly differentiated neuroblastic cells, mito­tic figures within the medullary epithelial stuctures and extraocular extension except for sarcomatous change. Slow growth of the tumor over a period of 14 years is unlike a malignant lesion from the beginning. It is likely, as evidenced by the duration of symptoms and history of rapid growth for one year, that it was a benign tumor initially and underwent a malignant transformation later on. It is emphasised that with such confusing clinical presentations, histopatho­logical diagnosis should not be made on the basis of a small biopsy from a part of the tumor only.

  Summary Top

Four cases of medullo-epithelioma of the pars ciliaris retinae were presented. The age incidence, different mode of presenta­tions and the biological behaviour of the tumor were discussed. Clinical documenta­tion of a slow growing tumor for 14 years which grew rapidly during period of one year has been correlated with histopathologic diagnosis of malignant medulloepithelioma of the ciliary body in an 18 year old male[7].

  References Top

Reese, A.B., 1976, Tumors of the eye. Haper & Row Publishers, New York.  Back to cited text no. 1
Soudakoff, P.S., 1936, A M.A. Arch, Ophthal mol. 15: 680.  Back to cited text no. 2
Nag, S.G., Dayal, Y., Sood, N.N. and Goswamy, V., 1976, Ind. J. Ophthalmol. 23 : 21.  Back to cited text no. 3
Mohamed, A. Virji, 1977, Brit J. Ophthalmol. 61 : 229.  Back to cited text no. 4
Sivde A.V., Asha, A.K. and Junnarkar, R.V., 1969, Brit. J. Ophthalmol. 53 : 352.  Back to cited text no. 5
Sayed, B.A., Seghal, K.N. and Desai, V.K. 1962, Brit. J. Ophthalmol. 46: 58.  Back to cited text no. 6
Zimmerman, L.E., 1971. Amer. J. Ophthal­mol. 72: 1039.  Back to cited text no. 7


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]


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