60-88.pdf

Congenital Hypertrophy of the R P E Dr. Yang, 2024 -deep pigmented outline 60 Congenital Hypertrophy of the R P E Pathology – RPE cells taller, more pigmented – Large compact spherical melanosomes...

Congenital Hypertrophy of the R P E Dr. Yang, 2024 -deep pigmented outline 60 Congenital Hypertrophy of the R P E Pathology – RPE cells taller, more pigmented – Large compact spherical melanosomes Management – Observation Dr. Yang, 2024 61  § Uveal nevus § Uveal melanocytoma Tumors of Uvea § Uveal melanoma § Choroidal hemangioma § Choroidal osteoma Dr. Yang, 2024 -all of these are benign except for uveal melanoma 62 Iris Nevus Clinical features – Localized or diffuse lesion of iris stroma – Variably pigmented – Relatively flat – Can grow slowly Pathology – Usually low-grade spindle cells – Benign Management – Observation Dr. Yang, 2024 63 Choroidal Nevus Clinical features – Flat or minimally elevated choroidal lesion – Slate gray/green, but can be amelanotic – Oval, round, distinct borders – Posterior pole or mid-peripheral fundus location – Overlying drusen – 5~20 %of the general population – Asymptomatic, incidental finding Dr. Yang, 2024 -probably present at birth and grows during childhood but rarely grows beyond puberty -usually green in color 64 Choroidal Nevus Dr. Yang, 2024 -typical pigmented choroidal nevus and OCT shows intact RPE and bruch’s membrane 65 Choroidal Nevus Pathology – Usually, low-grade spindle cells – Benign (0.01 ~ 0.02% risk of malignant transformation) Management – Consider risk factors – Fundus photography, ultrasonography, OCT – Periodic observation (q 3-6 months to q 9-12 months) Dr. Yang, 2024 66 Iris/Choroidal Melanocytoma Tumor of deeply pigmented round cells Generally benign, needs no treatment Relatively avascular Common problem is local growth Consider enucleation if extreme growth/size Risk of malignant transformation Dr. Yang, 2024 -still considered benign 67 Iris/Choroidal Melanocytoma Dr. Yang, 2024 -such tumor near the ONH tends to grow and the risk of malignant transformation is higher so location is impt 68 Uveal Melanoma The most common primary malignant intraocular tumor in adults – Choroid: ~90% – Ciliary body: ~6% (worse prognosis) – Iris: ~4% (better prognosis) Dr. Yang, 2024 -develops from melanocytes and is a type of cancer 69 Uveal Melanoma Prognostic factors – Cell type (spindle, epithelioid, mixed) – Tumor size – Location of the anterior margin of the tumor – Degree of ciliary body involvement – Extraocular extension Dr. Yang, 2024 -spindle cell melanoma carries the best prognosis and epithelioid carries the worst prognosis -as the tumor grows the prognosis becomes worse and can metastasize to distant tissue and distant sites 70 Iris Melanoma Caucasian with light-colored irides Predominantly spindle cell type Small discrete lesions or diffuse/infiltrative Growth is diagnostic Heterochromia, chronic uveitis, hyphema Enlarged scleral blood vessels Secondary glaucoma ~3% metastasis within 5 years Dr. Yang, 2024 71 Iris Nevus to Melanoma Rate of transformation Risk factors – ~5% in 5 years – Age of presentation ≤ 40 years – ~8% in 15 years – Blood (spontaneous hyphema) – Clock hour location (inferior quadrant) – Diffuse flat tumor configuration – Ectropion uveae – Feathery ill-defined margins Dr. Yang, 2024 -ectropion uveae refers to prolapse or protrusion of the iris pigment epithelium onto the anterior surface -not –defined edges 72 Iris Melanoma Ancillary tests – Anterior segment fluorescein angiography – High-resolution UBM Management – Observation and careful follow-up – Local resection – Plaque radiation therapy – Enucleation Dr. Yang, 2024 -melanoma is a highly vascularized to the tumor 73 Ciliary Body Melanoma Pigmented ciliary body mass Occult location posterior to iris May attain a large size before clinical diagnosis Nodule of extraocular extension Sentinel episcleral blood vessels May encroach on lens Occasional RD, vitreous hemorrhage Dr. Yang, 2024 -difficult to detect CB melanoma at an early age -middle pic shows ex of extraocular extension of the CB melanoma 74 Ciliary Body Melanoma Prognosis – More likely to progress than iris melanoma – 5-year survival rate is ~ 70% Management – Plaque radiation therapy – External-beam, charged-particle radiation therapy – Local tumor resection – Enucleation Dr. Yang, 2024 75 Choroidal Melanoma Epidemiology (U.S.) – Incidence: 5~6 new cases per 1,000,000 – 1,500~2,500 new cases each year – Mean age at diagnosis: 55~60 years of age – Increasing incidence rate, peaking at 70~75 years of age – Slightly higher incidence in men Dr. Yang, 2024 76 Choroidal Melanoma Risk factors – Caucasian race – Light-colored irides – Positive family history – Pre-existing nevus or melanosis – Chromosomal and genetic abnormalities – Possibly UV light exposure (?) à no clear evidence Dr. Yang, 2024 77 Choroidal Melanoma Pathogenesis – Arises from melanocytes within the choroid – Spindle, epithelioid, or mixed cell types – De novo growth or malignant transformation from pre-existing nevi Dr. Yang, 2024 78 Choroidal Melanoma Ocular symptoms – Asymptomatic unless optic nerve or macula involved – Blurry vision, scotoma – Painless, progressive VF loss – Floaters, flashes – Occasional ocular pain (uncommon) Dr. Yang, 2024 79 Choroidal Melanoma Clinical features – Usually, elevated dome-shaped mass – Break through Bruch’s membrane (mushroom shape) – May be diffuse – Pigmented or amelanotic – Surface orange pigment – Prominent vessels in dome of mass – Exudative RD à common due to buildup of subretinal fluid Dr. Yang, 2024 80 Choroidal Melanoma Dr. Yang, 2024 -bottom left pic is medium sized choroidal melanoma involving the macula and there is surrounding sub-retinal fluid -top right pic: hard to tell but there is a lesion in which the entire melanoma is surrounding the optic nerve -pic all the way to the right shows a large melanoma inferiorly and also shows a retinal detachment 81 Choroidal Melanoma Complications – Choroidal neovascularization – Retinal detachment – Vitreous hemorrhage – Vitritis, uveitis – Secondary glaucoma – Metastasis (liver, lungs, skin) Dr. Yang, 2024 82 Choroidal Melanoma Ancillary tests – Indirect ophthalmoscopy – Ultrasonography – Fluorescein/ICG angiography – CT, MRI – Fine needle aspiration biopsy – Liver function tests /ultrasound Dr. Yang, 2024 -blood worse is done for liver enzymes to find out of uveal melanoma invaded the liver 83 Choroidal Melanoma Ultrasonography – Low-medium reflectivity – Acoustic hollowness – Extrascleral extension Dr. Yang, 2024 -the first initial spike (circled in red) is the surface of the tumor and can see the fast oscillating signs which is characteristic of melanoma 84 Choroidal Melanoma Fluorescein angiography Dr. Yang, 2024 85 Choroidal Melanoma ICG angiography – “Double circulation” Dr. Yang, 2024 -this evidence of intrinsic vasculature is useful in differentiating melanotic melanoma from choroidal metastasize which is usually not vascularized 86 Choroidal Melanoma Dr. Yang, 2024 87 Choroidal Melanoma Dr. Yang, 2024 -lipofuscin deposits -orange pigment granules within the overlying RPE is dying on the surface of the tumor 88