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: 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

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

* 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.

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.

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.

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

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.

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 Classification Largest 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 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