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Choroidal Melanoma TNM staging system by A J C C (American Joint Committee on Cancer) Tumor (size, extent) Lymph Nodes Metastasis Collaborative Ocular Melanoma Study (COMS) Apical height Largest basal diameter...

Choroidal Melanoma TNM staging system by A J C C (American Joint Committee on Cancer) Tumor (size, extent) Lymph Nodes Metastasis Collaborative Ocular Melanoma Study (COMS) Apical height Largest basal diameter Small 1.5 – 2.4 mm 5 – 16 mm Medium 2.5 – 10 mm ≤ 16 mm Large > 10 mm > 16 mm Dr. Yang, 2024 89 Choroidal Melanoma Collaborative Ocular Melanoma Study (COMS) 1. Small-sized choroidal melanoma study Observational study 21% and 31% growth within 2 years and 5 years respectively Tumor-related mortality: ~1% at 5 years 2. Medium-sized choroidal melanoma study Enucleation vs. plaque radiotherapy No difference in survival rate 3. Large-sized choroidal melanoma study Pre-enucleation radiation vs. enucleation alone No difference in survival rate Dr. Yang, 2024 90 Choroidal Melanoma Management (COMS) 1. Small choroidal melanoma Still controversial Initial observation vs. early intervention 2. Medium choroidal melanoma Plaque brachytherapy à common therapy External beam irradiation 3. Large choroidal melanoma Enucleation à when it causes severe glaucoma or invades the optic nerve Dr. Yang, 2024 91 Choroidal Melanoma Management principles Factors that help determine treatment To get rid of the cancer and save life Tumor size, location, activity To save the eye if possible Potential for preserving vision To preserve as much vision as possible Status of opposite eye Patient’s age, general health Metastasis Dr. Yang, 2024 92 Choroidal Melanoma Conventional management Novel therapeutic approaches Observation Immunotherapy Laser photocoagulation Gene therapy Transpupillary Thermotherapy Radiation (plaque brachytherapy, external beam) Surgery (local resection, enucleation, exenteration) Dr. Yang, 2024 93 Choroidal Melanoma Prognostic factors – Cell type: Spindle A/B cells, epithelioid cells, intermediate cells – Cytogenetics: Chromosomes 3, 6, 8, 9 abnormalities – Tumor size/growth pattern: ~1mm ≈ 5% metastasis – Proximity to optic disc, extraocular extension – Metastasis: ~34% in 10 years – Overall high mortality rate: 30-50% death within 10 years Dr. Yang, 2024 94 Choroidal Melanoma Differential diagnoses – Choroidal nevus, melanocytoma – Congenital hypertrophy of the RPE – Choroidal hemangioma – Choroidal metastasis – Choroidal osteoma – Choroidal detachment Dr. Yang, 2024 95  Choroidal Nevus vs. Melanoma (Shields C L et al. Arch Ophthalmol. 2009;127(8):981-987) Suspicious signs for growth/malignant transformation T hickness > 2 mm F luid (subretinal) S ymptoms O range pigment (*) M argin < 3mm from optic disc (*) U ltrasonographic H ollowness (*) H alo absence (*) D rusen absence (*) “ T o F i n d S m a l l O c u l a r M e l a n om a U s i n g H e l p f u l H i n t s D a i l y ” Dr. Yang, 2024 -orange pigment is found in melanoma but not in nevus -hollowness in B scan is more likely to be melanoma -drusen is associated with nevus not melanoma 96 Choroidal Nevus to Melanoma (Shields C L et al. Arch Ophthalmol. 2009;127(8):981-987) TFSOM risk factors for growth in 5 years Rate of malignant transformation If no risk factors: < 3 - 5% At 5 years: 8.6% If one risk factor: 38% At 10 years: 12.8% If ≥ 3 factors: 50% At 15 years: 17.3% If all 5 risk factors: 95% Dr. Yang, 2024 97 Choroidal Nevus to Melanoma (Shields C L et al. B JO. 2018;103:1441-1447) Suspicious signs for growth/malignant transformation T hickness > 2 mm by ultrasonography F luid (subretinal) by OCT S ymptoms of VA loss of 20/50 or worse O range pigment by FAF M elanoma acoustic hollowness by ultrasonography DIaMeter > 5 mm by fundus photography “ To F i n d S m a l l O c u l a r M el a n om a D oi n g I M a g i n g ” Dr. Yang, 2024 -latest acroynms 98 Choroidal Nevus to Melanoma (Shields C L et al. B JO. 2018;103:1441-1447) Risk of malignant transformation based on TFSOMDIM – No risk factors: 1% risk – 1 risk factor: observe with closer follow up – 2 risk factors: follow up with imaging – 3 or more risk factors: can reach 100% depending on the specific combination Dr. Yang, 2024 99 Choroidal Hemangioma Clinical features – Uncommon, benign vascular tumor of choroid – Hamartoma composed of large mature choroidal vascular channels – Red-orange, ill-defined, disc-shaped choroidal tumor – Mostly located in posterior pole region – Probably congenital but not hereditary à detected later in life – No exact cause known Dr. Yang, 2024 -choroidal vascular tumor -usually located near the macula or the optic nerve -pt is symptomatic if the hemangioma is in the macular area or causes other complications like vision is blurry causing a hyperopic shift -there are two types; circumscribed and diffused forms 100 Choroidal Hemangioma Symptoms – Asymptomatic – Blurry vision – Metamorphopsia – Flashes of light Dr. Yang, 2024 101  Choroidal Hemangioma Circumscribed choroidal hemangioma – Raised, dome-shaped – Red-orange, salmon pink mass with distinct margins – Located near the disc or macula – Sporadic, unilateral, isolated occurrence – Diagnosed when visually symptomatic in 2nd~4th decades – No significant exudates Dr. Yang, 2024 -easier to detect bc of the dome shape and elevated, and margins are more defined 102  Choroidal Hemangioma Diffuse choroidal hemangioma – Sturge-Weber syndrome – Diffuse deep red discoloration of fundus – Diffuse uneven choroidal thickening – May have marked retinal vascular tortuosity – Pseudo-glaucomatous cupping – Usually diagnosed before the onset of visual symptoms Dr. Yang, 2024 -usually evident at birth -diffused form is detected much earlier than circumscribed form 103 Choroidal Hemangioma Fluorescein angiography Dr. Yang, 2024 -both circumscribed and diffused show similar features on FA, both fluoresceine and icga 104 Choroidal Hemangioma ICG angiography – Late “wash-out” phenomenon Dr. Yang, 2024 -characterized with early hyperfluorescent and then late hypo 105 Choroidal Hemangioma Dr. Yang, 2024 -can appreciate the smooth dome shaped surface 106 Choroidal Hemangioma Ultrasonography – High internal reflectivity – Acoustic solidity – No choroidal excavation Dr. Yang, 2024 -T indicates signal from the tumor -b scan shows a solid tumor -middle pic shows a diffused hemangioma -right pic shows diffused hemangioma with extensive serous retinal detachment 107 Choroidal Hemangioma Complications – Fibrous metaplasia of overlying RPE – Thickened/cystic changes of overlying retina – Serous retinal detachment – Secondary glaucoma: NVG, ACG Dr. Yang, 2024 108 Choroidal Hemangioma Management Prognosis – Observation if asymptomatic – Poor vision due to location of hemangioma – Photodynamic therapy – Poor prognosis due to retinal/choroidal complications and secondary glaucoma – Laser photocoagulation – More than 50% demonstrate VA < 20/40 (often ≤ 20/200) – Transpupillary thermotherapy – Radiation therapy – Anti-VEGF injections, oral beta-blocker – Enucleation if painful blind eye Dr. Yang, 2024 -many choroidal hemangiomas never grow or leak so they can be observed without intervention 109 Choroidal Osteoma Clinical features – Benign ossifying tumor – Yellowish white/orange placoid lesion – Relatively flat, well-defined, oval/round, scalloped edges – Black or brown pigment – Juxtapapillary or peripapillary location, macula – Unilateral > bilateral – Young healthy women in the 2nd~3rd decades, rarely reported – Slowly growing Dr. Yang, 2024 -slow growing tumor involving entire thickness of the choroid which progressively calcifies over time -overlying RPE is focally depigmented with sometimes clumps of pigments showing up as granules along bruch’s membrane -etiology is unknown 110 Choroidal Osteoma Ancillary tests – Ultrasonography – Computed tomography – Fluorescein angiography Dr. Yang, 2024 111 Choroidal Osteoma Dr. Yang, 2024 -CT scan shows plaque like lesion behind the left globe and is very hyperintense similar to bone tissue 112 Choroidal Osteoma Dr. Yang, 2024 113 Choroidal Osteoma Complications – Choroidal neovascular membrane – Decalcification – Sight threatening if located in the macula Dr. Yang, 2024 -the tumor itself does not need treatment but associated overlying retinal changes or choroidal neo may require treatment 114 Choroidal Osteoma Management – Observation – Laser photocoagulation – Photodynamic therapy – Anti-VEGF injection Dr. Yang, 2024 115 Choroidal Osteoma Differential diagnosis – Amelanotic choroidal melanoma or nevus – Choroidal hemangioma – Choroidal metastasis – Sclerochoroidal calcification Dr. Yang, 2024 116 Choroidal Osteoma DDx Sclerochoroidal calcification – Deposition of calcium salts in sclera/choroid – Idiopathic, hypercalcemia, other syndromes – Bilateral, multifocal – S/T location along arcades Dr. Yang, 2024 117