An amelanotic choroidal melanoma arising in a young man with tattoo-associated sarcoidosis

Abstract

Purpose: To describe a patient with an amelanotic choroidal melanoma, originally misdiagnosed as a choroidal granuloma, following his systemic diagnosis of tattoo-associated sarcoidosis.

Observations: The amelanotic choroidal tumor, suspected to be a granuloma, failed initial steroid treatment. Full-thickness chorioretinal biopsy demonstrated histologic presence of uveal melanoma and tumor genetics via GEP analysis demonstrated a PRAME negative, Class 1A lesion. The amelanotic choroidal melanoma was treated successfully with I-125 plaque brachytherapy.

Conclusion and importance: Despite a systemic diagnosis which predisposes a patient to uveal granuloma, amelanotic choroidal melanomas can still occur and should be considered. The association of uveal melanoma and sarcoidosis remains rare and of unclear significance.

Keywords: Eye; Sarcoidosis; Tumor; Uveal melanoma.

Fig. 1

Compilation of clinical images. A. Wide-angle Zeiss Clarus photograph demonstrating an inferotemporal subretinal amelanotic or partially pigmented choroidal lesion and associated exudative detachment. B. Wide-angle late phase Optos fluorescein angiograph demonstrating intense intrinsic hyperfluorescence of the lesion consistent with leakage. C. Late phase Heidelberg indocyanine green angiography demonstrates low intrinsic ICG fluorescence compared to background choroidal vasculature. D. Wide-field montage Zeiss Clarus autofluorescence demonstrates increased autofluorescence at the nasal and inferior edges of the lesion consistent with orange pigment. E. Posterior segment ultrasound demonstrates a dome-shaped choroidal lesion with low to medium echogenicity and an inferior exudative detachment. F. Superior-inferior oriented optical coherence tomography demonstrates a choroidal lesion with an overlying exudative detachment.

Fig. 2

A. Low magnification H and E stain demonstrating palisading spindle and epithelioid cells with nests of brown pigment. B. 20X magnification H and E image demonstrates mostly spindle cells with rare inflammatory cells and rare mitotic figures with scattered pigment. C. Low magnification Melan-A immunostain shows intense uptake. D. Low magnification Sox-10 immunostain shows intense uptake. E. Low magnification S-100 immunostain demonstrates minimal uptake. F. Low magnification P63 immunostain demonstrates minimal uptake.