Pigmented Lesions Flashcards
(198 cards)
1
Q
Definition of Hypertrophy
A
Increase in size of a cell
2
Q
Etiology of CHRPE
A
Idiopathic, congenital
3
Q
CHRPE: malignant or benign?
A
BENIGN
4
Q
CHRPE: symptoms
A
Usually asymptomatic
- Can cause VF defects (due to atrophy of PRs)
- Can cause VL (if located on fovea — but very, very rare)
5
Q
CHRPE is most commonly found in which quadrant?
A
Temporal
6
Q
TRUE/FALSE: In CHRPE, because the RPE cell is enlarged, it maintains the same amount of melanin thus the melanin appears to be less
A
FALSE; CHRPE are enlarged cells with large amounts of melanin
7
Q
Explain how an hypertrophy of an RPE cell can cause degeneration of a PR
A
Hypertrophy impairs RPE cells Phagocytic ability, leading to degeneration of PR
8
Q
Describe the typical SHAPE of a CHRPE
A
Flat, round, with distinct margins
9
Q
TRUE/FALSE: CHRPE don’t normally change in size
A
TRUE
10
Q
TRUE/FALSE: CHRPE is common in the posterior pole
A
FALSE
11
Q
CHRPE: typically unilateral or bilateral?
A
Unilateral
12
Q
CHRPE: what a lacunae?
A
Window view (within the CHRPE) of the underlying choroid and sclera
13
Q
TRUE/FALSE: lacunae is an example of chorioretinal atrophy
A
TRUE
14
Q
Describe the typical COLOR of a CHRPE
A
Light brown to jet black
(Potentially with white spots, “lacunae”)
15
Q
Describe the typical SIZE of a CHRPE
A
2-6 mm
16
Q
What are “bear tracks”?
A
Multiple CHRPE in one sector of fundus, with up to 30 lesions in each group
17
Q
For Bear Tracks, larger lesions are usually more _____ (posterior/peripheral), where as smaller lesions are usually more ____ (posterior/peripheral).
A
Large — peripheral
Smaller — posterior
18
Q
TRUE/FALSE: CHRPE are not associated with Gardner’s Syndrome
A
TRUE;
CHRPE is not associated with Gardner’s
19
Q
TX for CHRPE
A
Just monitor :)
20
Q
A
CHRPE
21
Q
How does CHRPE appear on Fundus Autofluorescence?
A
CHRPE itself will be hypopigmented but lacunae may be hyperpigmented
22
Q
What is Gardner Syndrome?
A
Familial Adenomatous Polyposis + extracolonic manifestations (e.g. ocular)
23
Q
What is Familial Adenomatous Polyposis?
A
AD genetic disorder, associated with colon polyps/cancer
24
Q
What is the prognosis of Familial Adenomatous Polyposis (FAP)?
A
100% malignancy, if left untreated
25
TRUE/FALSE: Familial Adenomatous Polyposis can occur without any family history
TRUE;
20% of cases — *spontaneous mutation of gene*
26
FAP makes up ____% of all colon cancer in US annually
1%
27
What ocular lesion is a specific and reliable marker for FAP?
And how prevalent is the lesion in pts with FAP?
“CHRPE-like” retinal hamartomas
Present in 70% of FAP genotypes
28
Management for “CHRPE-like” Retinal Hamartoma
Genetic testing and GI consult
29
Describe the appearance of the lesion associated with FAP/Gardner Syndrome (5)
1. Contains depigmented halo, pointing toward ON
2. Multiple lesions (no sectoral organization)
3. Usually small (< 0.1 DD)
4. Oval/Pisciform/Football
5. Less well-defined relative to CHRPE
30
Is the lesion associated with FAP/Gardner’s usually unilateral or bilateral?
Usually bilateral
31
Is the lesion associated with FAP/Gardner’s usually found more posteriorly or more peripherally?
Peripheral, esp near vortex veins
32
TRUE/FALSE: The ocular lesion associated with FAP has a higher sensitivity than specificity.
FALSE;
Specificity — 78% (absence of lesion does not rule out FAP)
Sensitivity — 95% (presence of lesion highly predictive of FAP)
33
Definition of Hyperplasia
Increase in **number** of cells
34
What does the RPE have a propensity to do, in response to ocular insullt?
Proliferate and migrate
35
What are some common stimuli to RPE Hyperplasia?
Inflammation and trauma
36
RPE Hyperplasia usually presents as _____
Scars or pigment clumps
37
RPE Hyperplasia:
Pigment clumping helps to identify localized regions of ________ that could produce a _______
Regions of **vitreoretinal adhesion** that could produce **retinal tears**
38
Describe the appearance of RPE Hyperplasia
1. Color: Jet-black
2. Irregular
3. Often stellate margins
4. May have fibrosis/gliosis
5. Almost always has sensory retinal degeneration (white appearance)
39
In RPE Hyperplasia, the RPE cells migrate to the ______ (anterior/posterior) retina
ANTERIOR
40
Management for RPE Hyperplasia
Observation
41
Melanocytomas are usually _______ (unilateral/bilateral)
Unilateral
42
Location of Melanocytoma
Eccentric to Optic Nerve
43
Racial p revalence of Melanocytoma
No racial preference
44
Melanocytes is composed of what type of cells?
Darkly-pigmented, plump, **polyhedral cells**
45
Melanocytoma: benign or malignant?
Benign
*Malignant transformation is rare (1-2% of cases)
46
Describe the appearance of a Melanocytoma (4)
1. Dark brown
2. Elevated
3. Usually does not exceed 1 DD
4. Indistinct (sometimes “feathered”) margins
47
What appearance in a Melanocytoma is indicative of extension into the NFL?
Feathered margins
48
Symptoms of Melanocytoma
*Usually* asymptomatic
But vision loss can occur due to…
1. Exudation into the macula
2. Compression of axons in ON head
3. Spontaneous necrosis of tumor
4. Pushes on CRV, causing occlusion
49
T/F: Melanocytoma can produce an APD
TRUE; affects ON
50
What would you expect to see on a Visual Field of a patient that has a Melanocytoma?
Enlarged blind spot and extensive NFL defects
51
What choroidal association would you find with a Melanocytoma?
Peripapillary choroidal nexus
52
T/F: growth is common in melanocytomas
TRUE
53
What is an indication of Melanocytoma malignancy?
Progressive growth with visual loss
54
Management of Malignant-transformed Melanocytoma
May have to consider enucleation
55
What is the most common intraocular tumor?
Choroidal nevus
56
Prevalence of Choroidal Nevi
~10% of population
57
Choroidal nevi are precursors for ____
Choroidal melanoma
58
Annual rate of choroidal nevus malignant transformation ______ (increases/decreases) with age
INCREASES
59
Rate at which choroidal nevi tranforms into a malignancy
1 in 8,845
60
Signs of Choroidal Nevus Chronicity (6)
1. Overlying drusen
2. Overlying PED
3. Chorioretinal Atrophy
4. RPE Hypertrophy
5. Fibrous metaplasia
6. RPE Trough
61
What is the best imaging to visualize and differentiate choroidal nevus from choroidal melanoma?
Enhanced Depth Imaging (EDI)
62
OCT tends to _______ (underestimate/overestimate) thickness of lesion by ___%
Underestimate by 50%
63
On OCT, any nevus larger than _____ mm is suspicious of melanoma
Larger than 1 mm
64
“Shaggy Receptors” are commonly seen in what type of lesion?
Melanomas
65
What risk factor is pathognomonic for choroidal melanoma?
NONE
66
What is the mnemonic for suspecting a Melanoma?
To Find Small Ocular Melanoma Doing Imaging (TFSOMDIm)
Thickness > 2 mm
Fluid (+)
Symptomatic
Orange pigment
Melanoma Acoustic Hollow
Diameter > 5 mm
67
Which risk factors have the 3 *highest* Hazard Ratios for Choroidal Melanoma?
1. Thickness > 2 mm
2. Fluid
3. Orange Pigment
68
How many choroidal nevi are halo nevi?
5%
69
Describe a Halo Nevus
Typical nevus, surrounded by irregular depigmented chorioretinal halo
70
Halo nevi are associated with *previous* diagnoses of…
Cutaneous melanoma
71
Halo nevi may represent…
An autoimmune reaction
72
T/F: Halo around nevi indicates growth
FALSE; it indicates **stability**
73
How to Manage Choroidal Nevus
1. Photo documentation (color photos, FAF, and OCT at *every* visit)
2. Use TFSOM criteria:
- No risk factors —> monitor
- 1 RF —> q6m-1yr
- Growth or 2+ RF —> consider treatment
74
Racial Melanosis is commonly found where?
Conjunctiva, near limbus
75
Racial melanosis ____ (increase/decrease) by age
INCREASE
76
T/F: Racial Melanosis increases risk of Melanoma
FALSE
77
Racial Melanosis is typically _____ (unilateral/bilateral)
Bilateral
78
**Primary Acquired Melanosis**:
- onset?
- unilateral vs bilateral?
- likelihood to transform to conj melanoma?
- Adult onset
- Unilateral
- 20%
79
Treatment Options for Choroidal Melanoma
1. Enucleation
2. Brachytherapy
3. Proton Laser Therapy
80
____% of Choroidal melanomas will demonstrate extra-scleral extension
3%
81
Ciliary Body Melanomas usually spread via
Scleral channels
“Sentinel vessels”
82
Posterior Melanomas spread via…
vortex veins and posterior ciliary arteries
83
Top Ten Pseudomelanomas (DDX)
1. Nevus
2. PERCH
3. CHRPE
4. Hemorrhagic Retinal or RPE detachment
5. Choroidal hemangioma
6. AMD
7. RPE Hyperplasia
8. Optic Disc Melanocytoma
9. Choroidal Metastasis
10. Hemorrhagic Choroidal Detachment
84
Most common location of choroidal melanoma metastasis
LIVER
also Lungs and Brain
85
RF for Metastasis of Choroidal Melanoma
Tumor touching ON margin or histopathologic, cytogenic, and genetic factors
86
About 25% of pts w/ uveal melanoma have ______ 5 yrs after treatment
Metastasis
87
What is the metastatic work up necessary for all melanoma patients?
1. Liver function test
2. Liver imaging
3. Chest x-ray
*annually*
88
CHRPE w/ lacunae
89
Bear Tracks (CHRPE)
90
Halo Choroidal Nevus
91
RPE Hyperplasia
92
CHRPE w/ lacunae
93
Retinal Hamartoma (as related to FAP)
94
RPE Hyperplasia
95
Nevus
*Note: No SRF*
96
Nevus
*Note: Drusen/PED*
97
Melanoma
*Note: Shaggy PRs and SRF*
98
Melanoma
*Note: Shaggy PR and SRF*
99
Melanoma
Note: Lipofuscin, SRF, and shaggy PRs
100
Nevus
*Note: Although PRs are irregular, fully attached to RPE (no PED)*
101
Nevus :)
102
Melanoma /:
*Note: Shaggy PR + SRF + minimal shadowing*
103
Nevus :D
*Note: Drusen + DENSE shadowing*
104
Melanocytoma of ON
*Note: the lesion extends intraocularly and outward, into the optic disc*
105
RPE Hyperplasia
106
Halo Choroidal Nevus
107
Melanocytoma of ON
108
RPE Hyperplasia
109
Melanocytoma w/ Peripapillary Choroidal Nevus
110
Retinal Hamartoma, as assc with FAP
111
What is the color of a Choroidal Nevus?
Gray-Brown
112
Can Choroidal Nevi be *amelanotic*?
YES
113
Margins of a nevus?
**INDISTINCT**
114
Elevation of Nevus?
Flat (*or just minimally elevated*)
115
Which clinical factor is pathognomonic for whether a melanocytic lesion is benign or malignant?
**NOTHING!**
116
Virtually all lesions < _____ mm thick are benign nevi
1 mm
117
Virtually all melanocytic lesions > ___ mm thick *are* melanomas
> 3 mm
118
A flat melanocytic lesion less than ____ mm in diameter is almost always benign
10 mm
119
What color pigment is indicative of a melanoma (instead of a nevus)?
ORANGE
120
Melanoma
*Note: orange pigment*
121
Melanoma
*Note: orange pigment*
122
Melanoma: orange pigment substance
Lipofuscin
123
Melanoma: why is orange pigment worrisome?
Indicates the the lesion is **actively** growing ‼️
124
Nevus or Melanoma: presence of drusen
Likely nevus (indicates chronicity)
125
Nevus or Melanoma: absence of drusen
Choroidal melanoma
126
Choroidal nevus with drusen
127
Choroidal nevus with drusen
128
Nevus or melanoma: absence of SRF
Likely nevus
129
Nevus or melanoma: presence of SRF
Likely melanoma
130
Nevus or melanoma: location near ONH
Likely melanoma
131
Choroidal melanoma
132
Drs. Shields Mnemonic and what does it represent
To Find Small Ocular Melanomas, Use Helpful Hints Daily
**T**hickness > 2 mm
**F**luid (+)
**S**ymptomatic
**O**range pigment
**M**argin touching ONH
**U**ltrasound **H**ollowness
**H**alo *Absence*
**D**rusen *Absence*
133
What symptoms are worrisome when suspecting a choroidal melanoma?
1. Metamorphopsia
2. Photopsia
3. VF Loss
134
What is the incidence rate of a choroidal/ciliary body melanoma?
1 in 6-7 million
135
Which is more common: cutaneous or intraocular melanoma? By how much?
**CUTANEOUS** (by 20x)
136
Peak age for choroidal/ciliary body melanoma
55-65
137
For choroidal/ciliary body melanoma, aside from the peak age, there is a smaller secondary peak age of:
20-40
138
For choroidal/ciliary body melanoma, what is the sex preference for the **main** peak age?
M = F
139
For choroidal/ciliary body melanoma, what is the sex preference for the **smaller/secondary** peak age?
F > M
140
Does intraocular melanoma occur in children?
Yes, but rarely
141
For intraocular melanoma, which age group has a better prognosis?
Kids have a better prognosis :)
142
Risk Factors for Choroidal/Ciliary Body Melanoma
1. Oculodermal melanocytosis (aka Nevus of Ota)
2. Light irides
3. Cigarette smoking
4. “Northern European” ethnicity (wypipo)
143
How does *dermal melanocytosis* present clinically?
Brown, gray, or blue pigmentation
144
How does *ocular melanocytosis* present clinically?
Slate-gray patches of episcleral pigment
145
In addition to choroidal melanoma, ocular melanocytosis has an increased risk of…
Glaucoma
*10% of pts with Nevus of Ota will develop GLC*
146
An example of oculodermal (ocular + dermal) melanocytosis
147
What is racial predilection of oculodermal melanocytosis?
Hispanic, African, and/or Asian
*pigmented people*
148
T/F: The presence of ocular melanocytosis in *African Americans* increases risk of melanoma
FALSE; normal in pigmented population
*worrisome in Caucasians (1 in 400)*
149
Is sun exposure a risk factor for choroidal melanoma?
*Maybe* (lacking data to confirm)
150
What is the acronym for the major clinical trial that addressed management of intraocular melanoma?
What does it stand for?
**COMS**:
Collaborative Ocular Melanoma Study
151
In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for *small* tumors?
Observational
152
In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for *medium* tumors?
Randomized Controlled Study
153
In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for *large* tumors?
Randomized Controlled Study
154
In the major clinical trial that addressed management of intraocular melanoma, how many *small* tumors were enrolled?
~200 (least)
155
In the major clinical trial that addressed management of intraocular melanoma, how many *medium* tumors were enrolled?
~1300 (most)
156
In the major clinical trial that addressed management of intraocular melanoma, how many *large* tumors were enrolled?
~1000
157
In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for *small* tumors?
Treatment vs. Observation
158
In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for *medium* tumors?
Enucleation vs. Plaque Therapy
159
In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for *large* tumors?
Enucleation w/ XBRT or Enucleation w/o XBRT
160
In the major clinical trial that addressed management of intraocular melanoma, did pre-op XBRT improve survival?
No /:
161
In the major clinical trial that addressed management of intraocular melanoma, did treatment modality affect survival for *medium* tumors?
Nope
162
In the major clinical trial that addressed management of intraocular melanoma, for *small* tumors, was the outcome better to treat or observe?
Not enough enrollment in ‘Treatment’ arm to compare
163
In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within *1 year*.
10%
164
In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within *5 year*.
20%
165
In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within *10 years*.
30%
166
Clinical Testing for Melanoma
1. Indirect Ophthalmoscopy
2. Gonioscopy
3. Transillumination
4. Fluorescein angiography
167
What is the *classic* FA pattern for a choroidal melanoma?
Late hyperfluorescence
*double circulation*
168
If a malignant lesion is noted in the choroid, how can you differentiate between a choroidal melanoma and metastatic choroidal tumor (on FA)?
Melanoma will have its own internal circulation —>
“Double circulation” or Late hyperfluorescence
169
The double circulation pattern is more evident under: FA or ICGA?
ICGA!
170
What is the #1 Ancillary study for Melanoma?
Ultrasound (both A & B scans)
171
T/F: CT/MRI scans are suggested for all pts that have a choroidal melanoma
Not really widely used
172
T/F: An OCT does not provide a great image that allows for differentiation of a melanoma
Eh, true and false —
SD-OCT does not penetrate enough, but
**Enhanced Depth OCT (EDI-OCT)** does
173
Melanoma: Explain the Callendar Classification System
Spindle A cellls —> spindle cell **nevus** — best prognosis
Spindle A + B cells —> spindle cell melanoma
Spindle + Epithelioid cells —> mixed melanoma
Epithelioid cells —> Epithelioid melanoma —worst prognosis
174
175
What percent of ocular melanomas have *demonstrable* metastasis at time of diagnosis?
2%
176
What percent of ocular melanomas have *occult* (hidden/imperceivable) metastasis at time of diagnosis?
Unknown, but higher than 2%
177
In terms of melanoma, COMS found that ____% of patients harbored a second malignancy
10%
178
Choroidal melanoma: what are some general pre-treatment tests?
1. Complete physical exam
2. Chest X-Ray
3. Liver Function Test
4. CT/MRI (not as much tbh)
179
In what cases should the appropriate treatment for the melanoma be *observation*?
1. Tumor < 1 mm thick
2. Pt unable to tolerate treatment
180
In what cases should the appropriate treatment for the melanoma be *enucleation *?
For *many medium tumors, but **all** large tumors
181
What are 3 radiation modalities employed for melanomas?
1. XBRT (External Beam Radiotherapy)
2. Plaque Therapy
3. Charged-particle
182
What is the main benefit of XBRT?
Reduced rate of orbital recurrence
*Reminder: XBRT has limited effect on survival rate /:*
183
T/F: For patients with medium sized tumors, XBRT can be used as monotherapy
FALSE; XBRT can *never* be used as monotherapy (always in conjunction with enucleation)
184
What would you expect to see on an A-scan of a melanoma?
First spike (retina)
Hollow/dip (melanoma)
Second spike (sclera)
185
What shapes are most commonly found on B scan of a melanoma?
Dome or mushroom/“collar button”
186
How would a melanoma look differently than an osteoma or hemangioma on a B-scan?
Melanomas appear *hollow*
Osteomas and hemangiomas would not
187
What adverse effects do Plaque Radiotherapy cause?
Radiotherapy retinopathy and optic neuropathy
*affects posterior*
188
What adverse effects do Charged-Particle (Proton) Radiotherapy cause?
Cataracts or NVG
*affects anterior*
189
True/False: both Plaque RT and Proton RT are said to have “good tumor control rate” as melanoma treatment options
TRUE
190
Which location for uveal melanoma has a better prognosis: anterior or posterior?
Posterior — better prognosis
Anterior — worse prognosis
191
What is the mechanism for metastasis of melanomas?
Hematogenous (via blood)
192
What is a *ring melanoma* and what does it indicate?
Poor prognosis
193
What are 8 factors that indicate poor prognosis in melanomas?
**LARGEST**
1. Larger size
2. Anterior location
3. Recurrence
4. Growth (documented)
5. Extraocular extension
6. Sclera contact
7. Type of cell: Epithelioid
194
What are the two *most important* factors indicating poor prognosis in melanoma?
Type of cell (Epithelioid) and Scleral contact
195
What is the median duration from treatment of melanoma to diagnosis of metastasis?
7 yrs
196
WHat is the median duration from diagnosis of melanoma metastasis to death?
6 months
197
Most common site of melanoma METS?
LIVER (by 95%)
198
What percent of fatalities, secondary to melanoma metastasis, only have liver Mets?
33%
