Financial Disclosures. The Eye in Neoplastic Disease. Course Goal. We wish to acknowledge and thank: Tumor Definition

Transcription

1 The Eye in Neoplastic Disease Carlo J. Pelino, OD, FAAO Joseph J. Pizzimenti, OD, FAAO Financial Disclosures! Speakers have no relevant financial relationships to declare. Course Goal We wish to acknowledge and thank:! To provide useful clinical information about neoplastic disease, with an emphasis on ophthalmic complications. Mark Dunbar, OD Lauren Richards, OD Brad Sutton, OD Sherrol Reynolds, OD Tumor Definition tumor /tu!mor/ (too mer) also called neoplasm. a new growth of tissue characterized by progressive, uncontrolled proliferation of cells. A tumor may be localized or invasive, benign or malignant. It may be named for its location, its cellular makeup, or for the person who first identified it. Mosby's Medical Dictionary, 8th edition. 2009, Elsevier.

2 Assessing Skin Lesions - ABCDE Rule " A Asymmetry (symmetry is good, asymmetry is bad) " B irregular Borders (more consistent with malignancy), Bleeding " C Color (variation of color within lesion consistent with malignancy) " D Diameter (bigger is not better) " E -- Elevation (greater elevation consistent with malignancy) " Misc: look for lash loss and feeder vessels as signs of lid malignancies Neoplastic Eye Disease in the Young Capillary Hemangioma " Common, benign lid or orbital tumor " Appears as strawberry nevus if located in more external tissue or as a blue eyelid mass if deeper in orbit " Occurs in first year of life and slowly involutes by age 5 " Eyelid involvement may cause ptosis, cyl, occlusion/deprivation amblyopia Capillary Hemangioma " Management/Treatment: Orbital Capillary Hemangioma " Orbital CT scan to determine extent of involvement. " No treatment if not affecting vision or involving the globe. " Treat when lesion induces refractive error, strabismus, occlusion of vision or invasion of globe. " Pediatric Dermatology or Oculoplastics Consult " Systemic/topical B-blocker, local injection of steroids, systemic steroids, interferon, laser therapy, radiation therapy or surgical excision.

3 Dermoid Cyst " Common, benign mass lesion " Painless, palpable, flesh colored " Composed of connective tissue " Presents in early childhood and may slowly enlarge Dermoid Cyst " Management: " Orbital CT scan " Excision by oculoplastic surgeon should be removed without rupturing capsule to prevent inflammation Rhabdomyosarcoma Rhabdomyosarcoma " Most common primary pediatric orbital malignancy. " Occurs in patients < 15 years of age (usually between 7-8) Rhabdomyosarcoma " Signs and symptoms: "Very rapid onset of unilateral proptosis "Lid edema "Possible lid discoloration

4 Rhabdomyosarcoma " Management/Treatment: "CT Scan of orbit (may show bone destruction) "R/O infection (orbital cellulitis) "Biopsy "Systemic evaluation "Treatment with local radiotherapy and/or systemic chemotherapy Retinoblastoma # Most common intraocular malignancy in childhood # 90% are diagnosed before 3 yrs old # 6% have + family history # 40% of all new RB cases have inheritable gene mutation # Bilateral RB is an inheritable form # Autosomal dominant (80% penetrance) # Leukocoria (61%) # Whitish-gray tumor # Chalky calcification # Necrosis # Retinal detachment # Normal globe size Clinical Signs of Retinoblastoma # Multiple or solitary tumor(s) $ Exophytic vs endophytic vs intraretinal Retinoblastoma $ Exophytic tumors extend beneath the sensory retina, while endophytic grow forward into vitreous # Total exudative (serous) RD # Invade choroid, optic nerve --> sub-arachnoid space --> brain # 1% spontaneous regression $ Phthisis bulbi

5 Retinoblastoma Retinoblastoma # Non-hereditary (Somatic): 60-70% $ Rb1 gene occurs in a single retinal cell $ Unilateral $ No increased risk for cancers elsewhere # Hereditary (Germline) 30-40% $ Due to sporadic germline mutations $ Autosomal dominant Retinoblastoma Retinoblastoma Treatment is Individulaized to the Patient # Enucleation- no hope of preserving vision # External beam radiation # Plaque brachytherapy # Chemotherapy/Chemoreduction # Chemothermotherapy # Cryo, Laser # Combination therapy RB: Systemic Work Up Coats Disease #CT scan (follow up MRI) #LP w/csf studies if ON involvement #Bone marrow aspiration if choroidal or orbital involvement

6 Coats Disease Coats Disease # AKA Exudative Retinitis # Progressive condition of retinal capillaries which occurs in children and young adults, usually males # Typically presents in first decade of life, gradual in progress, affects central vision, usually unilateral # Idiophatic disorder characterized by abnormal development of retinal vessels (telangiectasia) # Progressive deposition of intraretinal or subretinal exudates, potentially leading to exudative retinal detachment. Coats Disease Coats Disease # Treatment is directed toward closure of abnormal leaking vessels to facilitate resolution of exudation and retinal detachment. # Available treatments include laser and cryotherapy in the early stages. # More advanced cases require surgical techniques for RD, such as scleral buckling, pars plana vitrectomy and removal of vitreous membrane. # Stabilization of the disease course or clinical improvement is achieved in 70% of cases. Adult Tumors " External Adnexa: " Tan or darkly pigmented skin lesion " Irregular borders and varying pigmentation within lesion "May be flat or nodular

7 Gene Mutations in " Management/External: " Systemic evaluation for metastasis " Excision with wide margins Choroidal Nevus Nevus! Symptoms: " Most asymptomatic " Typically flat or mildly elevated " Secondary drusen and reactivity of the RPE common

8 Nevus Ocular! Predictive Factors for Growth: Thickness Location Symptoms: flashes/floaters/blurred vision Orange pigment CNVM (rare) Sub-retinal fluid (can also occur with nevi) " May be external or intraocular " Intraocular: Most common primary intraocular tumor in adults " External: Most lethal primary skin tumor-but rare on eyelids " Highly malignant and metastatic. " Metastasis is most commonly to the liver. Also to lung, brain, skin and GI tract. " Almost always unilateral " More common in light skinned patients Orange Pigment = Lipofuscin Many patients with choroidal melanoma have no symptoms. Their tumors are found during a "routine" eye examination. Choroidal melanoma has an annual incidence of six cases per million people per year. Amelanotic Choroidal Other than having blue or green eyes and a light complexion, studies have not identified any definite risk factors or exposures that predispose patients to developing this cancer.

9 " Intraocular: "Elevated, round mass in the choroid but protruding under or through the sensory retina. " Usually darkly pigmented, but may be amelanotic. To Find Small Ocular Symptoms: " Most are asymptomatic " Blurred vision "Scotoma or loss of visual field "Photopsia NEVOMA To Find Small Ocular Using Helpful Hints Daily T= thickness (>2mm) F= subretinal fluid S= symptoms O= orange pigment M= margin touches disk No risk factors (<4%) 1 risk factor (36%) 3 risk factors (50%) 5 risk factors (70%).9:!;<=><.&?@9A>'&B&;<C=D&<E<@F>'G=?!"#$%&'()*+,)&'#$-"&./#)01&!)-2/"3,$4&53))367& 5/)3&/8"($-7&42,"($&/8"($-!"##$%&'()#&('*+,-)(."/*&(*0-")#*1"#)(23) Ophthalmology , DOI: ( /j.ophtha )

10 Fundus Autofluorescence (FAF) on Choroidal Mass Fundus Autofluorescence (FAF) in Choroidal Echography of Choroidal - acoustic hollowness Echography B-Scan Echogram Assess topographic features, including tumor shape, surface contour and boundaries A-Scan Echogram Internal structure, reflectivity, tumor height (elevation)! Acoustic hollowness on B-scan of small melanoma. Echography of large melanoma Uveal melanoma is the second-most common form of melanoma and the most common primary intraocular malignancy. Up to one-half of patients are at risk for fatal metastatic disease. 7/29/2016

11 Variable Presentation of Choroidal Treatment of Choroidal! Observation indicated in elderly/infirm, lots of mets and very poor prognosis small melanomas! Enucleate, exenterate: large melanomas with secondary complications. Treatment depends on 45*/6)6"*27*268"9*":"***;5*#2.)<2( Treatment of Choroidal! Radiotherapy with plaque or external proton beam! Transcleral Resection! Multiple Treatment Modalities: Local resection + Plaque Rx + Photocoagulation " Management/Intraocular " Refer for retinal evaluation/ocular oncology " Small tumors (<10 mm diameter and <3 mm height) may be monitored carefully " Medium tumors (10-16 mm diameter and 3-10 mm height) may treat with laser photocoagulation, external proton beam or episcleral plaque irradiation " Large tumors (>16 mm diameter and >10 mm height): enucleation " Evaluate for metastasis Treatment of Ocular

12 Treatment Side Effects Choroidal melanomapre-radiotherapy! Main side effect of focal ocular treatment is! Radiation retinopathy!! NVD / NVE! Exudative changes! Macular edema! Occurs several weeks to months after therapy pre-tx echography (left), post-radiotherapy (right) Radiation Retinopathy: exudate, NVD Choroidal Acknowledgement: Sherrol Reynolds, OD, FAAO S/P Radiotherapy

13 Management of RR! Avastin/Lucentis/Eylea! Laser! Silicone oil at time of Brachytherapy attenuates radiation dose, and may protect against radiation retinopathy Metastatic Tumors! 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! The most common location is the lung, bone, lymph nodes and liver 8EDFGGHHH5()<2()#I9")/6.)(."9529'G3"6)/6)<.$I9")/6$.)(."9 Choroidal Choroidal Metastasis From Breast!"#$%&'()*()*+* Choroidal Metastasis

14 METS Multiple cream colored lesions in posterior pole Large lesion with neurosensory RD Metastatic Prostate Cancer Early IVFA OS Late IVFA! Note blocking of the background hyperflouresence in multiple areas including large central lesion! Note late staining of large central lesion Summary/Conclusions! and other ocular malignancies may be deadly. With early detection, you could save a life.! Be your patient s advocate: make sure they see someone who can help them and give expert information.! Several excellent sources of online patient information are the CURE OM, the Ocular Foundation, and the Eye Cancer Network. We wish to acknowledge and thank: Mark Dunbar, OD Lauren Richards, OD Brad Sutton, OD Sherrol Reynolds, OD

15 Thank You! Carlo and Joe