Journal List > J Korean Ophthalmol Soc > v.51(12) > 1008719

J Korean Ophthalmol Soc. 2010 Dec;51(12):1665-1670. Korean.
Published online December 21, 2010.  https://doi.org/10.3341/jkos.2010.51.12.1665
Copyright © 2010 The Korean Ophthalmological Society
A Case of Medulloepithelioma Originating from the Retina
Mijin Kim, MD,1 Ho-Kyung Choung, MD,1,2 Min Joung Lee, MD,1 Young A Kim, MD,3 and Sang In Khwarg, MD1
1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea
2Department of Ophthalmology, Seoul National University Boramae Medical Center, Seoul, Korea
3Department of Pathology, Seoul National University Boramae Medical Center, Seoul, Korea

Address reprint requests to Ho-Kyung Choung, MD. Department of ophthalmology, Seoul National University Hospital, #28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea. Tel: 82-2-2072-2438, Fax: 82-2-741-3187, Email: hokyung@medimail.co.kr
Received January 01, 2010; Accepted October 25, 2010.

Abstract

Purpose

To report a rare case of medulloepithelioma originating from the retina.

Case summary

A nine-year-old boy visited our clinic with conjunctival injection and decreased visual acuity in the left eye, which had developed two month previously. Slit lamp examination revealed a distorted pupil and an anteriorly protruding ciliary body displacing the peripheral iris toward the cornea on the inferonasal side. Funduscopic examination showed total retinal detachment accompanied by an inferior hypervascular mass. The examination of the right eye was unremarkable. On computer tomography imaging, a calcified mass was identified behind the iris of the left globe in the inferomedial aspect, and ultrasound biomicroscopy revealed a medium to high echogenic tumor with an uneven oval cystic cavity in the ciliary body. At the follow-up examination, the size of the mass was increased, so we performed enucleation of the left eye. Pathology demonstrated that the retrolental mass abutting the lens had arisen from the retina. Histological examination revealed that the tumor had originated from the retina and extended into the ciliary body, and most of the tumor was composed of hyaline cartilage with calicification. Tumor cells were identified in the periphery, forming elongated tubules and cord-like structures that were immunohistochemically positive for vimentin, neuron-specific enolase, and CD56 compatible with a teratoid medulloepithelioma. The patient was followed up for eight months without any metastasis in the orbit or elsewhere.

Conclusions

Medulloepithelioma should be considered in the differential diagnosis of pediatric orbital mass accompanied by calcification.

Keywords: Medulloepithelioma; Orbit; Retina; Teratoid

Figures


Figure 1
A.B. Anterior segment photographs at the first visit. Pupil is dragged inferonasally and band keratopathy is observed (A). On slit-lamp view, bulged iris touches the cornea (B). (C) Funduscopic finidng of the intraocular mass. Total retinal detachment is accompanied with inferior hypervascular mass.
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Figure 2
Ultrasound biomicroscopy of mass. The ultrasound biomicroscopy reveals the medium to high echogenic tumor in the retrolental space.
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Figure 3
Preoperative CT and MRI images (A,B). Preoperative CT images; Calcified mass is detected behind of iris in inferomedial aspect of left globe. Left globe looks slightly smaller. (C,D) MRI images; T1-weighted image showing marked enhancement of the ocular mass (C) and T2-weighted image showing 'V' shaped subretinal high attenuation (D).
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Figure 4
Microscopic findings of the specimen. (A) a 1.0 × 0.8 × 0.5 cm sized mass is confined to the posterior chamber of the left orbit. (B) The islands of cartilage with calcification are observed. (C)Tumor cells are present at the periphery of the ciliary body showing elongated tubules and cord-like structures. (D) Tumor cells (arrow head) and teratoid cartilage (asterisk) are located at the inner surface of the retinal pigment epithelium (arrow). (H&E, A: ×10, B: ×40, C: ×100, D: ×100)
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Figure 5
Immunohistochemical stainings show positivity to CD-56, vimentin and S-100. Ki-67 labeling index is 10%.
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References
1. Broughton WL, Zimmerman LE. A clinicopathologic study of 56 cases of intraocular medulloepitheliomas. Am J Ophthalmol 1978;85:407–418.
2. Shields JA, Eagle RC Jr, Shields CL, Potter PD. Congenital neoplasms of the nonpigmented ciliary epithelium. Ophthalmology 1996;103:1998–2006.
3. Steinkuller PG, Font RL. Congenital malignant teratoid neoplasm of the eye and orbit: a case report and review of the literature. Ophthalmology 1997;104:38–42.
4. Kim JH, Park ES, Yang HN. A case of benign nonteratoid medulloepithelioma of the optic nerve head. J Korean Ophthalmol Soc 1999;40:1727–1731.
5. Park YH, Lee HJ, Chung SM, et al. Medulloepithelioma of the ciliary body. J Korean Ophthalmol Soc 1999;40:2942–2947.
6. Zimmerman LE. Verhoeff's "terato-neuroma". A critical reappraisal in light of new observations and current concepts of embryonic tumors. Am J Ophthalmol 1971;72:1039–1057.
7. Sosińska-Mielcarek K, Senkus-Konefka E, Jaskiewicz K, et al. Intraocular malignant teratoid medulloepithelioma in an adult: clinicopathological case report and review of the literature. Acta Ophthalmol Scand 2006;84:259–262.
8. Font RL, Rishi K. Diffuse retinal involvement in malignant nonteratoid medulloepithelioma of ciliary body in an adult. Arch Ophthalmol 2005;123:1136–1138.
9. Chavez M, Mafee MF, Castillo B, et al. Medulloepithelioma of the optic nerve. J Pediatr Ophthalmol Strabismus 2004;41:48–52.
10. Chung EM, Specht CS, Schroeder JW. From the archives of the AFIP: pediatric orbit tumors and tumorlike lesions: neuroepithelial lesions of the ocular globe and optic nerve. Radiographics 2007;27:1159–1186.
11. Zhou M, Xu G, Bojanowski CM, et al. Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy: ciliary body medulloepithelioma. Acta Ophthalmol Scand 2006;84:137–139.
12. Ayres B, Brasil OM, Klejnberg C, et al. Ciliary body medulloepithelioma: clinical, ultrasound biomicroscopic and histopathologic correlation. Clin Experiment Ophthalmol 2006;34:695–698.
13. Al-Salam S, Algawi K, Alashari M. Malignant non-teratoid medulloepithelioma of ciliary body with retinoblastic differentiation: a case report and review of literature. Neuropathology 2008;28:551–556.