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A man in his 80s with pseudophakia presented for routine follow-up of an elevated choroidal nevus in his right eye. His medical history was notable for stage III non-Hodgkin follicular lymphoma in the hilar and axillary nodes, which was asymptomatic and actively monitored by oncology, prostate cancer treated surgically that was in remission, and atrial fibrillation managed with medication (digoxin and warfarin). The choroidal nevus had been observed for the past 2 years; ultrasonography from 2 years prior revealed dimensions of 1.9 (height) × 9.3 (base) mm with medium internal reflectivity. There were no other concerning features noted, such as orange pigment or associated subretinal fluid.
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Mantle cell orbital lymphoma
B. Obtain orbital imaging
The differential diagnosis includes lymphoproliferative lesions, granulomatosis with polyangiitis, IgG4-related disease, idiopathic orbital inflammation, solitary fibrous tumor, metastatic lesions, pleomorphic adenoma, and adenoid cystic carcinoma. While fluorescein angiography (choice A) can characterize the vasculature of choroidal lesions, including double circulation associated with choroidal melanomas, the lesion of concern here is located in the extraocular space. Choroidal melanomas are acoustically hollow, like the mass seen here, and they most commonly metastasize to the liver, but obtaining an invasive biopsy (choice C) without further orbital imaging is inappropriate. Close monitoring alone (choice D) in the setting of a new mass is inappropriate. Orbital computed tomography was obtained (choice B).
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Corresponding Author: Christina Y. Weng, MD, MBA, Department of Ophthalmology, Cullen Eye Institute, Baylor College of Medicine, 1977 Butler Blvd, 3rd Floor, Houston, TX 77030 (firstname.lastname@example.org).
Published Online: October 24, 2019. doi:10.1001/jamaophthalmol.2019.4106
Conflict of Interest Disclosures: Dr Weng has received personal fees from Alcon, Alimera Sciences, and Allergan. No other disclosures were reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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