History

• Patient #1 51 y/o female without significant medical or ocular history Referred for retinal lesion found after routine eye exam
• Patient #2 69 y/o male without significant medical or ocular history Referred for retinal lesion noted during DFE for flashes/floaters

Exam Findings

• Patient #1: Vision: 20/25 || 20/20. IOP: 15 || 19. Pupils: WNL OU. DFE 3Hx4V DD hyperpigmented lesion with minimal to moderate elevation with overlying orange pigment overlying superotemporal arcade, peripheral retinal cyst at 2 o’clock
• Patient #2: Vision: 20/20 OU. IOP: 17 OU. Pupils: WNL OU DFE 5 x5 DD hyperpigmented lesion with minimal to moderate elevation with overlying drusen. Ultrasound measurement 6.30 mm LBD 2.22 thickness lesion. OCT with choriocapillaris compression over choroidal mass

Differential Diagnosis

• Choroidal nevus
• Choroidal melanoma
• Pigmented metastasis
• RPE adenoma
• Melanocytoma
• Peripheral exudative hemorrhagic chorioretinopathy (PEHCR)
• Congenital hypertrophy of the retinal pigment epithelium (CHRPE)
• Choroidal hemangioma

Additional Investigations

• Patient #1: Ultrasound dome shaped medium reflectivity 6.69 mm LBD 1.47 thickness lesion. OCT with SRF and shaggy photoreceptors, elevated choroidal mass
• Patient #2: Ultrasound measurement 6.30 mm LBD 2.22 thickness lesion. OCT with choriocapillaris compression over choroidal mass

Diagnosis

• Patient #1: Choroidal melanoma
• Patient #2: Choroidal nevus

Pathophysiology

• Nevi:
• Benign melanocytic lesions of posterior uvea
• 4.6-7.9% in Caucasians (Singh 2005)
• Malignant transformation rare but increases with age
• 1 in ~5000-8000 nevi become malignant (Ganley 1973, Singh 2005)
• Typically asymptomatic
• Wide variation in dimensions
• Management typically with just monitoring
• Melanoma:
• Malignancy of melanocytes
• Part of uveal melanoma more broadly
• Mean age-adjusted incidence of uveal melanoma is ~4 per million
• Higher rate in those with preexisting choroidal nevus and congenital ocular/oculodermal melanocytosis (1/400 risk)
• Middle aged men and women
• Can be asymptomatic or symptomatic (photopsia, floaters, visual field loss, secondary glaucoma)
• Significant size overlap

Treatment

• Nevi: monitor
• Melanoma
• Depends on tumor size, location, symptoms general medical condition
• Options
• Monitor
• Enucleation
• Plaque radiotherapy
• Charged particle irradiation
• Transpupillary thermotherapy

Prognosis and Future Directions

• ED-OCT
• Nevi findings:
• choriocapillaris thinning (and shadowing) (94% of cases)
• RPE changes(43%)
• photoreceptor loss (43%)
• IS/OS irregularity (37%)
• ELM irregularity (18%)
• overlying SRF (16%)
• Melanoma with fresh SRF and shaggy photoreceptors
• (Shields et al for above figures)

Conclusions

• Differentiating choroidal nevi and small choroidal melanomas is a clinical diagnosis
• Benefits significantly from ancillary imaging: ultrasound B-scan and A-scan, OCT-EDI, fluorescein angiography, serial fundus photography
• Caution as there is overlap within diagnostic criteria
• Including key differentiators of size (thickness, longest basal diameter) and OCT characteristics
• To answer title slide: Size of melanocytic lesions does matter, but doesn’t tell the whole picture
• Get an ophthalmic oncology expert involved

References

• Augsburger JJ, Correa ZM, Trichopoulos N, Shaikh A. Size Overlap between Benign Melanocytic Choroidal Nevi and Choroidal Malignant Melanomas. Invest Ophthalmol Vis Sci. 2008;49(7):2823. doi:10.1167/iovs.07-1603
• Augsburger JJ, Schroeder RP, Territo C, Gamel JW, Shields JA. Clinical parameters predictive of enlargement of melanocytic choroidal lesions. British Journal of Ophthalmology. 1989;73(11):911-917. doi:10.1136/bjo.73.11.911
• Ganley JP, Comstock GW. Benign Nevi and Malignant Melanomas of the Choroid. American Journal of Ophthalmology. 1973;76(1):19-25. doi:10.1016/0002-9394(73)90003-2
• Gass JD. Problems in the differential diagnosis of choroidal nevi and malignant melanomas. The XXXIII Edward Jackson Memorial Lecture. Am J Ophthalmol. 1977;83(3):299-323. doi:10.1089/ten.2005.11.1254
• Li HK, Shields CL, Mashayekhi A, et al. Giant Choroidal Nevus. Ophthalmology. 2010;117(2):324-333. doi:10.1016/j.ophtha.2009.07.006
• McLean IW, Zimmerman LE, Evans RM. Reappraisal of Callender’S Spindle a Type of Malignant Melanoma of Choroid and Ciliary Body. American Journal of Ophthalmology. 1978;86(4):557-564. doi:10.1016/0002-9394(78)90307-0
• Shah SU, Kaliki S, Shields CL, Ferenczy SR, Harmon SA, Shields JA. Enhanced Depth Imaging Optical Coherence Tomography of Choroidal Nevus in 104 Cases. Ophthalmology. 2012;119(5):1066-1072. doi:10.1016/j.ophtha.2011.11.001
• Shields CL. Combination of Clinical Factors Predictive of Growth of Small Choroidal Melanocytic Tumors. Arch Ophthalmol. 2000;118(3):360. doi:10.1001/archopht.118.3.360
• Shields CL, Kaliki S, Rojanaporn D, Ferenczy SR, Shields JA. Enhanced Depth Imaging Optical Coherence Tomography of Small Choroidal Melanoma: Comparison With Choroidal Nevus. Arch Ophthalmol. 2012;130(7):850. doi:10.1001/archophthalmol.2012.1135
• Shields C, Manalac J, Saktanasate J, Shields J, Das C. Review of spectral domain enhanced depth imaging optical coherence tomography of tumors of the choroid. Indian J Ophthalmol. 2015;63(2):117. doi:10.4103/0301-4738.154377
• Shields CL, Manalac J, Das C, Ferguson K, Shields JA. Choroidal melanoma: clinical features, classification, and top 10 pseudomelanomas. Current Opinion in Ophthalmology. 2014;25(3):177-185. doi:10.1097/ICU.0000000000000041
• Singh AD, Kalyani P, Topham A. Estimating the Risk of Malignant Transformation of a Choroidal Nevus. Ophthalmology. 2005;112(10):1784-1789. doi:10.1016/j.ophtha.2005.06.011
• Thiagalingam S. Absence of Change in Choroidal Nevi Across 5 Years in an Older Population. Arch Ophthalmol. 2004;122(1):89. doi:10.1001/archopht.122.1.89
• You QS, Xu L, Jonas JB, Wang S, Yang H. Change in choroidal nevi during a 5-year follow-up study: the Beijing Eye Study. British Journal of Ophthalmology. 2010;94(5):575-578. doi:10.1136/bjo.2009.165720

Contact

• Jesse Berry, MD, Assistant Professor of Clinical Ophthalmology and Associate Director of Ocular Oncology, jesse.berry@med.usc.edu
• Jonathan Lu, MD, PGY-2 Ophthalmology resident, jonathan.lu@med.usc.edu

Section Editors

• Charles Flowers, Jr., MD, Associate Professor of Clinical Ophthalmology, Program Director, charles.flowers@med.usc.edu
• Brandon Wong, MD, Assistant Professor of Clinical Ophthalmology, Associate Program Director, brandon.wong@med.usc.edu