Diagnosis/Therapy in Ophthalmology

Differential diagnosis of anteriorchamber cysts with ultrasoundbiomicroscopy: ciliary bodymedulloepithelioma

Min Zhou,1,2 Gezhi Xu,2 Christine M. Bojanowski,1 Yuelian Song,2

Rongjia Chen,2 Xinhuai Sun,2 Weiji Wang2 and Chi-Chao Chan1

1Laboratory of Immunology, National Eye Institute, National Institutes of Health,

Bethesda, Maryland, USA2Department of Ophthalmology, Fudan University Shanghai Eye and ENT Hospital,

Shanghai, China

ABSTRACT.

Purpose: To describe a case with motile cyst in the anterior chamber in the right

eye of a 7-year-old boy.

Methods: The right eye’s visual acuity was 20/50. Intraocular pressure was

59 mmHg. Slit-lamp examination showed prominent rubeosis iridis and a grey-

white mass floating freely in the anterior chamber. Ultrasound biomicroscopy

revealed a cystic mass in the anterior chamber. A diagnostic cyclectomy with

removal of the anterior chamber cyst was performed. Histopathology of the

anterior chamber lesion showed an intact cyst composed of medullary epithelial

cells. Medulloepithelioma with malignant criteria was diagnosed and the eye was

enucleated.

Results: Pathology demonstrated an medulloepithelioma with a few mitotic

figures and nuclear pleomorphisms within the ciliary body. The patient was

followed for 8 months without any metastasis in the orbit or elsewhere.

Conclusion: Intraocular medulloepithelioma is a rare embryonic benign or

malignant neoplasm typically diagnosed in the first decade of life as a ciliary

body mass. A dislodged, free-floating anterior chamber cyst associated with

neovascular glaucoma is typical of medulloepithelioma in children. This unique

presentation should be differentiated from congenital iris epithelial, post-

traumatic, epithelial, parasitic and neoplastic cysts. Ultrasound biomicroscopy

is useful for analysing the structure of the anterior segment mass. Ciliary body

medulloepithelioma is characterized by echogenic mass heterogeneity and an

irregular surface containing multiple cystic cavities. Lack of glial differentiation

may predict a better clinical outcome in primary neuroectodermal brain tumours.

Key words: anterior chamber – ciliary body – cyst – medulloepithelioma – ultrasound

biomicroscopy

Acta Ophthalmol. Scand. 2006: 84: 137–139Copyright # Acta Ophthalmol Scand 2005.

doi: 10.1111/j.1600-0420.2005.00542.x

Case report

A 7-year-old boy developed a motilecyst in the anterior chamber of hisright eye. Visual acuity was 20/50and intraocular pressure (IOP) was59 mm Hg. Slit-lamp examinationshowed prominent rubeosis iridis anda grey-white mass floating freely inthe anterior chamber with positiveTyndall light phenomenon (Fig. 1).The rest of the right eye and MRIof the orbit and head wereunremarkable.

Ultrasound biomicroscopy (UBMP40; Paradigm Medical Industries, Inc.,Salt Lake City, Utah, USA) revealed acystic mass measuring 1205 mm in dia-meter located in the anterior chamber(Fig. 2A). Ultrasound biomicroscopydemonstrated a medium-to-high reflec-tive mass at 360-degrees surroundingthe entire ciliary body. This mass, witha solid region measuring from 414 mmto 1856 mm in thickness, containedmultiple hypoechoic ovoid cysticcavities ranging from 201 mm to1897 mm in diameter (Fig. 2B�F).The anterior chamber angle wascompletely closed.

A diagnostic cyclectomy with removalof the anterior chamber cyst was per-formed. Histopathology of the anteriorchamber lesion showed an intact cyst

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composed of 1–2 cellular layers ofmedullary epithelial cells (Fig. 3A).The ciliary body biopsy disclosedpoorly differentiated neuroepithelialcells and cords that closely resemblethe medullary epithelium. A diagnosisof medulloepithelioma with malignantcriteria (Shields et al. 1996) was madeand the eye was enucleated. Pathologydemonstrated an intraocular tumourwith a few mitotic figures and nuclearpleomorphisms within the ciliarybody. Homer Wright rosettes wereprominent (Fig. 3B). Some neoplasticcells were shown to have spread intothe posterior chamber and anteriorvitreous; however, there was no inva-sion or extension outside the globe.Immunohistochemistry showed posi-tive staining for neuron-specific eno-lase protein (NSE) (Fig. 4A) but wasnegative for glial fibrillary acidicprotein (GFAP) (Fig. 4B). The patientwas followed for 8 months withoutany metastasis in the orbit orelsewhere.

Discussion

Intraocular medulloepithelioma is arare embryonic benign or malignantneoplasm typically diagnosed in thefirst decade of life as a ciliary bodymass (Shields et al. 1996). Clinically,ciliary body medulloepithelioma isusually identified as a fleshy pink lesionand can present as either a pigmentedor non-pigmented mass in the ciliarybody (Andersen 1962). A dislodged,free-floating anterior chamber cystassociated with neovascular glaucomais typical of medulloepithelioma in chil-dren. This unique presentation shouldbe differentiated from congenital irisepithelial, post-traumatic (ocular pene-trating injury) epithelial, parasitic(cysticercus) and neoplastic (medullo-epithelioma or ectopic lacrimalchoristoma) cysts (Shields et al. 1999).An epithelial ingrowth might alsoresult in a floating cyst; however, neo-vascular glaucoma would be unlikely.In general, melanomas and adenomasdo not become free-floating cysts in theanterior chamber. Ultrasound biomi-croscopy is useful for analysing thestructure of the anterior segment mass(Garcia-Feijoo et al. 2005). Ciliarybody medulloepithelioma is character-ized by echogenic mass heterogeneity

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Fig. 1. Slit-lamp biomicroscopy photograph of the right eye showing a greyish-white mobile cyst

(asterisk) in the anterior chamber and prominent rubeosis iridis (arrow). Insert: Higher magnifica-

tion showing the irregular surface of the cyst (asterisk).

(A) (B)

(C) (D)

(E) (F)

Fig. 2. Ultrasound biomicroscopy; caliper measurement resolution is � 5 mm). (A) Cystic mass

(arrow) in the anterior chamber measuring 1205 mm in diameter. Multiple spurs protrude from the

cyst wall (arrow). (B) Transverse section of the tumour in the ciliary body. (C, D) Two temporal

radial sections of the tumour, showing a medium-to-high echogenic tumoral area with multiple

uneven oval cystic cavities (arrows) in the ciliary body. The cysts seem to contribute to the

irregular shape of the tumour surface. (E, F) Two nasal radial sections of the tumour, showing

a medium-to-high echogenic ciliary body with less tumoral volume and fewer cysts (arrow).

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and an irregular surface containingmultiple cystic cavities. Lack of glialdifferentiation (negative GFAP) maypredict a better clinical outcome in pri-mary neuroectodermal brain tumours(Janss et al. 1996).

ReferencesAndersen S (1962): Medulloepithelioma of

the retina. In: Tumours of the eye and

adnexa. Int Ophthalmol Clin 2: 483–506.

Garcia-Feijoo J, Encinas JL, Mendez-

Hernandez C, Ronco IS, Martinez de la

Casa JM & Garcia Sanchez J (2005): Medullo-

epithelioma of the ciliary body: ultra-

sonographic biomicroscopic findings.

J Ultrasound Med 24: 247–250.

Janss AJ, Yachnis AT, Silber JH et al. (1996):

Glial differentiation predicts poor

clinical outcome in primitive neuroectodermal

brain tumours. Ann Neurol 39: 481–489.

Shields JA, Shields CL, Lois N & Mercado G

(1999): Iris cyst in children: classification,

incidence and management. The 1998

Torrence A. Makley Jr Lecture. Br J

Ophthalmol 83: 334–338.

Shields JA, Eagle RC, Shields CL &

Potter PD (1996): Congenital neoplasms of

the non-pigmented ciliary epithelium

(medulloepithelioma). Ophthalmology 103:

1998–2006.

Received on May 13th, 2005.

Accepted on July 5th, 2005.

Correspondence:

Chi-Chao Chan MD

Building 10, Room 10 N103, NIH/NEI

10 Center Drive

Bethesda

Maryland 20892-1857

USA

Tel: þ 1 301 496 0417

Fax: þ 1 301 402 8664

Email: chanc@nei.nih.gov

(A) (B)

Fig. 4. Microphotographs of the medulloepithelioma in the ciliary body. (A) Positive staining

(arrows, black-coloured cells) for neuron-specific enolase protein (NSE). (B) Negative staining for

glial fibrillary acidic protein (GFAP). (Avidin-biotin immunoperoxidase, methyl green for coun-

terstaining of cell nuclei in bluish-green colour; original magnification � 200.)(B)

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(A)

*

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Fig. 3. Microphotographs of the anterior

chamber. (A) Intact cyst (asterisk) composed

of 1–2 cellular layers of medullary epithelia.

(B) Poorly differentiated neuroepithelial cells

with a few mitotic figures and nuclear

pleomorphisms arranged in multilayered

cords and sheets separated by cystic spaces

(asterisks). Homer Wright rosettes (arrows)

were prominent. (Haematoxylin and

eosin; original magnification: A � 200, B

� 100.)

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