CLINICAL PEARLS IN OCULAR ONCOLOGY

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1 CLINICAL PEARLS IN OCULAR ONCOLOGY IRIS NEVUS - Two kinds circumscribed and diffuse - Photodocumentation important to monitor growth - Risk Factors for iris nevus growth to melanoma (ABCDEF) A Age (young), B Blood (hyphema, feeder vessel, intrinsic vessel), C clock hour (inferior), D Diffuse configuration (iris stromal and angle seeding), E Ectropion, F Feathery marin IRIS MELANOCYTOMA - Dark brown to black in color - Cobblestone appearance - Feathery margin - Bits can break off and clog trabecular meshwork - Growth in 23% patients - Malignant transformation rare CHOROID NEVUS % incidence - Photodocumentation important - Risk of melanoma TFSOM ( To Find Small Ocular Melanoma Using Helpful Hints Daily) o Thickness > 2mm o Fluid o Symptoms o Orange pigment o Margin close to disc ( 3 mm) o Drusen absence o Ultrasound hollowness o Halo absence

2 CONGENITAL HYPERTROPHY OF RETINAL PIGMENT EPITHELIUM (CHRPE) - Well circumscribed - Lacunae + - Halo + - Can have nodules - Rare risk of transformation to adenocarcinoma MULTIFOCAL CHRPE - Bear Tracks - Bilateral, multifocal - Gardners syndrome 100% chance of intestinal malignancy - Eye features 78% sensitive, 95% specific CHOROIDAL HEMANGIOMA - Circumscribed in posterior pole with subretinal fluid - Often misdiagnosed and treated as central serous retinopathy - FA and ICG diagnostic - Treatment PDT - Diffuse hemangioma Sturge Weber syndrome PRIMARY INTRAOCULAR LYMPHOMA - 5 th to 7 th decade - Associated with primary CNS lymphoma - Bilateral posterior uveitis - Diffuse vitreous cells with deep subretinal yellow-white infiltrates - Keep in mind with an elderly person with atypical uveitis - Diagnostic vitrectomy required

3 INTRAOCULAR METASTASIS - Most common tumor of the eye - Most common site Choroid - 25% have no known history of cancer. Primary site unknown in 10% - Most common breast in women and lung in men - Bilateral 20-50% - Multifocal lesions 30% - Usually cream colored lesions with subretinal fluid - SRF indicates active metastasis - Leopard skin appearance clumps of brown pigment CONJUNCTIVAL PAPILLOMA - Due to human papilloma virus infection - Spontaneous regression possible - Exicision with cryotherapy curative - Can also try oral cimetidine CONJUNCTIVAL SQUAMOUS CELL CARCINOMA - Most common conjunctival tumor - Advanced age, male gender, exposure to solar UV radiation, infection with HPV and HIV - Carcinoma in situ (CIN) if not through basement membrane - Treatment surgery with possible topical chemotherapy CONJUNCTIVAL NEVUS - Cysts are diagnostic seen in 70% - Nevi stop abruptly at the limbus - Pigmented lesions on tarsal conjunctiva, fornix, caruncle or plica should be excised and biopsied

4 PRIMARY ACQUIRED MELANOSIS (PAM) - Patchy pigment - Can be without or without atypia - With atypia 50% chance of melanoma - Can be differentiated only with biopsy CONJUNCTIVAL MELANOMA - 70% arise from PAM, 2% from nevi and rest denovo - Chance of metastasis 25% - Early detection and complete excision curative NEVUS OF OTA - Pigmentation of skin, sclera, iris and choroid - Associated pigment of ipsilateral meninges and ipsilateral hard palate - Risk of melanoma 1 in Risk of glaucoma 10% - Annual exam warranted with gonioscopy at every visit and detailed fundus exam PHAKOMATOSES NEUROFIBROMATOSIS 1 - Eyelid Neurofibroma, Café au lait - Conjunctiva Plexiform neurofibroma - Cornea Prominent corneal nerves - Iris Ectropion, Nevus, Lisch nodule - Glaucoma ipsilateral to plexiform neurofibroma - Retina / Choroid Retinal glial hamartoma - Orbit Glioma, Pulsating exophthalmos, Neurofibroma, Schwannoma

5 NEUROFIBROMATOSIS 2 - Lens cortical cataract, posterior subcapsular cataract - Retina Combined hamartoma, epiretinal membrane - Orbit Optic nerve sheath meningioma, optic nerve glioma TUBEROUS SCLEROSIS - Hamartoma (seen in 40-50% of TS patients) - Other features early onset cataract, hamartoma of iris pigment epithelium, eyelid angiofibroma, strabismus, coloboma, iris depigmentation