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X. Post Seg 2 > Pigmented Lesions > Flashcards

Pigmented Lesions Flashcards

1
Q

Definition of Hypertrophy

A

Increase in size of a cell

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2
Q

Etiology of CHRPE

A

Idiopathic, congenital

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3
Q

CHRPE: malignant or benign?

A

BENIGN

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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)
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5
Q

CHRPE is most commonly found in which quadrant?

A

Temporal

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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

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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

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8
Q

Describe the typical SHAPE of a CHRPE

A

Flat, round, with distinct margins

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9
Q

TRUE/FALSE: CHRPE don’t normally change in size

A

TRUE

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10
Q

TRUE/FALSE: CHRPE is common in the posterior pole

A

FALSE

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11
Q

CHRPE: typically unilateral or bilateral?

A

Unilateral

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12
Q

CHRPE: what a lacunae?

A

Window view (within the CHRPE) of the underlying choroid and sclera

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13
Q

TRUE/FALSE: lacunae is an example of chorioretinal atrophy

A

TRUE

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14
Q

Describe the typical COLOR of a CHRPE

A

Light brown to jet black
(Potentially with white spots, “lacunae”)

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15
Q

Describe the typical SIZE of a CHRPE

A

2-6 mm

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16
Q

What are “bear tracks”?

A

Multiple CHRPE in one sector of fundus, with up to 30 lesions in each group

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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

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18
Q

TRUE/FALSE: CHRPE are not associated with Gardner’s Syndrome

A

TRUE;
CHRPE is not associated with Gardner’s

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19
Q

TX for CHRPE

A

Just monitor :)

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20
Q
A

CHRPE

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21
Q

How does CHRPE appear on Fundus Autofluorescence?

A

CHRPE itself will be hypopigmented but lacunae may be hyperpigmented

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22
Q

What is Gardner Syndrome?

A

Familial Adenomatous Polyposis + extracolonic manifestations (e.g. ocular)

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23
Q

What is Familial Adenomatous Polyposis?

A

AD genetic disorder, associated with colon polyps/cancer

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24
Q

What is the prognosis of Familial Adenomatous Polyposis (FAP)?

A

100% malignancy, if left untreated

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25
Q

TRUE/FALSE: Familial Adenomatous Polyposis can occur without any family history

A

TRUE;
20% of cases — spontaneous mutation of gene

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26
Q

FAP makes up ____% of all colon cancer in US annually

A

1%

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27
Q

What ocular lesion is a specific and reliable marker for FAP?

And how prevalent is the lesion in pts with FAP?

A

“CHRPE-like” retinal hamartomas
Present in 70% of FAP genotypes

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28
Q

Management for “CHRPE-like” Retinal Hamartoma

A

Genetic testing and GI consult

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29
Q

Describe the appearance of the lesion associated with FAP/Gardner Syndrome (5)

A
  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
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30
Q

Is the lesion associated with FAP/Gardner’s usually unilateral or bilateral?

A

Usually bilateral

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31
Q

Is the lesion associated with FAP/Gardner’s usually found more posteriorly or more peripherally?

A

Peripheral, esp near vortex veins

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32
Q

TRUE/FALSE: The ocular lesion associated with FAP has a higher sensitivity than specificity.

A

FALSE;
Specificity — 78% (absence of lesion does not rule out FAP)
Sensitivity — 95% (presence of lesion highly predictive of FAP)

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33
Q

Definition of Hyperplasia

A

Increase in number of cells

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34
Q

What does the RPE have a propensity to do, in response to ocular insullt?

A

Proliferate and migrate

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35
Q

What are some common stimuli to RPE Hyperplasia?

A

Inflammation and trauma

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36
Q

RPE Hyperplasia usually presents as _____

A

Scars or pigment clumps

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37
Q

RPE Hyperplasia:
Pigment clumping helps to identify localized regions of ________ that could produce a _______

A

Regions of vitreoretinal adhesion that could produce retinal tears

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38
Q

Describe the appearance of RPE Hyperplasia

A
  1. Color: Jet-black
  2. Irregular
  3. Often stellate margins
  4. May have fibrosis/gliosis
  5. Almost always has sensory retinal degeneration (white appearance)
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39
Q

In RPE Hyperplasia, the RPE cells migrate to the ______ (anterior/posterior) retina

A

ANTERIOR

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40
Q

Management for RPE Hyperplasia

A

Observation

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41
Q

Melanocytomas are usually _______ (unilateral/bilateral)

A

Unilateral

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42
Q

Location of Melanocytoma

A

Eccentric to Optic Nerve

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43
Q

Racial p revalence of Melanocytoma

A

No racial preference

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44
Q

Melanocytes is composed of what type of cells?

A

Darkly-pigmented, plump, polyhedral cells

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45
Q

Melanocytoma: benign or malignant?

A

Benign
*Malignant transformation is rare (1-2% of cases)

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46
Q

Describe the appearance of a Melanocytoma (4)

A
  1. Dark brown
  2. Elevated
  3. Usually does not exceed 1 DD
  4. Indistinct (sometimes “feathered”) margins
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47
Q

What appearance in a Melanocytoma is indicative of extension into the NFL?

A

Feathered margins

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48
Q

Symptoms of Melanocytoma

A

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

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49
Q

T/F: Melanocytoma can produce an APD

A

TRUE; affects ON

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50
Q

What would you expect to see on a Visual Field of a patient that has a Melanocytoma?

A

Enlarged blind spot and extensive NFL defects

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51
Q

What choroidal association would you find with a Melanocytoma?

A

Peripapillary choroidal nexus

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52
Q

T/F: growth is common in melanocytomas

A

TRUE

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53
Q

What is an indication of Melanocytoma malignancy?

A

Progressive growth with visual loss

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54
Q

Management of Malignant-transformed Melanocytoma

A

May have to consider enucleation

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55
Q

What is the most common intraocular tumor?

A

Choroidal nevus

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56
Q

Prevalence of Choroidal Nevi

A

~10% of population

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57
Q

Choroidal nevi are precursors for ____

A

Choroidal melanoma

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58
Q

Annual rate of choroidal nevus malignant transformation ______ (increases/decreases) with age

A

INCREASES

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59
Q

Rate at which choroidal nevi tranforms into a malignancy

A

1 in 8,845

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60
Q

Signs of Choroidal Nevus Chronicity (6)

A
  1. Overlying drusen
  2. Overlying PED
  3. Chorioretinal Atrophy
  4. RPE Hypertrophy
  5. Fibrous metaplasia
  6. RPE Trough
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61
Q

What is the best imaging to visualize and differentiate choroidal nevus from choroidal melanoma?

A

Enhanced Depth Imaging (EDI)

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62
Q

OCT tends to _______ (underestimate/overestimate) thickness of lesion by ___%

A

Underestimate by 50%

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63
Q

On OCT, any nevus larger than _____ mm is suspicious of melanoma

A

Larger than 1 mm

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64
Q

“Shaggy Receptors” are commonly seen in what type of lesion?

A

Melanomas

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65
Q

What risk factor is pathognomonic for choroidal melanoma?

A

NONE

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66
Q

What is the mnemonic for suspecting a Melanoma?

A

To Find Small Ocular Melanoma Doing Imaging (TFSOMDIm)

Thickness > 2 mm
Fluid (+)
Symptomatic
Orange pigment
Melanoma Acoustic Hollow
Diameter > 5 mm

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67
Q

Which risk factors have the 3 highest Hazard Ratios for Choroidal Melanoma?

A
  1. Thickness > 2 mm
  2. Fluid
  3. Orange Pigment
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68
Q

How many choroidal nevi are halo nevi?

A

5%

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69
Q

Describe a Halo Nevus

A

Typical nevus, surrounded by irregular depigmented chorioretinal halo

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70
Q

Halo nevi are associated with previous diagnoses of…

A

Cutaneous melanoma

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71
Q

Halo nevi may represent…

A

An autoimmune reaction

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72
Q

T/F: Halo around nevi indicates growth

A

FALSE; it indicates stability

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73
Q

How to Manage Choroidal Nevus

A
  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
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74
Q

Racial Melanosis is commonly found where?

A

Conjunctiva, near limbus

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75
Q

Racial melanosis ____ (increase/decrease) by age

A

INCREASE

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76
Q

T/F: Racial Melanosis increases risk of Melanoma

A

FALSE

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77
Q

Racial Melanosis is typically _____ (unilateral/bilateral)

A

Bilateral

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78
Q

Primary Acquired Melanosis:
- onset?
- unilateral vs bilateral?
- likelihood to transform to conj melanoma?

A
  • Adult onset
  • Unilateral
  • 20%
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79
Q

Treatment Options for Choroidal Melanoma

A
  1. Enucleation
  2. Brachytherapy
  3. Proton Laser Therapy
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80
Q

____% of Choroidal melanomas will demonstrate extra-scleral extension

A

3%

81
Q

Ciliary Body Melanomas usually spread via

A

Scleral channels
“Sentinel vessels”

82
Q

Posterior Melanomas spread via…

A

vortex veins and posterior ciliary arteries

83
Q

Top Ten Pseudomelanomas (DDX)

A
  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
Q

Most common location of choroidal melanoma metastasis

A

LIVER

also Lungs and Brain

85
Q

RF for Metastasis of Choroidal Melanoma

A

Tumor touching ON margin or histopathologic, cytogenic, and genetic factors

86
Q

About 25% of pts w/ uveal melanoma have ______ 5 yrs after treatment

A

Metastasis

87
Q

What is the metastatic work up necessary for all melanoma patients?

A
  1. Liver function test
  2. Liver imaging
  3. Chest x-ray

annually

88
Q
A

CHRPE w/ lacunae

89
Q
A

Bear Tracks (CHRPE)

90
Q
A

Halo Choroidal Nevus

91
Q
A

RPE Hyperplasia

92
Q
A

CHRPE w/ lacunae

93
Q
A

Retinal Hamartoma (as related to FAP)

94
Q
A

RPE Hyperplasia

95
Q
A

Nevus

Note: No SRF

96
Q
A

Nevus

Note: Drusen/PED

97
Q
A

Melanoma

Note: Shaggy PRs and SRF

98
Q
A

Melanoma

Note: Shaggy PR and SRF

99
Q
A

Melanoma

Note: Lipofuscin, SRF, and shaggy PRs

100
Q
A

Nevus

Note: Although PRs are irregular, fully attached to RPE (no PED)

101
Q
A

Nevus :)

102
Q
A

Melanoma /:

Note: Shaggy PR + SRF + minimal shadowing

103
Q
A

Nevus :D

Note: Drusen + DENSE shadowing

104
Q
A

Melanocytoma of ON

Note: the lesion extends intraocularly and outward, into the optic disc

105
Q
A

RPE Hyperplasia

106
Q
A

Halo Choroidal Nevus

107
Q
A

Melanocytoma of ON

108
Q
A

RPE Hyperplasia

109
Q
A

Melanocytoma w/ Peripapillary Choroidal Nevus

110
Q
A

Retinal Hamartoma, as assc with FAP

111
Q

What is the color of a Choroidal Nevus?

A

Gray-Brown

112
Q

Can Choroidal Nevi be amelanotic?

A

YES

113
Q

Margins of a nevus?

A

INDISTINCT

114
Q

Elevation of Nevus?

A

Flat (or just minimally elevated)

115
Q

Which clinical factor is pathognomonic for whether a melanocytic lesion is benign or malignant?

A

NOTHING!

116
Q

Virtually all lesions < _____ mm thick are benign nevi

A

1 mm

117
Q

Virtually all melanocytic lesions > ___ mm thick are melanomas

A

> 3 mm

118
Q

A flat melanocytic lesion less than ____ mm in diameter is almost always benign

A

10 mm

119
Q

What color pigment is indicative of a melanoma (instead of a nevus)?

A

ORANGE

120
Q
A

Melanoma

Note: orange pigment

121
Q
A

Melanoma

Note: orange pigment

122
Q

Melanoma: orange pigment substance

A

Lipofuscin

123
Q

Melanoma: why is orange pigment worrisome?

A

Indicates the the lesion is actively growing ‼️

124
Q

Nevus or Melanoma: presence of drusen

A

Likely nevus (indicates chronicity)

125
Q

Nevus or Melanoma: absence of drusen

A

Choroidal melanoma

126
Q
A

Choroidal nevus with drusen

127
Q
A

Choroidal nevus with drusen

128
Q

Nevus or melanoma: absence of SRF

A

Likely nevus

129
Q

Nevus or melanoma: presence of SRF

A

Likely melanoma

130
Q

Nevus or melanoma: location near ONH

A

Likely melanoma

131
Q
A

Choroidal melanoma

132
Q

Drs. Shields Mnemonic and what does it represent

A

To Find Small Ocular Melanomas, Use Helpful Hints Daily

Thickness > 2 mm
Fluid (+)
Symptomatic
Orange pigment
Margin touching ONH
Ultrasound Hollowness
Halo Absence
Drusen Absence

133
Q

What symptoms are worrisome when suspecting a choroidal melanoma?

A
  1. Metamorphopsia
  2. Photopsia
  3. VF Loss
134
Q

What is the incidence rate of a choroidal/ciliary body melanoma?

A

1 in 6-7 million

135
Q

Which is more common: cutaneous or intraocular melanoma? By how much?

A

CUTANEOUS (by 20x)

136
Q

Peak age for choroidal/ciliary body melanoma

A

55-65

137
Q

For choroidal/ciliary body melanoma, aside from the peak age, there is a smaller secondary peak age of:

A

20-40

138
Q

For choroidal/ciliary body melanoma, what is the sex preference for the main peak age?

A

M = F

139
Q

For choroidal/ciliary body melanoma, what is the sex preference for the smaller/secondary peak age?

A

F > M

140
Q

Does intraocular melanoma occur in children?

A

Yes, but rarely

141
Q

For intraocular melanoma, which age group has a better prognosis?

A

Kids have a better prognosis :)

142
Q

Risk Factors for Choroidal/Ciliary Body Melanoma

A
  1. Oculodermal melanocytosis (aka Nevus of Ota)
  2. Light irides
  3. Cigarette smoking
  4. “Northern European” ethnicity (wypipo)
143
Q

How does dermal melanocytosis present clinically?

A

Brown, gray, or blue pigmentation

144
Q

How does ocular melanocytosis present clinically?

A

Slate-gray patches of episcleral pigment

145
Q

In addition to choroidal melanoma, ocular melanocytosis has an increased risk of…

A

Glaucoma

10% of pts with Nevus of Ota will develop GLC

146
Q
A

An example of oculodermal (ocular + dermal) melanocytosis

147
Q

What is racial predilection of oculodermal melanocytosis?

A

Hispanic, African, and/or Asian
pigmented people

148
Q

T/F: The presence of ocular melanocytosis in African Americans increases risk of melanoma

A

FALSE; normal in pigmented population
worrisome in Caucasians (1 in 400)

149
Q

Is sun exposure a risk factor for choroidal melanoma?

A

Maybe (lacking data to confirm)

150
Q

What is the acronym for the major clinical trial that addressed management of intraocular melanoma?

What does it stand for?

A

COMS:
Collaborative Ocular Melanoma Study

151
Q

In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for small tumors?

A

Observational

152
Q

In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for medium tumors?

A

Randomized Controlled Study

153
Q

In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for large tumors?

A

Randomized Controlled Study

154
Q

In the major clinical trial that addressed management of intraocular melanoma, how many small tumors were enrolled?

A

~200 (least)

155
Q

In the major clinical trial that addressed management of intraocular melanoma, how many medium tumors were enrolled?

A

~1300 (most)

156
Q

In the major clinical trial that addressed management of intraocular melanoma, how many large tumors were enrolled?

A

~1000

157
Q

In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for small tumors?

A

Treatment vs. Observation

158
Q

In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for medium tumors?

A

Enucleation vs. Plaque Therapy

159
Q

In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for large tumors?

A

Enucleation w/ XBRT or Enucleation w/o XBRT

160
Q

In the major clinical trial that addressed management of intraocular melanoma, did pre-op XBRT improve survival?

A

No /:

161
Q

In the major clinical trial that addressed management of intraocular melanoma, did treatment modality affect survival for medium tumors?

A

Nope

162
Q

In the major clinical trial that addressed management of intraocular melanoma, for small tumors, was the outcome better to treat or observe?

A

Not enough enrollment in ‘Treatment’ arm to compare

163
Q

In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within 1 year.

A

10%

164
Q

In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within 5 year.

A

20%

165
Q

In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within 10 years.

A

30%

166
Q

Clinical Testing for Melanoma

A
  1. Indirect Ophthalmoscopy
  2. Gonioscopy
  3. Transillumination
  4. Fluorescein angiography
167
Q

What is the classic FA pattern for a choroidal melanoma?

A

Late hyperfluorescence
double circulation

168
Q

If a malignant lesion is noted in the choroid, how can you differentiate between a choroidal melanoma and metastatic choroidal tumor (on FA)?

A

Melanoma will have its own internal circulation —>
“Double circulation” or Late hyperfluorescence

169
Q

The double circulation pattern is more evident under: FA or ICGA?

A

ICGA!

170
Q

What is the #1 Ancillary study for Melanoma?

A

Ultrasound (both A & B scans)

171
Q

T/F: CT/MRI scans are suggested for all pts that have a choroidal melanoma

A

Not really widely used

172
Q

T/F: An OCT does not provide a great image that allows for differentiation of a melanoma

A

Eh, true and false —
SD-OCT does not penetrate enough, but
Enhanced Depth OCT (EDI-OCT) does

173
Q

Melanoma: Explain the Callendar Classification System

A

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
Q
A
175
Q

What percent of ocular melanomas have demonstrable metastasis at time of diagnosis?

A

2%

176
Q

What percent of ocular melanomas have occult (hidden/imperceivable) metastasis at time of diagnosis?

A

Unknown, but higher than 2%

177
Q

In terms of melanoma, COMS found that ____% of patients harbored a second malignancy

A

10%

178
Q

Choroidal melanoma: what are some general pre-treatment tests?

A
  1. Complete physical exam
  2. Chest X-Ray
  3. Liver Function Test
  4. CT/MRI (not as much tbh)
179
Q

In what cases should the appropriate treatment for the melanoma be observation?

A
  1. Tumor < 1 mm thick
  2. Pt unable to tolerate treatment
180
Q

In what cases should the appropriate treatment for the melanoma be *enucleation *?

A

For *many medium tumors, but all large tumors

181
Q

What are 3 radiation modalities employed for melanomas?

A
  1. XBRT (External Beam Radiotherapy)
  2. Plaque Therapy
  3. Charged-particle
182
Q

What is the main benefit of XBRT?

A

Reduced rate of orbital recurrence

Reminder: XBRT has limited effect on survival rate /:

183
Q

T/F: For patients with medium sized tumors, XBRT can be used as monotherapy

A

FALSE; XBRT can never be used as monotherapy (always in conjunction with enucleation)

184
Q

What would you expect to see on an A-scan of a melanoma?

A

First spike (retina)
Hollow/dip (melanoma)
Second spike (sclera)

185
Q

What shapes are most commonly found on B scan of a melanoma?

A

Dome or mushroom/“collar button”

186
Q

How would a melanoma look differently than an osteoma or hemangioma on a B-scan?

A

Melanomas appear hollow
Osteomas and hemangiomas would not

187
Q

What adverse effects do Plaque Radiotherapy cause?

A

Radiotherapy retinopathy and optic neuropathy

affects posterior

188
Q

What adverse effects do Charged-Particle (Proton) Radiotherapy cause?

A

Cataracts or NVG

affects anterior

189
Q

True/False: both Plaque RT and Proton RT are said to have “good tumor control rate” as melanoma treatment options

A

TRUE

190
Q

Which location for uveal melanoma has a better prognosis: anterior or posterior?

A

Posterior — better prognosis
Anterior — worse prognosis

191
Q

What is the mechanism for metastasis of melanomas?

A

Hematogenous (via blood)

192
Q

What is a ring melanoma and what does it indicate?

A

Poor prognosis

193
Q

What are 8 factors that indicate poor prognosis in melanomas?

A

LARGEST

  1. Larger size
  2. Anterior location
  3. Recurrence
  4. Growth (documented)
  5. Extraocular extension
  6. Sclera contact
  7. Type of cell: Epithelioid
194
Q

What are the two most important factors indicating poor prognosis in melanoma?

A

Type of cell (Epithelioid) and Scleral contact

195
Q

What is the median duration from treatment of melanoma to diagnosis of metastasis?

A

7 yrs

196
Q

WHat is the median duration from diagnosis of melanoma metastasis to death?

A

6 months

197
Q

Most common site of melanoma METS?

A

LIVER (by 95%)

198
Q

What percent of fatalities, secondary to melanoma metastasis, only have liver Mets?

A

33%

X. Post Seg 2 (13 decks)

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