On the Classification and Grading of Medulloepithelioma of the Eye

Abstract

Medulloepithelioma is a rare congenital tumor of the ciliary body and iris. The current classification and grading of medulloepithelioma shows inconsistencies and does not reflect clinical behavior. This position paper discusses the backgrounds of the current classification and intends to initiate a discussion on an alternative classification and grading scheme.

Adult ocular medulloepithelioma diagnosed by transscleral fine needle aspiration: A case report

“…In the series of Faulkner-Jones et al the positive predictive value of cytologic diagnosis increased using immunohistochemistry from 93% to 96% and specificity from 67% to 83%. 5 It is important to notice that PAX 5 was also negative, while pulmonary neuroendocrine small cell carcinomas used to show positive nuclear staining in majority of cases. ME was reported to be positive for NSE, Synaptophysin and focally for S100 protein.…”

“…Retrospectively, going against the original histological diagnosis of lung tumor, we cannot exclude whether the lung tumor could be in fact a metastasis of the medulloepithelioma, and could be considered as malignancy. 5 It is important to notice that PAX 5 was also negative, while pulmonary neuroendocrine small cell carcinomas used to show positive nuclear staining in majority of cases. This may also speak against pulmonary origin.…”

“…In a review, only 15 adult cases were identified, and the rarity of such setting may be a source of clinical errors making ocular ME probably underestimated and misdiagnosed as malignant melanoma. 5 This tumor often displays multiple lines of differentiation, including neural, glial, teratoid, and mesenchymal elements with distinctive tubular or trabecular arrangements of neoplastic neuroepithelium that is unique. 4 Recently, ocular ME has been classified into three grades according to histological feature and potential of metastasis, but all grades from benign to malignant are supposed to be a different stage of ME's growth.…”

“…4 Recently, ocular ME has been classified into three grades according to histological feature and potential of metastasis, but all grades from benign to malignant are supposed to be a different stage of ME's growth. 5 This tumor often displays multiple lines of differentiation, including neural, glial, teratoid, and mesenchymal elements with distinctive tubular or trabecular arrangements of neoplastic neuroepithelium that is unique. 6 Transscleral FNA has already proved its effectiveness for diagnosing atypical intraocular tumors with uncertain presentation in many centers.…”

Strabismus Revealing Ciliary Body Medulloepithelioma: A Case Report and Literature Review

“…Later Fuchs in 1908 renamed it diktyoma [7]. The name of medulloepithelioma was proposed by Grinker in 1931 [8] considering the histological resemblance of the tumor to the neuroepithelium of the embryonic neural tube [9]. This was based on the work on a rarer form of medulloeptihelioma of the brain described by Bailey and Cushing [10,11].…”