Characteristics, management, and outcome of ocular medulloepithelioma: systematic review of literature and case report
Abstract
Background: Ocular medulloepithelioma (OM) is a rare ocular malignancy. This is a report of a rare case of medulloepithelioma that was misdiagnosed as retinoblastoma.
Material and methods: A case report and review of published, peer-reviewed, English language literature reporting on ocular medulloepithelioma.
Results: A seven-year-old girl presented with a white mass in the anterior chamber of her left eye. The initial diagnosis was retinoblastoma with anterior chamber invasion, and therefore it was enucleated. Microscopy showed a cellular tumour composed of malignant primitive cells forming sheets, rosettes, and tubular structures. Based on the presence of prominent pleomorphism the tumour was diagnosed as malignant teratoid medulloepithelioma. At last date of follow-up three years after enucleation, the patient was alive without metastasis. A systematic review of literature, analysed 177 cases of OM. The tumour was localised in the ciliary body in 134 (92%) cases, and 26 (23%) cases had extraocular extension. Primary management was enucleation in 84 (55%) cases, tumour resection in 32 (21%) cases, and radiation therapy in 20 (13%) cases. Histopathology disclosed benign features in 36 (22%) eyes, malignant features in 124 (78%) eyes, teratoid features in 72 (59%), and non-teratoid features in 51 (41%) eyes. At a mean follow-up of five years, 14 (8%) patients had metastasis, and six (6%) patients were dead.
Conclusions: Ocular medulloepithelioma most commonly affects children. The most common type is the malignant
teratoid type, but it has a favourable prognosis, and distant metastasis and mortality are relatively rare, at 8%
and 6%, respectively.
Keywords: ciliary bodyirismedulloepitheliomaretinoblastomatumour
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