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Congenital malignant ciliary body medullepithelioma in two newborns
Author(s) -
Hadjistilianou T.,
Mittica P.,
Bagaglia S.,
Fruschelli M.,
Menicacci C.,
Fusco F.,
Defrancesco S.,
Borri M.,
Galluzzi P.
Publication year - 2016
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2016.0675
Subject(s) - ciliary body , medicine , retinoblastoma , differential diagnosis , histopathology , ophthalmology , benign tumor , pathology , biology , biochemistry , gene
Purpose Intraocular medulloepithelioma is an extremely rare unilateral intraocular tumor arising from the nonpigmented ciliary epithelium. Medulloepitheliomas may be classified as benign and malignant and as teratoid and nonteratoid tumors. Ciliary body medulloepithelioma is usually manifesting in early childhood, rarely at birth. Differential diagnosis includes in particular unilateral retinoblastoma. Intraocular medulloepithelioma may also occur as masquerade‐syndrome simulating uveitis. Methods We report two new cases of intraocular malignant ciliary body medulloepithelioma in newborns presenting as leucokoria and buphthalmos at birth.. Results Both infants were enucleated and diagnosis was confirmed by histopathology. Conclusions Medulloepithelioma is a rare childhood tumor and should be considered in the differential diagnosis of an intraocular mass in a child. Imaging plays a limited role in differentiating the mass from other solid and cystic ciliary body masses but it is valuable in determining tumor extension and recurrences. Advanced medulloepithelioma at birth with buphthalmos can simulate retinoblastoma and easily be misdiagnosed.