ACTIVATED OR NOT? RETINAL CASE PRESENTATION Shorye Payne, MD Medical Retinal Specialist Robley Rex VA Eye Clinic

Transcription

1 ACTIVATED OR NOT? RETINAL CASE PRESENTATION Shorye Payne, MD Medical Retinal Specialist Robley Rex VA Eye Clinic

2 C We anticipate that the future management of posterior uveal melanoma (PUM) will focus on detection of clinical and imaging clues for the earliest diagnosis, prompt local tumor treatment, and systemic targeted therapies for microscopic metastasis or prevention of metastasis. Shields, JA, Shields, CA. Management of posterior uveal melanoma: past, present, and future. The Charles L. Schepens Lecture. Ophthalmology 2015; 122;:

3 INDETERMINATE LESIONS Five factors associated with risk of growth of small choroidal melanocytic lesions less than 3 mm in thickness 1) tumor thickness greater than 2.0 mm 2) sub-retinal fluid 3) visual symptoms 4) orange pigment 5) posterior tumor margin touching the disc Present a challenge with regard to diagnosis and management Shields CL, Shields JA, Kiratli H, De Potter P, Cater JR. Risk factors for growth and metastasis of small choroidal melanocytic lesions. Ophthalmology 1995;102:

4 FACTORS PREDICTIVE OF GROWTH AND TREATMENT OF SMALL CHOROIDAL MELANOMAS Review of 188 small lesions 1-3 mm thick and 5-16 mm largest basal diameter 1) greater apical tumor thickness 2) larger initial basal diameter 3) presence of orange pigment 4) absence of drusen 5) absence of retinal pigment epithelial change adjacent to the tumor COMS report No. 5. Arch Ophthalmol. 1997;115 (12):

5 To Find Small Ocular Melanoma Using Helpful Hints Daily Features Hazard Ratio Tumor Growth If Present (%) Thickness > 2.0 mm Fluid Symptoms Orange pigment Margin (posterior tumor) < 3 mm to disc Ultrasound Hollowness Halo absent Drusen na na na Tumor Growth If Absent(%) Adapted from Shields, CL, Furuta M, Berman, EL, et al. Choroidal nevus transformation into melanoma. Analysis of 2514 consecutive cases. Arch of Ophthalmol 2009; 127;

6 A. Suspicious choroidal nevus with overlying orange pigment B. Showed basal growth in basal dimensions during 6 yrs E. Suspicious choroidal nevus with overlying orange pigment and subtle subretinal fluid F. Showed enlargement during 2 yrs Adapted from Shields, CL, Furuta M, Berman, EL, et al. Choroidal nevus transformation into melanoma. Analysis of 2514 consecutive cases. Arch of Ophthalmol 2009; 127; 981-7

7 E. Halo choroidal nevus F. Remained stable at 3 yr f/u G. Choroidal nevus with subtle drusen and RPE atrophy H. Remained stable at 23 yr f/u Adapted from Shields, CL, Furuta M, Berman, EL, et al. Choroidal nevus transformation into melanoma. Analysis of 2514 consecutive cases. Arch of Ophthalmol 2009; 127; 981-7

8 . The purpose of this review is to look at two patients who presented to the VA Eye clinic with suspicious choroidal nevi and to allow the 2 imaging modalities of autofluorescence and OCT to help characterize the activity status of the nevus

9 AUTOFLUORESCENCE ADDED VALUE The RPE is known to be an important tissue in the assessment of choroidal melanoma. It can manifest hyperplasia, atrophy, fibrous metaplasia, and osseous metaplasia with long-standing tumors It can display intracellular lipofuscin accumulation with more active tumors as seen as orange pigment clinically one of the 7 RF predictive of tumor growth. Normal choroid emit minimal to no autofluorescence. Choroidal melanoma emit little to no autofluorescence. Lipofuscin remnants in RPE show bright autofluorescence. SRF show slightly increased autofluorescence. Detection of subclinical lipofuscin (orange pigment) by fundus autofluorescence imaging could play a role in the early detection of choroidal melanoma. Shields CL, Bianciotto C, Pirondini C, Materin MA Harmon SA, Shields JA. Autofluorescence of orange pigment overlying small choroidal melanoma. Retina 2007;27 (8)

10 SUBRETINAL FLUID FACTOR SRF is a strong risk factor for tumor growth It is difficult to assess clinically SRF presents 2 challenges: 1. Does the SRF represent chronic retinal changes over a dormant lesion? 2. Does the SRF represent active retinal changes that s a RF for tumor growth? SRF Inactive Active Overlying Retina Chronic changes, like retinal thinning and intraretinal cyst Elevated, but normal retina Interventional case series of 33 cases demonstrated a correlation between an active subretinal fluid on OCT and documented tumor growth. Espinoza G, Rosenblatt B, Harbour W. Optical coherence tomography in the evaluation of retinal changes associated with suspicious choroidal melanocytic tumors. Am J Ophthalmol 2004;137:90-95.

11 OPTICAL COHERENCE TOMOGRAPHY ADDED VALUE OCT is useful in distinguishing active subretinal fluid from chronic retinal changes overlying a choroidal melanocytic tumor. OCT findings may have predictive value in identifying tumors that are likely to grow and require treatment. Fig. A-E choroidal lesion with Active OCT pattern showing SRF with retinal separation & normal retina appearance. Espinoza G, Rosenblatt B, Harbour W. Optical coherence tomography in the evaluation of retinal changes associated with suspicious choroidal melanocytic tumors. Am J Ophthalmol 2004;137:90-95.

12 HISTORY ACTIVATED OR NOT... C.P. Patient 1 G.F. Patient 2 CC: Want new glasses. CC: Double vision when looking at the moon. HPI: 72 y/o Caucasian male presented for 6 mth retinal f/u for choroidal nevus surveillance. Nevus had been present since July At the time of detection, patient recalls being told presence of assoc. bleeding with lesion. Since detection, vision has remained unchanged. HPI: 55 y/o Caucasian male presented for 12 mth retinal f/u for pigmented choroidal lesion possible peripapillary nevus surveillance. Lesion had been followed since 2010 with low suspicion for choroidal melanoma.

13 HISTORY C.P. Patient 1 G.F. Patient 2 POH: Choroidal nevus OS POH: Choroidal Nevus OD Cataracts OU Dry Eyes PMH: A fib, HTN, hyperlipidemia, PMH: HTN, hyperlipidemia, Gout, osteoarthritis Sleep apnea, asthma FH: Unknown FH: Unknown ROS: Negative ROS: Negative

14 EXAM C.P. Patient 1 2/20/14 G.F. Patient 2 5/29/14 bcva: OD 20/20 OS 20/40-2 bcva: OD 20/30 OS 20/20 Pupils: OD dilated OS dilated Pupils: OD dilated OS dilated IOP: OS 12 OS 12 IOP: OS 15 OS 17 A.S.: OD 1+ NS OS 1+ NS A.S.: OD tr NS OS tr NS B scan: ~1.0 mm not elevated B scan: 2.5 mm Ht 8.7 mm width mm mm mm

15 C.P. Patient 1 Color Fundus Photo and Autofluorescence Grayish brown and partially amelanotic lesion with spotty brown colored lipofuscin on amelanotic portion measuring 7 mm V x 5 mm H basal diameter extending 0.4 mm from disc and 1.0 mm from fovea FAF wide view - Bright lacy increased autofluorescence over lesion and more diffuse macula involvement. Compliments of James J. Augsburger, MD Univ. of Cincinnati Physicians

16 C.P. Patient 1 Autofluorescence and FA Comparison Linear increased autofluorescence of lipofuscin with mildly increased autofluorescence of macular SRF FA late venous ~40 sec with linear hypofluorescent blocking of lipofuscin

17 C.P. Patient 1 Digital Imaging FA Recirculation 1:39 and 3:12 Mild diffuse macular hyperfluorescence suggesting NSD; suspicious superior disc v. Mild pinpoint hyperfluorescent leakage

18 C.P. Patient 1 SD OCT Central Lesion Approximation Lipofuscin seen as irregularity at the level of RPE; mild RPE hyperplasia; and elevation of the choroidal lesion

19 C.P. Patient 1 SD OCT Superior Macula 2/2014 and 10/2014 Mild SRF; normal RPE, good retinal integrity Reduced SRF; normal RPE, good retinal integrity

20 C.P. Patient 1 SD OCT Fovea 2/2014 and 10/2014 Shallow SRF at fovea; nasal RPE thickening/irregularity of lipofuscin near disc Reduced but temporal extension of SRF involving fovea; nasal RPE thickening/irregularity near disc

21 C.P. Patient 1 SD OCT Inferior Macula 2/2014 and 10/2014 Mod SRF; normal RPE, good retinal integrity Mostly resolved SRF

22 G.F. Patient 2 Color Fundus Photo and Autofluorescence COMPLIMENTS OF JAMES AUGSBURGER, MD Juxtapapillary mostly pigmented choroidal lesion with possible central fibrosis. Focal increased autofluorescence mildly over lesion and moderately surrounding lesion border with lesion itself isoautofluorescent.

23 G.F. Patient 2 Digital FA Early Venous and Autofluorescence Hypofluorescent blockage of lesion Slight pinpoint increased autofluorescence over lesion and mildly increased diffuse hyperfluorescence at nasal border.

24 G.F. Patient 2 Digital Imaging FA/ICG Recirculation ~ 2.25 min Spotty hyperfluorescent leakage

25 G.F. Patient 2 SD OCT Central Lesion Approximation Lost of retinal integrity with large intraretinal cyst/pseudo SRF, retinal thinning RPE thinning and irregularity

26 Intraretinal cyst, retinal thinning, RPE thinning suggesting chronic /pseudofluid but nasal border appear more cystoid macular edema G.F. Patient 2 SD OCT Fovea Superior border thicken NFL near disc; intraretinal cyst, RPE thinning, trace SRF

27 B SCAN COMPLIMENTS OF JAMES AUGSBURGER, MD C.P. Patient 1 G.F. Patient mm maximal thickness Relatively low internal reflectivity 2.0 mm maximal thickness Relatively low to mod internal reflectivity

28 RISK FACTORS C.P. Patient 1 G.F. Patient 2 Orange Pigment Subretinal Fluid Margin < 3 mm to disc Orange Pigment Subretinal Fluid Margin < 3 mm to disc Thickness 1.3 mm final B scan No visual symptoms Thickness 2.0 mm final B scan Subjective monocular diplopia

29 DIAGNOSIS C.P. Patient 1 G.F. Patient 2 Activated Choroidal Nevus -versus- Small Choroidal Melanoma Activated Choroidal Nevus -versus- Small Choroidal Melanoma Treatment Options If melanoma treat with plaque XRT or proton beam irradiation If nevus continue monitoring Diagnostic FNAB and subsequent management based on cytopathology Treatment Options If melanoma treat with plaque XRT or proton beam irradiation If nevus continue monitoring Diagnostic FNAB and subsequent management based on cytopathology

30 DIAGNOSIS C.P. Patient 1 G.F. Patient 2 Activated Choroidal Nevus -versus- Small Choroidal Melanoma Activated Choroidal Nevus -versus- Small Choroidal Melanoma Treatment Choice Observation with continued monitoring Treatment Choice Diagnostic FNAB and subsequent management based on cytopathology

31 G.F. Patient 2 COMPLIMENTS OF JAMES J. AUGSBURGER, MD Cytopathological analysis of the aspirates confirmed spindle cell B type uveal melanoma H&E

32 G.F. Patient 2 COMPLIMENTS OF JAMES J. AUGSBURGER, MD Notched I-125 Plaque Radiotherapy

33 SURVEILLANCE FOR SUSPICIOUS ACTIVATED NEVI To Find Small Ocular Melanoma Using Helpful Hints Daily 0 features should be initially monitored twice yearly and followed up annually thereafter if their condition is stable 1 or 2 features should be monitored every 4 to 6 months. 3 or more features should be evaluated at an experienced center for management alternatives and possible treatment owing to the high risk of ultimate growth. Shields, CL, Furuta M, Berman, EL, et al. Choroidal nevus transformation into melanoma. Analysis of 2514 consecutive cases. Arch of Ophthalmol 2009; 127;

34 SPECIAL THANKS VA EYE TECHNICIANS JINGHUA CHEN, MD, PHD UNIV. OF LOUISVILLE OPHTHALMOLOGY RESIDENT JAMES J. AUGSBURGER, MD OCULAR ONCOLOGIST UNIVERSITY OF CINCINNATI EYE PHYSICIANS GROUP