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You Can`t Afford To Miss This! - American Academy of Optometry
8/1/2013 You Can’t Afford to Miss This! Understanding Posterior Ocular Tumors Lauren L. Richards, OD Carlo J. Pelino, OD, FAAO Disclosure Statement: • Nothing to disclose Please silence all mobile devices. Unauthorized recording of this session is prohibited. You Can’t Afford To Miss This! Understanding Posterior Ocular Tumors Lauren L. Richards, OD Carlo J. Pelino, OD, FAAO The Pennsylvania College of Optometry at Salus University Squamous Cell Carcinoma tumor /tu·mor/ (too´mer) also called neoplasm. a new growth of tissue characterized by progressive, uncontrolled proliferation of cells. The tumor may be localized or invasive, benign or malignant. A tumor may be named for its location, for its cellular makeup, or for the person who first identified it. Mosby's Medical Dictionary, 8th edition. © 2009, Elsevier. Basal Cell Carcinoma Tumor Definition • Eyelid Tumors • Iris / Ciliary Body / Choroidal Tumors • Retina / ONH and Pigment Epithelium • Conjunctival Tumors Ocular Tumors • • • • • • • • • • Choroidal Nevus Choroidal Melanoma Congenital Hypertrophy of the RPE Choroidal Metastasis Melanocytoma Combined Hamartoma of the Retina & RPE Choroidal Hemangioma Choroidal Osteoma Retinal Astrocytic Hamartoma Retinoblastoma Lecture Outline 1 8/1/2013 Ocular Anatomy NFL ELM NFL: Nerve Fiber Layer ILM: Inner Limiting Membrane GCL: Ganglion Cell Layer IPL: Inner Plexiform Layer INL: Inner Nuclear Layer ILM IS GCL IPL IS/OS OS Retinal Anatomy INL RPE OPL: Outer Plexiform Layer ONL: Outer Nuclear Layer ELM: External limiting membrane IS: Photoreceptor Inner Segment OPL ONL Choroid IS/OS: Junction of inner and outer photoreceptor segments OS: Photoreceptor Outer Segment RPE: Retinal Pigment Epithelium Cirrus HD-OCT Healthy Macula www.studyblue.com Melanocyte vs Melanosome • Most common intraocular tumor • Proliferation of choroidal melanocytes • Present in ~ 7.9% of Caucasians CHOROIDAL NEVUS • Growth is rare after puberty ???? Choroidal Nevus 2 8/1/2013 As we age: • Nevi are less than 2mm in thickness • Nevi increase in number and thickness • No known relationship to sunlight exposure • Pigment changes • Have indistinct borders • Metaplasia • May undergo malignant change into melanoma • Drusen / lipofuscin Choroidal Nevus Choroidal Nevus • Typically do not cause visual symptoms • Signs of Suspicion • • • • • http://www.eyetumour.co.uk Population by Race for the US: 2012 Vision changes > 2 mm in thickness Orange pigment Proximity to optic disc Subretinal fluid RACE 1 in 8845 transform into melanoma Total population . . . . . . . . . . . . 313,914,040 100.0% Caucasian . . . . . . . . . . . . . . . . . 244,852,951 78.% Retina. 2005 Apr-May;25(3):243-52. westcoastretina.com eyecancer.com Clinical Findings Source: quickfacts.census.gov To Find Small Ocular Melanoma Nevoma? • • • • • T= thickness F= subretinal fluid S= symptoms O= orange pigment M= tumor margin touches disk • • • • No risk factors (<4%) 1 risk factor (36%) 3 risk factors (50%) 5 risk factors (70%) DOCUMENTED GROWTH - MEANS EVERYTHING Using Helpful Hints = Ultrasound hollow, Halo 3 8/1/2013 Nevus in 2004 Nevus in 2009 ? Nevus vs Melanoma? OCT • Can detect retinal edema, subretinal fluid, retinal thinning and RPE detachment • Helpful in determining presence of drusen FA • Findings depend on the amount of pigment within nevus and the overlying RPE • Hypofluorescence due to blockage of choroidal filling • Hyperfluorescence of surface drusen Special Testing CAN J OPHTHALMOL—VOL. 42, NO. 2, 2007 • Baseline fundus photography • Consider OCT if location permits • Consider B-Scan if suspicious • Yearly dilated fundus examination CHOROIDAL MELANOMA Treatment & Management 4 8/1/2013 • Accounts for 90% of all uveal melanomas • Cells undergo neoplasia, reproducing at a faster rate than usual • Typically large • Although 30% are less than 3 mm thick • Orange pigment • May be amelanotic • Look for subretinal fluid • 91% vs 14% of nevi • Intermittent blurred vision or flashes secondary to fluid shift • Fluid = significant risk of metastasis Choroidal Melanoma Clinical Findings Choroidal Melanoma Choroidal Melanoma Retinal Arterial Macroaneurysm 5 8/1/2013 Asymptomatic Caucasian ethnicity Flashes Systemically well Light colored eyes (blue) Fair skin Pain is rare Propensity to burn when exposed to UV light Scotoma Cutaneous nevi or freckles Vitreous hemorrhage Iris nevi VF defect www.abouttrend.com Welders Presentation of Melanoma Risk Factors of Melanoma www.sightnation.com Quadrant Uveal Melanoma in 8100 patients OCT • Can detect sub-retinal fluid • Has been shown to detect early seeding • Helpful in monitoring response to treatment Superior 22% • Macula 4% Nasal 21% Temporal 28% • • • • B Scan Acoustically hollow Choroidal excavation with orbital shadowing Classic mushroom appearance (less common) Can identify extraocular extension FA • No pathognomonic pattern • Typically, mottled fluorescence during arteriovenous phase, followed by leakage and staining Diffuse 3% Inferior 22% Special Testing CAN J OPHTHALMOL—VOL. 42, NO. 2, 2007 Shields CL, et al. Uveal melanoma analyses in 8100 consecutive patients. Report #1 Sentinel Vessels Intrinsic Blood Vessels 6 8/1/2013 Collaborative Ocular Melanoma Study • Organized and funded in 1985 to address issues related to the management of choroidal melanoma. > 4000 patients. 65% pts eligible • Primarily to study the overall survival of patient following treatment Small melanomas < 2.5 mm in height Medium melanomas Extraocular Extension 2.5 – 10.0 mm in height Large melanomas > 10.0 mm in height • Secondary outcomes = metastasis-free survival, years of useful vision Eye 2001 Vol: 15(4):550-551. Sinead Fenton; Teresa Sandinha; William R Lee; Ewan G Kemp Risk factors for metastasis • Enucleation • Radioactive plaques • Proton beam radiotherapy • Transpupillary thermotherapy • Local resection Most widely accepted Less common • Thickness > 2 mm • Symptoms: Flashes, floaters, loss of vision • Proximity to the optic nerve • Documented growth Treatment & Management Uveal Melanoma Shields Cl Shields JA. Risk factors for metastasis of small choriodal melanocytic Lesions. Ophthalmology 1995 • 10 year mortality for uveal melanoma • Large 50% • Medium 25% • Small 12% • Most common sites of metastasis for uveal melanoma • • • • Liver 89% Lung 29% Bone 17% Skin / Subcutaneous 12% • Pattern of metastasis • Uveal: hematogenous • Cutaneous: lymphatic • Median survival after dx of metastasis - 6 months Choroidal Melanoma • Using fluorescence in situ hybridization and molecular assay techniques, several genetic abnormalities in uveal melanoma were found on chromosomes 1, 3, 6, and 8 • Monosomy 3 • Found in up to 50% of uveal melanomas • Imparts a worse prognosis. • In small melanoma it provokes the argument for earlier treatment than observation. Role of Cytogenetics 7 8/1/2013 Interesting Case… 8 8/1/2013 • Common benign lesion CONGENITAL HYPERTROPHY OF THE RETINAL PIGMENT EPITHELIUM • Focal area in which the RPE cells are taller and more densely packed with melanosomes Congenital Hypertrophy of the Retinal Pigment Epithelium • These lesions are not true tumors • Discrete margins, typically near the equator • No growth • No subretinal fluid • Lacunae are common • Ultrasound will show flat lesion • Multiple lesions may be confined to one sector of the fundus – bear tracks • No intrinsic vessels dro.hs.columbia.edu, mrcophth.com, webeye.ophth.uiowa.edu eyedrd.org Clinical Findings Congenital Hypertrophy RPE 9 8/1/2013 Familial Adenomatous Polyposis (FAP) • Typically remains stationary • AD inheritance • Adenomatous polyps throughout rectum & colon • Starts to develop in adolescence (15-40 yrs) • If untreated – all pts will develop colorectal cancer • May occasionally increase in diameter or spawn an elevated nodule that may represent adenoma or adenocarcinoma >80% of patients with FAP have atypical CHRPE lesions Systemic Association Treatment & Management •Most common site for uveal metastases -90% •Estimated incidence of 30,000 cases/year • Choroidal melanoma 2,500 cases per year •Primary site • Breast (women) • Lung (men) CHOROIDAL METASTASIS http://www.eyecancer.com, Dr. Shivan Maini Br J Ophthalmol 2008;92:156-157 Choroidal Metastasis Lung Metastasis • Creamy-white, placoid choroidal lesion • Fast growing • Usually found in posterior pole • Exudative RD common • Tumors may metastasize elsewhere Clinical Findings Eye Cancer Network Choroidal Metastasis 10 8/1/2013 Breast Metastasis Metastatic Prostate Cancer Choroidal Metastasis OCT • Dome shaped elevation of retina and RPE with adjacent subretinal fluid • Can detect retinal edema, RPE thickening and RPE detachment B Scan • Shows diffuse choroidal thickening • Moderately high internal acoustic reflectivity FA • Shows early hypofluorescence and diffuse late staining Metastatic Breast Cancer Special Testing Dr. Aaron Gold • Observation • Ocular metastases are the most common ocular malignancies • Radiotherapy • TTT • Estimated incidence of 30,000 cases per year • Systemic therapy • Enucleation Treatment & Management • Choroidal melanoma 2,500 cases per year Choroidal Metastasis 11 8/1/2013 • Breast cancer is the most common tumor to metastasize to the eye - followed by lung cancer • 85% of patients with breast cancer metastases will have a known history of breast cancer • Breast cancer metastases tend to be bilateral and multiple • Breast cancer in 2010 • (< 1%) new cancers in men • 192,400 (27%) new cancers in women • Metastasis from breast cancer occurs in 25% of women at a median of 5 years. 3 years if ocular metastasis • 40% of these patients have a brain metastasis • The most common location is the lung, bone, lymph nodes and liver Metastatic Tumors Metastatic Tumors http://www.nationalbreastcancer.org/metastatic-breast-cancer • Lung cancer metastasis rarely bilateral and multifocal • They often produce pain • Lung cancer metastasis 70% of patients don’t know they have cancer MELANOCYTOMA • Chest x-ray very important www.medicalartstudio.com Metastatic Tumors • Benign melanocytic uveal tumor • Patients are usually asymptomatic • Composed of large, plump magnocular nevus cells that are heavily pigmented • Adjacent to or within the optic nerve • Can present in all age groups and races • • African-Americans Females Melanocytoma • Black in color with feathery margins • Visual field defect may be present • APD may be present Clinical Findings 12 8/1/2013 OCT • Disruption of the internal limiting membrane • Disorganization of involved retina • Visualization of subretinal surface FA • Shows hypofluorescence of deep vessels due to blockage • The disc margin may stain in late phases Special Testing Melanocytoma Dr. Aaron Gold CAN J OPHTHALMOL—VOL. 42, NO. 2, 2007 • No systemic association • Typically stationary • 2% of cases develop into melanoma COMBINED HAMARTOMA OF THE RETINA & RPE • No treatment is recommended Treatment & Management • Grey pigmentation with superficial gliosis • Rare, benign tumor • Likely congenital • Involves retina, RPE and overlying vitreous • Typically presents in late childhood or early adulthood • • • • Strabismus Blurred vision Metamorphopsia Loss of vision • Secondary retinal wrinkling and vessel tortuosity • Lesions can be • Juxtapapillary • Peripapillary • Within posterior pole Can be Asymptomatic Wills Eye Resident Case Series, April 2007 Combined Hamartoma of the Retina & RPE Absence of retinal detachment, hemorrhage, exudation or Wills Eye Resident Case Series, April 2007 vitreous inflammation Clinical Findings 13 8/1/2013 OCT • Neurofibromatosis Type 1 • • • • • • Shows retinal disorganization and elevation with overlying ERM • Abilty to monitor macular changes 2° to traction Café-au-late spots Neurofibromas Lisch nodules Short stature Scoliosis B Scan • Can be used to rule out compressive lesions • May be unable to pick up depth of hamartoma • Findings not characterisitc • Neurofibromatosis Type 2 • • • • Acoustic neuromas Hearing loss Balance problems Cataracts FA • Rarely diagnostically important • Findings are characteristic Systemic Association Special Testing • Patients are monitored annually for • Growth of lesion • Vision changes secondary to macular traction • Consider annual OCT of macula • Ask pertinent history questions in regards to NF-1 and NF-2 • Refer for vitrectomy with membrane peel in cases where vision is significantly affected CHOROIDAL HEMANGIOMA Treatment & Management CIRCUMSCRIBED • Mass of vascular channels within choroid Orange/red mass with indistinct margins • Circumscribed vs. Diffuse [Sturge-Weber] Usually peripapillary • Symptoms in adulthood secondary to RD Acoustically solid lesion eyecancer.com Choroidal Hemangioma Subretinal fluid or macular edema webeye.ophth.uiowa.edu Choroidal Hemangioma 14 8/1/2013 DIFFUSE Affects over ½ the choroid Enlarges slowly Deep-red colored fundus Found exclusively in patients with Sturge-Weber Syndrome Choroidal Hemangioma Choroidal Hemangioma Dr. Aaron Gold Sturge-Weber Syndrome OCT • Acute leakage shows subretinal fluid with intact photoreceptor layer and normal retinal thickness • Chronic leakage shows loss of photoreceptors, retinoschisis, and retinal edema • Congenital hamartomatous malformations • May affect • Eye • Skin • CNS B Scan • Acoustically solid lesion • High internal reflectivity • Motor seizures – 85% • Mental retardation – 60% • Facial cutaneous venous dilation (port-wine stain) present in 96% FA • Rapid spotty hyperfluorescence in the early arterial phase • Diffuse intense late hyperfluorescence Systemic Association Diffuse • • • • • Observation Amblyopic therapy Laser photocoagulation Irradiation Retinal detachment surgery • Enucleation seconday to neovascular glaucoma Special Testing Circumscribed • Transpupillary thermotherapy • Photodynamic therapy for secondary retinal detachment CHOROIDAL OSTEOMA Treatment & Management 15 8/1/2013 • Benign ossifying tumor of the choroid • • • • • Composed of mature bone Orange-yellow lesion Typically unilateral Well-defined Usually juxtapapillary • Young females • Vision loss 2° to • Benign – may grow • • • • VA < 20/200 in 56-58% after 10 yrs Subretinal Fluid Neovascularization Photoreceptor loss telemedicine.orbis.org, eyewiki.aao.org archopht.ama-assn.org Choroidal Osteoma Clinical Findings OCT • Inner retina preserved • No systemic association B Scan • Highly reflective anterior surface • Orbital shadowing • No malignant potential FA • Irregular, diffuse mottled hyperfluorescence in the early and late phases • Neovascularization may be present. Special Testing • CNVM may develop Treatment & Management • Vascular and glial tumor of the retina • Rare and benign • No threat to vision RETINAL ASTROCYTIC HAMARTOMA • Frequently seen in tuberous sclerosis Optical Coherence Tomography of Retinal Astrocytic Hamartoma in 15 Cases C. Shields, et al., utsouthwestern.edu, pconsupersite.com Retinal Astrocytic Hamartoma 16 8/1/2013 • May be multiple or bilateral • Can be calcified • Can be • Endophytic • Vitreous • Exophytic • Subretinal Clinical Findings OCT • Inner retinal thickening and disorganization with a gradual transition to normal retina • Retinal traction (27%) • Intratumoral cysts (67%) • Adjacent edema (47%) FA • Hyperfluorescence without leakage Special Testing • Solitary lesions may be found in normal individuals • Tuberous Sclerosis • • • • 50% of patients have astrocytomas Multiple/bilateral Rare genetic disease Benign tumors grow on skin, brain, kidneys, and heart Systemic Association • Usually relatively stable • Can show progressive growth • Rare cases – exudative retinal detachment and neovascular glaucoma Treatment & Management • The most common primary, intraocular malignancy in childhood • Results from malignant transformation of primitive retinal cells before final differentiation. RETINOBLASTOMA • Seldom seen after age 3 Retinoblastoma 17 8/1/2013 Retinoblastoma – life threatening disease • Heritable • germline • • 70% of cases Pinealblastoma • Non-heritable • somatic • 30% of cases Retinoblastoma Initial Features: • Leukocoria 70 % • Strabismus 25 % • Other (VA) 5 % ~ 15 % will present with iris neovascularization Most common intraocular malignancy in children / dx=18 month 300 new cases of retinoblastoma diagnosed each year in the US Leukocoria • Retinoblastoma • Coats disease • Congenital Cataract • Coloboma • Toxocariasis • ROP • PPHV • Retinal Detachment Hyphema may also be present Retinoblastoma Retinoblastoma In 75 % of cases enucleation is performed Special Testing In 25 % of cases conservative treatment is administered Treatment & Management 18 8/1/2013 Intravenous chemoreduction (carboplatin, etoposide, vincristine) International Classification of Retinoblastoma Subconjunctival carboplatin Plaque, TTT Group A Small RB < 3 mm size Group B Larger RB > 3 mm size Plaque radiotherapy Group C Contained seeds External beam radiotherapy Group D Diffuse seeds Group E Extensive > 50% globe, NVI, Opaque media Transpupillary thermotherapy Cryotherapy Laser photocoagulation Chemoreduction W / WO EBRT Intra-arterial Chemotherapy (IAC) Retinoblastoma Treatment Recurrences = within 1st year Primary Intraocular Uveal LYMPHOID TUMORS Char DH: Clinical Ocular Oncology. 2nd ed. Philadelphia, Lippincott-Raven, 1996. okulistyka.com.pl, surgical-pathology.com Lymphoid Tumors • Proliferation of B lymphocytes • Accounts for 65% of cases of intraocular lymphoma • 90% of the time it is bilateral • Increased incidence in immunosuppressed patients Primary Intraocular Lymphoma • Symptoms = unilateral floaters, blurred vision, red eye or photophobia • Patchy yellow lesions • Secondary vitreous cells - “clumps” • Primarily affects the retina and/or optic disc Clinical Features 19 8/1/2013 • Radiotherapy – if tumor is confined to the eye • Intravitreal methotrexate • Systemic chemotherapy Rospond-Kubiak, I., Kocięcki, J. & Stopa, M. Primary uveal lymphoma effectively managed with oral chlorambucil: a case report. (2013). • Uveal lymphoma = systemic • Typically nonHodgkin B-Cell lymphoma • • • Treatment & Management • Usually unilateral • Yellow-orange lesions • Can occur as a solitary mass • Vitreous is usually clear • Secondary exudative retinal detachment T-cell lymphoma Hodgkin disease Other forms of lymphoma Primary uveal lymphoma effectively managed with oral chlorambucil: a case report Iwona Rospond-Kubiak*, Jarosław Kocięcki and Marcin Stopa Uveal Lymphoma Clinical Features • Systemic chemotherapy • Whole-eye irradiation • Plaque radiotherapy LEUKEMIA A rare ocular manifestation of non-Hodgkins’ lymphoma Michael S. Peterson, O.D. and Lori S. Kirwan, O.D. Treatment & Management 20 8/1/2013 • Abnormal proliferation of white blood cells • Ocular involvement typically seen in the acute form • Most patients have known systemic disease • Usually a sign of severe disease • Diffuse or patchy thickening of the retina and choroid • Retinal hemorrhage • Optic disc infiltration • Vitreous cells imagebank.asrs.org Leukemia http://www.nejm.org Clinical Features • Management of systemic disease • Ocular radiotherapy http://www.aao.org Treatment & Management Ocular involvement in 80% of patients choroidal nevus choroidal melanoma retinoblastoma choroidal metastasis choroidal hemangioma retinoblastoma choroidal osteoma choroidal hemangioma astrocytic hamartoma choroidal melanoma choroidal osteoma lymphoid tumor 21 8/1/2013 Kanski, J. Clinical Ophthalology: A Systemic Approach. 6th Edition. Butterworth-Heinemann. Palmar, M., Carney, M., and Combs, J. Combined Hamartomas of the Retinal Pigment Epithelium and Retina. Retina. Vol 10, 1990, pp. 33-36 Rospond-Kubiak, I., Kocięcki, J. & Stopa, M. Primary uveal lymphoma effectively managed with oral chlorambucil: a case report. (2013 The End! Say, E., et al. Optical Coherence Tomography of Retinal and Choroidal Tumors. Journal of Ophthalmology. Vol 2011, Article ID 385058, 12 pages Shields, C., Materin, M. & Shields, J. Review of optical coherence tomography for intraocular tumors. Current opinion in ophthalmology 16, 141–54 (2005). Shields, J. and Shields, C. Intraocular Tumors: An Atlas and Textbook. Second Edition. Lippincott Williams & Wilkins, 2008 Shields, J. and Shields, C. Tumors of the Retinal Pigment Epithelium. Retinal Physician. May 2005 Shields, Jerry. Ocular Oncology Essentials: Melanoma Update [based upon presentation at the 2006 Retinal Physician Symposium] Retinal Physician. Sept 2006. Sinead Fenton, Teresa Sandinha, William R Lee, and Ewan G Kemp. (2001). Massive extraocular extension as the presenting feature of a choroidal melanoma. Eye. doi:10.1038/eye.2001.175 Tasman, W and Jaegar, E. Wills Eye Hospital Atlas of Clinical Ophthalmology, 2nd Ed. Chapter 7 Retinal Tumors. References Thank you Please complete your session evaluation using EyeMAP online at http://eyemap.cistems.net Tweet about this session using the official meeting hashtag #aaoptom13 22
