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Intraocular Medulloepithelioma in a 15-Year-Old: A Rare Presentation

7 July 2017


BACKGROUND: A 15-year old male presented with decrease in vision for the past 2 years. He was referred for a potential tumor due to an obstructed view of the right eye posterior segment. The patient was in good systemic health with no current medications.

FINDINGS: Best-corrected visual acuities were 20/63 OD and 20/16 OS through -4.00 DS OD and -4.25 DS OS. A dull fundus reflex was noted OD. Pupils were normal with no RAPD and biomicroscopy showed no cells or flare. IOP was 11 mm Hg OD and 12 mm Hg OS. The nasal quadrant of the right eye pupil failed to dilate completely. Fundus examination OD revealed diffuse vitreous haze and a large exudative retinal detachment due to a choroidal lesion spanning from 1 to 5 o’clock, sparing the macula.

ASSESSMENT: B-scan ultrasound showed a circumferential retinochoroidal mass with no calcification or excavation of underlying ocular structures. The dimensions of the tumor were 21.7mm ✕ 21.2mm ✕ 12.5mm. Ultrasound biomicroscopy showed anterior iris displacement from 3 to 5 o’clock. A large homogeneous mass containing multiple cystic spaces was noted. There was the presence of a lental apposition to the mass, retrolental cyclytic membrane. This atypical presentation led to the finding of a ciliary body tumor supplied by sentinel vessels extending to the retina causing exudative retinal detachment. The patient was referred for chest CT to rule out pleuropulmonary blastoma. MRI results showed hypointense signal in T2 weighted imaging with respect to the vitreous. Based on the findings we suspected this case to be an amelanotic melanoma or medulloepithelioma.

MANAGEMENT: The patient was referred to an ocular oncologist for fine needle aspiration biopsy under general anesthesia. Histopathology reports confirmed the presence of intraocular medulloepithelioma. Since the tumor base was too large for globe salvage and too thick for radioactive plaque, enucleation was performed and a vicryl mesh wrapped orbital ball implant was inserted.


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