Differential diagnosis of anterior chamber cysts with ultrasound biomicroscopy: ciliary body medulloepithelioma

Abstract

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 greywhite 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, posttraumatic, 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.

Free-Floating, Pigmented Cysts in the Anterior Chamber Causing Ocular Hypertension

“…There have been at least 10 prior case reports describing free-floating cysts in the AC, the first published over 40 years ago [4,5,6,7,8,9,10,11,12,13]. Nearly all of these case reports describe 1 unilateral solitary free-floating cyst in a single patient.…”

“…Three reports describe surgical removal of the cyst to treat visual disturbance and for diagnostic purposes [11,12,13]. A dislodged, free-floating AC cyst associated with neovascular glaucoma in a 7-year-old boy was removed and diagnosed as medulloepithelioma [11]. Two case reports describe a solitary free-floating AC cyst occurring in a young man, requiring surgical removal to treat visual symptoms [12,13].…”

“…One report describes a 14-year-old boy with 2 free-floating cysts which caused visual disturbance and were successfully treated with YAG laser [10]. Three reports describe surgical removal of the cyst to treat visual disturbance and for diagnostic purposes [11,12,13]. A dislodged, free-floating AC cyst associated with neovascular glaucoma in a 7-year-old boy was removed and diagnosed as medulloepithelioma [11].…”

Intraocular Medulloepithelioma

“…[22][23][24][25][26][27][28][29][30] Limited and conflicting results have been reported for chromogranin, synaptophysin, glial fibrillary acid protein, S100 protein, and HMB-45. 12,[22][23][24][25][26][27][28][29][30] Positive immunohistochemistry for pancytokeratins and cytokeratin (CK)18, with no reactivity for CK7, CK20, and epithelial membrane antigen, has been described and awaits confirmation. 23 Fine-needle aspiration biopsy will probably have a greater role in diagnosing medulloepithelioma in the future.…”

Post‐traumatic inclusion cysts of the iris: a longterm prospective case series

“…1). The general features of these cysts on UBM have been extensively described (Finger et al 1995;Zhou et al 2006). The majority of studies advocate surgical methods over laser treatment and quote fair outcomes (Okun & Mandell 1974;Bruner et al 1981;Naumann & Rummelt 1992).…”