|Year : 1973 | Volume
| Issue : 4 | Page : 195-199
Medulloepithelioma (adult type)
Asha V Kher1, AV Shivde1, KD Sharma1, RN Gandhewar2
1 Department of Pathology, Medical College and Hospital, Nagpur, India
2 Department of Ophthalmology, Medical College and Hospital, Nagpur, India
Asha V Kher
Department of Pathology, Medical College and Hospital, Nagpur
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kher AV, Shivde A V, Sharma K D, Gandhewar R N. Medulloepithelioma (adult type). Indian J Ophthalmol 1973;21:195-9
|How to cite this URL:|
Kher AV, Shivde A V, Sharma K D, Gandhewar R N. Medulloepithelioma (adult type). Indian J Ophthalmol [serial online] 1973 [cited 2019 Aug 24];21:195-9. Available from: http://www.ijo.in/text.asp?1973/21/4/195/34629
Tumours of the pars-ciliaris retinae are interesting and rare. Three types of tumours are known to arise from this area- (1) Benign epithelioma (Fuchs  ), (2) medulloepithelioma, embryonal type (Diktyoma) and (3) medulloepithelioma (adult type). Out of these the adult type is rarest. Very few cases have been reported so far in the world literature-Reese.  The classification and histogenesis of the tumours of the pars-ciliaris retinae have been discussed by many authors in the past and recently by WOLTER and PFISTER  and ZIMMERMAN. 
A case of medulloepithelioma in a thirty-five year old male is being presented because of its rarity. This is the third case of this kind from this hospital during the period 1952-1972 and the first of the adult type. The earlier cases from this institute were reported by KESHWACHAR AND JUNNARKAR  and SHIVDE et al. 
| Case Report|| |
A thirty-five year old male was admitted in the Plastic Surgery department, Medical College Hospital, Nagpur on 27th January, 1972 with pain and swelling and total loss of vision in the right eye of one month's duration. The patient had noticed a corneal opacity in the right eye one year ago and later on noticed nodular swelling over the conjunctiva, gradually increasing in size. This was accompanied by progressive diminution of vision leading to total blindness of one month's duration. He also complained of intermittent bleeding from the growth. The left eye was normal. There was no history of injury to the eyeball in the past.
Examination: The right eye was totally blind and showed nodular masses, reddish white in colour protruding through the right palpebral fissure. The tumour masses moved with the eyeball, were firm and not tender. The cornea was pushed up under the upper eyelid. The upper and lower palpebral conjunctivae were free from the growth. There was no lymphadenopathy. Systemic examination did not reveal any abnormality. Haemogram and X-ray chest were normal. Enucleation of the eye was done and the specimen was sent for histopathological examination with a provisional diagnosis of malignant intraocular tumour.
Gross examination of the specimen showed an enucleated eyeball infiltrated by irregular and nodular tumour mass. It was 4 cms. in the sagittal, 3 cms. in the coronal and 3 cms. in the vertical diameter. The cornea was opaque and the conjunctiva was invaded by nodular tumour tissue. On bisection it was found to be situated mainly in the anterior chamber replacing its normal structures. The greyish opaque tumour tissue was extending to the posterior chamber along the posterior wall causing retinal detachment. The sclera of the posterior half was intact [Figure - 1].
Microscopical examination: Sagittal sections of the eyeball along the growth were taken and stained with haematoxylin and eosin and Masson's trichrome stains. The tumour tissue was occupying the anterior half of the eyeball causing polypoidal protrusion and ulceration of the conjunctival epithelium and infiltration of periorbital tissue. It also extended to the posterior chamber closely along the posterior wall causing folding and detachment of retina. The tumour tissue was mainly composed of bands of columnar cells with prominent oval nuclei. At places the cells formed incomplete vesicles or tubules which were limited by a distinct basement membrane. The tubules were lined by single layer of columnar epithelium. In a few sections there were round cells with scanty cytoplasm arranged in sheets showing attempt at rosette formation and resembling retinoblastoma. Groups of neuroglial cells were also seen in most of the sections. The connective tissue stroma as demonstrated by Masson's trichrome stain was very, scanty., Dispersion of pigment was seen mostly in the tumour tissue around the choroid. The pigment was coarsely granular and intracellular in nature. None of the sections studied showed presence of cartilage, bone or muscle fibres [Figure - 2],[Figure - 3].
With the above histological and clinical findings the case was diagnosed as medulloepithelioma-adult type.
Six months after the enucleation, a mass was noticed in the right orbit. The patient was referred to this institute for local recurrence in October, 1972. Local examination revealed a mass 5 x 5 cm. occupying the orbital cavity. It was firm and greyish white in colour, upper cervical lymph nodes were enlarged and hard [Figure - 4]. The tumour mass from the orbital region was sent for histopathological diagnosis.
Gross examination of the specimen showed an irregular nodular mass attached to the eyelids. The mass was 6.5 cm. x 5 cm, oval, solid and firm. Cut section was homogenous, opaque and white [Figure - 5].
Microscopically the slides showed groups and sheets of cuboidal and low columnar cells separated by marked amount of fibramyxomatous stroma. At places the groups of cells were lining the cleft like spaces in the stroma. A few sections showed definite glandular pattern [Figure - 6].
| Discussion|| |
Adult type of medulloepithelioma is very rare. REESE  has reviewed cases reported by elevan authors. Although malignant ciliary tumours arise in older subjects the age range given by various authors extends from 10 to 73 years. . In the present case the age of the patient was thirty-five years. No definite sex incidence has been observed by any of the authors.
Adult type of medulloepithelioma tends to arise in chronically infected eyes and sometimes in subjects whose eyes have been blind and atrophic since childhood. ,,,, Cases have also been reported to have developed spontaneously in normal eyes.,, In the present case, indication of previous inflammatory eye lesion was suggested by the presence of corneal opacity.
Histologically, the tumour consists of proliferating cuboidal or columnar elements of differentiated type arranged in tubular, alveolar and lace-like patterns. The tumour invariably contains pigment as both layers of ciliary body are involved. In the present case columnar cells showing tubular and alveolar pattern with presence of pigment were conspicuous in primary presentation. In contrast, marked amount of stromal reaction consisting of broad bands of fibromyxomatous tissue separating sheets of cuboidal and low columnar epithelial cells characterised the recurrence. Alveolar pattern was visible at a few places.
GRINKER  first used the term medulloepithelioma for this type of malignant tumour. ANDERSEN  stated that adult and embryonic types of medulloepithelioma arising from ciliary epithelium are merely different stages of development of the same tumour. If the tumour develops from cells in the stage of differentiation corresponding to embryonic retina prior to the 6th foetal week the result is diktyoma, whereas if the tumour develops from cells at a later stage the result is a growth simulating adult type. The adult type of ciliary epithelial tumour is associated with rather benign clinical course. Local recurrence has been reported by WOLTER AND PFISTER.  However, tumour metastasis has not been reported in the literature. The cause of cervical lymphadenopathy in the present case remained inconclusive as consent for biopsy of the cervical lymph node could not be obtained.
| Summary|| |
A case of adult type of medulloepithelioma with recurrence in a thirty-five year old male has been reported. The age incidence, predisposing factors, histogenesis and biological behaviour are discussed.
| Acknowledgements|| |
We are grateful to the Dean, Medical College and Hospital, Nagpur for facilities to study this case. We are also indebted to Dr. C. S. Vaidya, Department of Plastic Surgery, Medical College and Hospital, Nagpur for clinical data.
| References|| |
Andersen, S. R.: Medulloepitheliomas, diktyoma and malignant epithelioma of the ciliary body. Acta. Ophthal. 26: 313-330, 1948.
Barrow, R. H. B. and Stallard, H. B.: A case of primary melanocarcinoma of ciliary body. Brit. J. Ophthal. 16: 98-102, 1932.
Butler, E. H. and Assinder, E. W.: A case of endothelioma of the ciliary body. Brit. J. Ophthal. 7: 549-51, 1923.
Collins, E. T.: A case of primary tumour of ciliary body of glandular structure. Tr. Ophtha. Soc. U.K. 14: 83-86, 1894.
Fuchs, E.: Wucher-Ungen and Geschwulstedes Ciliarepethels Arch. Ophtha. (Von Graefes), 68: 534-587, 1908.
Grinker, R. R.: Gliomas of the retina. Arch. Ophth. 5: 920, 1931.
Hoffman, P. and Matulay, K.: Ganglioneuroma of the third ventricle. Bratisi. Lekar Listy. 13: 174-180, 1933.
Keshavachar, K. R. and Junnarkar, R. V.: Diktyoma. Brit. J. Ophthal. 44: 693-695, 1960.
Maertens, M.: Eine Primare, bosartigs, epitheliale geschwalst des augeninnerr beim erwachsenen. Arch. of Augenh. 89: 1-22, 1921.
Meller, J.: Ueber eine epitheliale geschwulst des ciliarkorpers. Arch. Optha. (Von Graefes), 85(2): 191203, 1913.
Merkel, F.: Primates karcinom des ziliarkorpes mit melanotischer pigmentirung. Arch. Augen. 97: 308-313, 1926.
Reese, A. B.: In "Tumours of the eye"; 2nd Edn. New York, Harper and Row, 1963.
Sayed, B. A.; Sahgal, K. N. and Desai, V. K.: Brit. J. Ophthal. 46: 58-60, 1962.
Schlipp, R.: Ueber einen epithelialen tumor des ciliarkorpers. Arch. Ophth. (Von Graefes) 48: 351-361, 1936.
Shivde, A. V.; Kher, A. and Junnarkar, R. V.: Diktyoma. Brit. J. Ophthal. 53: 352, 1969.
Wolter, J. R. and James, B. R.: Adult type of medullopithelioma of the ciliary body. Am. J. Ophthal. 46: 19-24, 1958.
Wolter, J. R. and Pfister, R. R.: Tumours of the pars ciliaris retinae. Am. J. Ophthal. 52: 659-671, 1961.
Zimmerman, L. E.: Verhoeff's teratoneuroma. Am. J. Ophthal. 72: 1039-1057, 1971.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]