J Korean Ophthalmol Soc.  2010 Dec;51(12):1665-1670.

A Case of Medulloepithelioma Originating from the Retina

• Kim M
• Choung HK
• Lee MJ
• Kim YA
• Khwarg SI

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.

Keyword

Medulloepithelioma; Orbit; Retina; Teratoid

MeSH Terms

Ciliary Body
Cornea
Diagnosis, Differential
Eye
Follow-Up Studies
Humans
Hyaline Cartilage
Iris
Microscopy, Acoustic
Neoplasm Metastasis
Neuroectodermal Tumors, Primitive
Orbit
Phosphopyruvate Hydratase
Pupil
Retina
Retinal Detachment
Vimentin
Visual Acuity
Phosphopyruvate Hydratase
Vimentin

Figure

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  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%.

Reference

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