Ciliary adenoma

From Felipedia

The owners noticed an increasing red mass behind the iris during the 4 months prior to the examination. The tumor originated from the ciliary body epithelium and extended posteriorly, detaching the retina.

Ciliary adenomas are a rare feline eye disease which arises from the non-pigmented inner layer of the ciliary epithelium and usually causes hyphema (blood in the anterior chamber) or glaucoma.

Iris and ciliary body tumors can be asymptomatic, larger tumors may cause subluxation of the lens, segmental cataract or secondary glaucoma. Adenoma of the nonpigmented ciliary body epithelium is clinically indistinguishable from amelanotic malignant melanoma of the ciliary body or metastatic carcinoma. A failure of the adenoma to block transilumination and high internal reflectivity in the ultrasound examination may be helpful feature to distinguish it from primary melanoma. Primary tumors of the ciliary epithelium have been classified as congenital or acquired. Acquired tumors include pseudoepitheliomatous hyperplasia (Fuchs adenoma) and ciliary body unpigmented, pigmented or mixed epithelial tumors - adenoma and adenocarcinoma, congenital - glioneuroma and medulloepithelioma.

In differential diagnosis amelanotic malignant melanoma, foreign body, metastatic disease, granuloma, epithelial cyst, haemangioma, schwannoma, leiomyoma and tumor of ciliary epithelium should be considered. Adenomas of nonpigmented ciliary epithelium (NPCE) are rare tumors, that may be suspected on clinical examination.

Opticin

Opticin is a recently discovered glycoprotein present predominantly in the vitreous humour. It is synthesised and secreted by the ciliary body epithelium (CBE) from the initiation of CBE development in the embryo, and production continues throughout life. It is theorised that opticin is produced by the non-pigmented CBE throughout adult life. Loss of opticin expression by this tissue is associated with and could contribute towards neoplastic transformation of the ciliary body.

References

1. Cursiefen C, Schlotzer-Schrehardt U, Holbach LM, Nauman GOH: (1999) Adenoma of the nonpigmented ciliary epithelium mimicking a malignant melanoma of the iris. Arch Ophthalmol 117, 113-116.

2. Grossniklaus HE, Zimmerman LE, Kachmer ML (1990) Pleomorphic adenocarcinoma of the ciliary body; immunohistochemical and electron microscopic features. Ophthalmology 97: 763-768.

3. Grossniklaus HE, Lim JI (1994) Adenoma of nonpigmented ciliary epithelium. Retina, 1994, 14, 452-456.

4. Iliff WJ, Green WR (1972) The incidence and histology of Fuchs’ adenoma. Arch Ophthalmol 88: 249-254.

5. Laver NV, Rubinfeld RS, Baum M, Brito PA (1996) Cytomorphology of a mixed pigmented/nonpigmented pleomorphic adenoma of the ciliary body. A diagnostic pitfall in the evaluation of intraocular tumors. Diagn Cytopathol 14: 259-262.

6. Shields JA, Augsburger JJ, Waller PH, Shah HG (1983) Adenoma of the nonpigmented epithelium of the ciliary body. Ophthalmology 90: 1528-1530.

7. Shields JA (19920 Diagnosis and Management of Intraocular Tumors. WB Saunders, Philadelphia 1992.

8. Shields JA, Eagle RC, Shields CL, De Potter P (1996) Acquired neoplasms of the nonpigmented ciliary epithelium (adenoma and adenocarcinoma). Ophthalmology 103:2007-2016.

9. Shields JA, Eagle RC, Shields CL, Singh AD, Torrisi PF (2001) Progressive growth of benign adenoma of the pigment epithelium of the ciliary body. Arch Ophthalmol 119: 1859-1861.

10. Takagi T, Tsuda N, Watanabe F, Takaku I (1987) An epithelioma of the ciliary body. Ophthalmologica 195: 13-20.