CPC

Alethea Hein

Clinical History

• 53y/o M w/ decreased vision in right eye x 2weeks

• Seen by retina specialist & diagnosed with choroidal melanoma

Clinical Exam• Vision:

– 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

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

Fundus PhotoOptos 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.

*

Fluorescein Angiogram

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.

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.

Pathology Slides

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

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

Mass contains spindle B cells

More spindle B cells and many blood vessels

PAS. Break in Bruch’s membrane by mass

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

PAS stain. Showing ciliary body with thickened epithelial basement membrane

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

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

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

Diagnosis

• Choroidal melanoma

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

Clinical ClassificationLargest Diameter (mm) Largest Elevation (mm)

Very Small </= 7.0 </= 2.0

Small 7.7-10 2.1-3.0

Medium 10.1-15.0 3.1-5.0

Large >15.0 >5.0

5 Risk Factors for Growth of Small Melanocytic Choroidal Tumors

• Tumor thickness >2mm• Posterior tumor margin touching disc• Visual symptoms• Orange pigment• Subretinal Fluid

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– Mitotic figures are rare– 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

Best Indicators of Prognosis

• Size– <1cm cubed = very favorable prognosis– >1cm cubed = poor prognosis

• Cell type• Scleral extension• Mitotic Activity

Clinical Features to Help Predict Metastasis

• Posterior tumor location touching the optic nerve

• Increased Tumor thickness• Symptoms of blurred vision• Documented tumor enlargement

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%

Associated Cytology

• Positive for S-100, HMB-45, Ki-67

Interesting Tidbit

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

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)

Treatment Options

• Enucleation• Plaque Brachytherapy• Charged Particle Radiotherapy• Transpupillary Thermotherapy• Stereotactic Radiotherapy• Local Resection

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

Summary

• Choroidal Melanoma with favorable prognosis• Ciliary body basement membrane thickening

of unknown significance. May indicate underlying diabetic process.

Sources

• Basic Clinical Science Course Section 12: Retina and Vitreous. American Academy of Ophthalmology 2008-2009

• Yanoff, Myron and Fine, Ben. Ocular Pathology. Mosby. 2002