1. Grand Rounds Conference
Brett Mueller, D.O., Ph.D.
6/3/2016
University of Louisville
Department of Ophthalmology and Visual Sciences

2. Patient Presentation CC: Blurry vision right eye
HPI: 62 yo WF presents w/ progressive blurry vision OD for the past 11 months. She also mentions having monocular diplopia.

3. History POHx: Cataracts-NVS OU PMHx:
1) Hypertension w/ blood pressure being 141/81 in the ER.
FAMHx: none
ROS: none
MEDS: none
ALLERGIES: none

4. Exam 3--2 13 VA P T 14 EOM: full OU CVF: full OU
No APD
EOM: full OU
CVF: full OU

5. Exam OD OS LIDS/LASHES WNL WNL CONJ WNL WNL CORNEA WNL WNL
IRIS WNL WNL
LENS NS NS

6. Fundus Photos OD Color fundus photos:
Orange bigmen is lipofuscin. Retinal precipitates
Color fundus photos:
OD: 0.3 C/D, Large Macular elevation w/ inferior retinal precipitates and nasal RPE hyperplasia
OS: 0.3 C/D, M, V, and P WNL

7. Ocular Ultrasound
B scan hollowness, and low interreflecticity
Elevated choroidal macular lesion measuring 12mm X 8mm X 5mm

8. Autofluorescence

9. OCT Retina
Edge of lfuid there and all on that side

10. Summary DDx: Amelanotic Choroidal Melanoma Metastasis
62 y/o WF an elevated choroidal lesion in her right eye measuring 12mm X 10mm X 5mm
DDx:
Amelanotic Choroidal Melanoma
Metastasis
PLAN: Obtain FNA and place plaque for treatment.
Melanoma systemic testing is

11. FNA of Choroidal Mass H&E Stain Sox10 S100 (Sample)
Pleomorphic polyhedral cells with eosinophilic cytoplasm and a larger, rounder nucleus than spindle cells.
SOX10 is a nuclear transcription factor that plays an important role in melanocytic cell differentiation
The S100 family of proteins is a member of the calcium-binding EF-hand superfamily

12. Choroidal Melanoma Risk Factors
To Find Small Ocular Melanoma Using Helpful Hints Daily
Thickness > 2mm
Subretinal fluid
Symptoms
Orange Pigment
Margin w/in 3mm of the optic disc
Ultrasonographic hollowness
Absence of halo
Absence of drusen
Melanoma metastasis at 10 years was 10% for small melanoma (<3-mm thickness), 23% for medium-sized melanoma (3.1- to 8-mm thickness), and 52% for large melanoma (>8-mm thickness). More specifically, metastasis (per millimeter of thickness) at 10 years was found in 7% (0-1 mm), 13% (1-2 mm), 10% (2-3 mm), 16% (3-4 mm), 24% (4-5 mm), 27% (5-6 mm), 27% (6-7 mm), 39% (7-8 mm), 49% (8-9 mm), 53% (9-10 mm), and 58% (>10 mm) of cases. It is thus evident from these studies that tumor size strongly correlates with patient prognosis. Early detection of uveal melanoma at a time when the malignancy is small is critical in improving patients' survival.
A halo refers to a pigmented choroidal nevus surrounded by a circular band of depigmentation.)
istogenesis of cutaneous halo nevus involves damage to the nevus cells with vacuolization of cells and reduction in melanosomes in the halo. Invading macrophages display large aggregates of melanosomes. In the halo, the vacuolated melanocytes show coagulation of cytoplasm and autophagocytosis of melanosomes
Shields CL, Furuta M, Berman EL, Zahler JD, Hoberman DM, Dinh DH, Mashayekhi A, Shields JA. Choroidal nevus transformation into melanoma: analysis of 2514 consecutive cases. Arch Ophthalmol Aug;127(8): doi: /archophthalmol PubMed PMID:

13. Choroidal Melanoma Risk Factors
Rishi P, Koundanya VV, Shields CL. Using risk factors for detection and prognostication of uveal melanoma. Indian J Ophthalmol Feb;63(2): doi: / PubMed PMID: ; PubMed Central PMCID: PMC
Shields CL, CaterJ, Shields JA, Singh AD, Santos MC, Carvalho C. Combination of clinical factors predictive of growth of small choroidal melanocytic tumors. Arch Ophthalmol 2000;118:360‑4.
Shields CL, Furuta M, Berman EL, Zahler JD, Hoberman DM, Dinh DH, et al. Choroidal nevus transformation into melanoma: Analysis of 2514 consecutive cases. Arch Ophthalmol 2009;127:981‑7.

14. Choroidal Melanoma Most common primary intraocular malignancy
Age normally around 60
Histopathologically: spindle and epithelioid cell type
Orbital spread through scleral channels and vortex veins
Metastasis commonly found in the liver, and only 1-2 % of pts have detectable metastases at the time of presentation
Mortality at 10 years is 50%
CM is 80% of all uveal melanoma but is relaitly uncommon.

15. Amelanotic Choroidal Masses
Differential Diagnosis
Epidemiology
Signs
Symptoms
Amelanotic Melanoma
50s and 60s
White, blue-eyed blondes
Orange pigment, serous retinal detachment, low internal reflectivity
Asymptomatic  blurring of visionocular pain proptosis
Choroidal Metastasis
Males: lung (40%), GI (9%), kidney (8%)
Females: Breast (68%), lung (12%)
Yellow in color, subretinal fluid, high internal reflectivity
Visual symptoms 90%: burred vision, flashes/floaters, and pain
Choroidal hemangioma
Rare, likely congenital, Caucasians
Amelanotic, high internal reflectivity
No symptoms until adulthood, Can get blurred vision 2ndary to RD
88% of metastases are to the choroid, then iris and CB
Choroidal can be bilateral and multifocal and have a lot of subretinal fluid if the mets are active. Very rarely metasis can be hollow
FA and ICG for chroidal hemangioma
Differential Diagnosis
Epidemiology
Signs
Symptoms
Amelanotic Melanoma
50s and 60s
White, blue-eyed blondes
Orange pigment, serous retinal detachment, low internal reflectivity
Asymptomatic  blurring of visionocular pain proptosis
Choroidal Metastasis
Males: lung (40%), GI (9%), kidney (8%)
Females: Breast (68%), lung (12%)
Yellow in color, subretinal fluid, high internal reflectivity
Visual symptoms 90%: burred vision, flashes/floaters, and pain
Choroidal hemangioma
Rare, likely congenital, Caucasians
Amelanotic, high internal reflectivity
No symptoms until adulthood, Can get blurred vision 2ndary to RD

16. Conclusion
Differentiate between choroidal nevus and choroidal melanoma
To Find Small Ocular Melanoma Using Helpful Hints Daily
Differential diagnosis of amelanotic choroidal masses
Distinguish between amelanotic choroidal melanoma and metastasis to the choroid
Sai chavala

17. THANK YOU

18. References
Shields CL, Furuta M, Berman EL, Zahler JD, Hoberman DM, Dinh DH, Mashayekhi A, Shields JA. Choroidal nevus transformation into melanoma: analysis of 2514 consecutive cases. Arch Ophthalmol Aug;127(8): doi: /archophthalmol PubMed PMID:
Shields CL, CarterJ, Shields JA, Singh AD, Santos MC, Carvalho C. Combination of clinical factors predictive of growth of small choroidal melanocytic tumours. Arch Ophthalmol. 2000;188:360-4
Rishi P, Koundanya VV, Shields CL. Using risk factors for detection and prognostication of uveal melanoma. Indian J Ophthalmol Feb;63(2): doi: / PubMed PMID: ; PubMed Central PMCID: PMC
Kanski’s Clinical Ophthalmology A systemic Approach, Eighth Edition. Brad Bowling
Ophthalmic Pathology and Intraocular tumors, BSCS
 Phelps, P. O., et al. "Metastatic mixed germ-cell neoplasm presenting as retinal detachment." Journal of clinical oncology: official journal of the American Society of Clinical Oncology 30.13 (2012): e133
Intraocular Tumors: An Atlas and Textbook By Jerry A. Shields, Carol L. Shields
Gunduz K, Shields CL, Shields JA, et al. Presumed Choroidal granuloma with VH resembling choroidal melanoma
Regillo C. Shields CL, Shields JA, et al. Ocular TB. JAMA 1991; 266; 1490
Demirci H, Shields CL, Shields JA, et al. Ocular TB masquerading as ocular tumors. Surv Ophthalmol 2004;49:78-89.
Angioi K, Terrada C, Locatelli A, Hoang PL, Béné MC, Bodaghi B. Ocular Manifestations of X-linked Chronic Granulomatous Disease: About Two Atypical Case Reports. Ocul Immunol Inflamm Dec;23(6): doi: / Epub 2014 Mar 28. PubMed PMID: