1. CPC
Alethea Hein

2. Clinical History 53y/o M w/ decreased vision in right eye x 2weeks
Seen by retina specialist & diagnosed with choroidal melanoma

3. Clinical Exam Vision: Color vision: Visual fields:
Right eye: 20/50-2
Left eye: 20/20
Color vision:
Right eye: 7/13
Left eye: 13/13
Visual fields:
B-scan right eye: mushroom-shaped choroidal mass with low internal reflectivity
Anterior segment:
Bilateral trace nuclear sclerosis, otherwise normal
Fundus exam:
Right eye:
Optic nerve head obscurred by mass
Inferior nasal mushroom shaped, amelanotic mass at 4-5 o’clock, on a base of pigmented, flatter mass, with serous retinal detachment 3-6 o’clock
Left eye: wnl

4. Assessment and Plan
Choroidal melanoma with amelanotic mushroom component and secondary retinal detachment – right eye
Discussed enucleation vs. Brachytherapy with plaque radiation.
Patient chose enucleation

5. *
Optos photo of right eye showing infranasal mass (*) with partial obscurration of the optic nerve head. Also visible is a retinal detachment (white arrow) surrounding mass. Otherwise, wnl.
Fundus Photo
Optos photo of right eye showing infranasal mass (*) with partial obscurration of the optic nerve head. Also visible is a retinal detachment (white arrow) surrounding mass.

6. Fluorescein Angiogram
1st image shows arterial phase of angiogram. Can see the mass has good perfusion.
2nd image shows venous phase of angiogram. Can see mass and area of retinal detachment have increased signal suggesting leakage of fluorescein from blood vessels.
Arterial phase of angiogram.
The mass has good perfusion.
Venous phase of angiogram. Mass and area of retinal detachment have increased signal suggesting leakage of fluorescein from blood vessels.

7. Recirculation phase of angiogram continues to show hyperfluorescent mass and area surrounding mass with corresponds to retinal detachment. Shows that there is leakage of fluorescein from the blood vessels.
Recirculation phase of angiogram continues to show hyperfluorescent mass and area surrounding mass which corresponds to retinal detachment. Shows that there is leakage of fluorescein from the blood vessels.

8. Pathology Slides Surgical Pathology #: PHS10-17321
Highly cellular mass (blue arrow)overlying area of proteinaceous fluid (white arrow)
Surgical Pathology #: PHS
Highly cellular mass (blue arrow)overlying area of proteinaceous fluid (white arrow)

9. Blue arrow: Can see that the mass is sub-retinal

10. Mass contains spindle B cells

11. More spindle B cells and many blood vessels

12. PAS. Break in Bruch’s membrane by mass

13. PAS. Again, can see break in bruch’s membrane

14. PAS stain. Showing ciliary body with thickened epithelial basement membrane.

15. Melanoma cells extend along blood vessels into the sclera, but only slightly.

16. Melin A melanocyte marker –
Melin A melanocyte marker – shows melanocytes (red) around a blood vessel within an emissary canal
Melin A melanocyte marker –
shows melanocytes (red) around a blood vessel within an emissary canal

17. KI-67 shows proliferating cells
KI-67 shows proliferating cells. Shows more proliferation than would see with a nevus.
KI-67 shows proliferating cells. Shows more proliferation than would see with a nevus.

18. Diagnosis
Choroidal melanoma

19. Discussion Median Age: 55yrs M>F
Caucasians>>African Americans (15:1)
Unilateral most common
Blue irides>Brown irides
Intense exposure to UV light increases risk
Bilateral primary melanoma ~1.8% of uveal melanoma patients
Most common presentation: mass found on routine examination or after complaint of blurred vision.
Neural retinal detachment seen in ~75% of cases

20. Clinical Classification
Largest Diameter (mm)
Largest Elevation (mm)
Very Small
</= 7.0
</= 2.0
Small
7.7-10
Medium
Large
>15.0
>5.0

21. 5 Risk Factors for Growth of Small Melanocytic Choroidal Tumors
Tumor thickness >2mm
Posterior tumor margin touching disc
Visual symptoms
Orange pigment
Subretinal Fluid

22. Callender Classification and Prognosis
Spindle A
2nd rarest type (5%)
Cohesive cells that contain small, spindled nuclei having central dark stripe
No distinct nucleoli
Mitotic figures are rare
Survival rate: ~92%
Spindle B
Common (39%)
Cohesive cells with spindled nuclei with distinct nucleoli
~6% form a palisaded arrangement called a fascicular pattern
Survival rate: ~75%
Epithelioid
Rarest type (3%)
Noncohesive cells with large, round nuclei
Prominent nucleoli
Mitotic figures are common
Survival rate ~28%
Mixed
Most common type (45%)
Contains both a significnt spindle cell component and an epithelioid cell component
Survival rate ~41%
Necrotic
Uncommon (7%)
Cell type not identifiable because tumor is so necrotic

23. Best Indicators of Prognosis
Size
<1cm cubed = very favorable prognosis
>1cm cubed = poor prognosis
Cell type
Scleral extension
Mitotic Activity

24. Clinical Features to Help Predict Metastasis
Posterior tumor location touching the optic nerve
Increased Tumor thickness
Symptoms of blurred vision
Documented tumor enlargement

25. Associated Findings Invasion of Bruch’s membrane ~63% of tumors
If membrane intact, tumor is oval in shape
If membrane ruptured, tumor is mushroom shaped
Invasion of scleral canals ~32% of tumors
Invasion of optic nerve ~5% of tumors
Invasion of vortex veins ~13% of tumors
Vortex veins should be sampled on all enucleated globes
Vortex vein invasion carries extremely unfavorable prognosis
Neural retinal detachment present in ~75%
Extraocular extension ~13% of tumors
If tumor is transected during enucleation, recurrence rate is ~50%

26. Associated Cytology
Positive for S-100, HMB-45, Ki-67

27. Interesting Tidbit
~4% of eyes with opaque media enucleated from white patients (blind for ~6mos) harbor malignant melanoma

28. Differential Diagnosis
Hemorrhage
Cyst
Serous retinal detachment
Subretinal neovascularization
Tumor (hemangioma, nevus, metastatic carcinoma, lymphoma & lesions of pigmented epithelium)
Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP)

29. Treatment Options Enucleation Plaque Brachytherapy
Charged Particle Radiotherapy
Transpupillary Thermotherapy
Stereotactic Radiotherapy
Local Resection

30. Our Patient’s Tumor and Prognosis
Size:
<1cm cubed = very favorable prognosis
Cell type: Spindle B (survival rate with Spindle B: 75%)
Scleral extension: yes, but minimal
Mitotic Activity: low mitotic activity
Therefore: good prognosis

31. Summary Choroidal Melanoma with favorable prognosis
Ciliary body basement membrane thickening of unknown significance. May indicate underlying diabetic process.

32. Sources
Basic Clinical Science Course Section 12: Retina and Vitreous. American Academy of Ophthalmology
Yanoff, Myron and Fine, Ben. Ocular Pathology. Mosby. 2002