PMC9082137_01
Clinical appearance of medulloepithelioma prior to and following plaque brachytherapy. a Slit-lamp photo of the left eye demonstrating a large whitish ciliary body tumor. b Fundus photo of the left eye demonstrating the large medulloepithelioma temporally with adjacent subretinal fluid involving the macula. c Transverse B scan at 3:00 demonstrating the medulloepithelioma with adjacent exudative retinal detachment (arrowhead). d Scan showing moderate to high internal reflectivity throughout the tumor. e Slit-lamp photo showing stable appearance of the medulloepithelioma 1 year following brachytherapy. f Longitudinal B scan at 3:00 demonstrating interval worsening of the subretinal fluid (asterisk) adjacent to the tumor.
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F1
Slit-lamp exam was remarkable for a temporal cataract and a large adjacent ciliary body tumor (shown in Fig. 1a). The tumor was whitish in appearance and associated with a shallow exudative retinal detachment involving the macula (shown in Fig. 1b), but no vitreous seeding or retinal infiltrative lesions were noted. B scan showed a 13.9 mm (transverse) x 7.8 mm (longitudinal) x 9.4 mm (height) ciliary body tumor with adjacent subretinal fluid and no internal calcifications (shown in Fig. 1c). A scan demonstrated moderate to high internal reflectivity throughout the tumor (shown in Fig. 1d). Over the following year, there was minimal tumor regression (shown in Fig. 1e) to a size of 13.3 mm (transverse) x 7.7 mm (longitudinal) x 8.7 mm (height). While the subretinal fluid was stable initially postoperatively, by 1 year following brachytherapy, the subretinal fluid had increased (shown in Fig. 1f) despite stable tumor dimensions.
a Horizontally sectioned left eye showing temporal white firm tumor of the ciliary body and exudative retinal detachment. b Temporal ciliary body tumor. c Tumor composed mostly of cartilage and fibromyxoid tissue (asterisk) without neuroepithelial elements (x1). d Posterior base of tumor with reactive RPE proliferation (x10).
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F2
There was a white elongated firm tumor in the temporal ciliary body corresponding to the location of the shadow seen on transillumination (shown in Fig. 2a). H&E-stained sections revealed a tumor located in the temporal ciliary body, in the pars plana and extending minimally or overhanging the peripheral retina (shown in Fig. 2b). The tumor was elongated (taller than wide) and was composed only of heteroplastic elements, namely cartilage admixed with small amounts of loose, myxoid stroma, containing few bland spindled and stellate cells (shown in Fig. 2c). Toward the posterior base of this tumor and adjacent to peripheral retina, there was reactive proliferation of pigmented epithelium admixed with small fragments of disorganized neural retina with cystoid change (shown in Fig. 2d).
a Peripheral retina immediately adjacent to tumor showing occluded hyalinized vessels, degenerative changes in retina, photoreceptor atrophy, subretinal exudate, and preretinal fibrous tissue (asterisk) (x10). b Fibrous bands (arrowhead) attached to the disc (x4). c RNFL with cystoid change/edema (x10). d Preretinal fibrocellular membrane (x20).
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F3
Immediately posterior to the tumor base, the peripheral temporal retina was thinner and demonstrated some hyalinization presumably around blood vessels and microcystic changes (shown in Fig. 3a). Fig. 3c). Focal subneural retinal fibrous membranes were seen sometimes admixed with pigmented cells. Preretinal fibrocellular membranes with or without tiny blood vessels were also present within the vitreous cavity (shown in Fig. 3d).