New insights into the development of infantile intraocular medulloepithelioma.
Am J Ophthalmol
; 158(6): 1275-1296.e1, 2014 Dec.
Article
em En
| MEDLINE
| ID: mdl-25174896
ABSTRACT
PURPOSE:
To define the maturational sequence of 3 infantile intraocular medulloepitheliomas.DESIGN:
Retrospective clinicohistopathologic and immunohistochemical study.METHODS:
Immunoreactivity of paraffin sections for CRX (cone-rod homebox transcription factor) and NeuN (biomarker for neuronal differentiation) were investigated together with other biomarkers, including S100, glial fibrillary acidic protein, epithelial membrane antigen, and various cytokeratins.RESULTS:
Three infants (aged 1, 6, and 8 months) had iris neovascularization, 2 had anterior ciliary body tumors, and 1 a posterior tumor associated with a retinochoroidal coloboma. Each tumor displayed a premedullary monolayer of cuboidal epithelium that was S100(+), NeuN(-), and CRX(-) and that transitioned into a multilaminar medullary epithelium forming neurotubules with adluminal cells that were CRX(+). NeuN first appeared in ablumenal neurotubular cells in 1 tumor and was also discovered among neuroblast-appearing cells in another. The third tumor associated with a coloboma was CRX(-) and NeuN(-).CONCLUSIONS:
A simple premedullary epithelial monolayer appears to be the fundamental source for the tumor and its multilaminar medullary epithelium. CRX(+) and NeuN(+) cells within the multilayered medullary layer approximate expression patterns similar to those found in retinal development and differentiation. Discovery of these biomarkers in the neoplastic ciliary epithelium in a small number of tumors indicates preliminarily that the most anterior layers of the optic cup have a retained retinal and neuroglial differentiation potentiality. The third case was CRX(-) and NeuN(-) and possibly arose from embryonic pigment epithelium at the edge of the retinochoroidal coloboma. These immunohistochemical findings offer histogenetic and potential diagnostic insights.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Neoplasias Uveais
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Corpo Ciliar
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Tumores Neuroectodérmicos Primitivos
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Neoplasias da Retina
Tipo de estudo:
Observational_studies
/
Risk_factors_studies
Limite:
Female
/
Humans
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Infant
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Male
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Newborn
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article