PMC9175007_01
Presentation of the initial tumor. (A) The image of the posterior pole illustrates a melanotic tumor with subretinal fluid. (B) The B-scan ultrasound reveals a multilobulated tumor with a size of 13.6 x 10.8 mm and a thickness of 6.0 mm and with surrounding subretinal fluid.
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Fundoscopy of the left eye revealed a prominent melanocytic proliferation in the inferior hemisphere with surrounding subretinal fluid extending towards the inferior arcade ( Figure 1 ). Using ultrasound B-scans, we recognized a medium reflective tumor with a base of 13.6 x 10.8 mm and a thickness of 6.0 mm, with surrounding tumor-related retinal detachment ( Figure 1 ).
Presentation of the recurrence. (A) The slit-lamp examination of the anterior segment shows an episcleral sentinel vessel and vascularized, melanotic lesions infiltrating the iris circumferentially. (B) Close-up image focusing on the iridial lesions with vascularization. (C) The ultrasound biomicroscopy (UBM) of the temporal-inferior iridociliary junction illustrates the thickening of the iris. (D) The UBM of the inferior-anterior segment reveals ciliary body infiltration, measuring 2.75 x 3.84 mm at this site. With UBM, iridial and ciliary body thickening was unveiled circumferentially.
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SF3B1 (encoding splicing factor 3B subunit 1), leading to p.Arg625His. Analyses concerning GNA11 (encoding G Protein Subunit Alpha 11) showed a wild-type status. Soon after vitrectomy and endoresection, we noticed that the iridial lesions had increased in number and size, along with severe iridial vascularization and a prominent episcleral (sentinel) vessel ( Figure 2 ). In UBM, we saw a circumferential infiltration of the iris and ciliary body, leading to the diagnosis of a ring melanoma ( Figure 2 ).
Histopathological examination. Hematoxylin and eosin stain of the iridociliary junction, magnification: x10. (A) Superior, (B) nasal parts of the iris were missing due to paraffin embedding; (C) inferior; (D) temporal. All images expose infiltration of an epithelioid-cell-type tumor infiltrating the ciliary body.
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The enucleation took place without complications, and the histopathological analyses of the enucleated eye confirmed the annular growth of the tumor around the iris ( Figure 3 ).
The timeline illustrates the rapid disease course in this case. In about 1 year and 2 months, the patient received the primary diagnosis of uveal melanoma, underwent brachytherapy with good tumor regression, was treated for radiation retinopathy, then diagnosed with a ring-shaped relapse, and underwent enucleation of the eye. The left gray side lists important clinical features, and the right blue side demonstrates surgical interventions. IVI, intravitreal injection; Ru106, ruthenium-106; 23G, 23-gauge.
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