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
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)
Figure 1: Patient #1, 51 y/o female. Likely melanoma clinically, close monitoring for growth and possible brachytherapy.Figure 2: LBD: 6.69, Thickness: 1.49Figure 3: Patient #2, 69 y/o male. Nevus, monitor. Initial vision symptoms from a schisis/RDFigure 4: LBD: 6.30, Thickness: 2.22
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
Due to ongoing developments with COVID-19, we are only able to see patients with urgent eye problems at this time. If you have any questions or concerns, please call us at 323-442-6335.