Pigmented Lesions Flashcards
Definition of Hypertrophy
Increase in size of a cell
Etiology of CHRPE
Idiopathic, congenital
CHRPE: malignant or benign?
BENIGN
CHRPE: symptoms
Usually asymptomatic
- Can cause VF defects (due to atrophy of PRs)
- Can cause VL (if located on fovea — but very, very rare)
CHRPE is most commonly found in which quadrant?
Temporal
TRUE/FALSE: In CHRPE, because the RPE cell is enlarged, it maintains the same amount of melanin thus the melanin appears to be less
FALSE; CHRPE are enlarged cells with large amounts of melanin
Explain how an hypertrophy of an RPE cell can cause degeneration of a PR
Hypertrophy impairs RPE cells Phagocytic ability, leading to degeneration of PR
Describe the typical SHAPE of a CHRPE
Flat, round, with distinct margins
TRUE/FALSE: CHRPE don’t normally change in size
TRUE
TRUE/FALSE: CHRPE is common in the posterior pole
FALSE
CHRPE: typically unilateral or bilateral?
Unilateral
CHRPE: what a lacunae?
Window view (within the CHRPE) of the underlying choroid and sclera
TRUE/FALSE: lacunae is an example of chorioretinal atrophy
TRUE
Describe the typical COLOR of a CHRPE
Light brown to jet black
(Potentially with white spots, “lacunae”)
Describe the typical SIZE of a CHRPE
2-6 mm
What are “bear tracks”?
Multiple CHRPE in one sector of fundus, with up to 30 lesions in each group
For Bear Tracks, larger lesions are usually more _____ (posterior/peripheral), where as smaller lesions are usually more ____ (posterior/peripheral).
Large — peripheral
Smaller — posterior
TRUE/FALSE: CHRPE are not associated with Gardner’s Syndrome
TRUE;
CHRPE is not associated with Gardner’s
TX for CHRPE
Just monitor :)
CHRPE
How does CHRPE appear on Fundus Autofluorescence?
CHRPE itself will be hypopigmented but lacunae may be hyperpigmented
What is Gardner Syndrome?
Familial Adenomatous Polyposis + extracolonic manifestations (e.g. ocular)
What is Familial Adenomatous Polyposis?
AD genetic disorder, associated with colon polyps/cancer
What is the prognosis of Familial Adenomatous Polyposis (FAP)?
100% malignancy, if left untreated
TRUE/FALSE: Familial Adenomatous Polyposis can occur without any family history
TRUE;
20% of cases — spontaneous mutation of gene
FAP makes up ____% of all colon cancer in US annually
1%
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
Management for “CHRPE-like” Retinal Hamartoma
Genetic testing and GI consult
Describe the appearance of the lesion associated with FAP/Gardner Syndrome (5)
- Contains depigmented halo, pointing toward ON
- Multiple lesions (no sectoral organization)
- Usually small (< 0.1 DD)
- Oval/Pisciform/Football
- Less well-defined relative to CHRPE
Is the lesion associated with FAP/Gardner’s usually unilateral or bilateral?
Usually bilateral
Is the lesion associated with FAP/Gardner’s usually found more posteriorly or more peripherally?
Peripheral, esp near vortex veins
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)
Definition of Hyperplasia
Increase in number of cells
What does the RPE have a propensity to do, in response to ocular insullt?
Proliferate and migrate
What are some common stimuli to RPE Hyperplasia?
Inflammation and trauma
RPE Hyperplasia usually presents as _____
Scars or pigment clumps
RPE Hyperplasia:
Pigment clumping helps to identify localized regions of ________ that could produce a _______
Regions of vitreoretinal adhesion that could produce retinal tears
Describe the appearance of RPE Hyperplasia
- Color: Jet-black
- Irregular
- Often stellate margins
- May have fibrosis/gliosis
- Almost always has sensory retinal degeneration (white appearance)
In RPE Hyperplasia, the RPE cells migrate to the ______ (anterior/posterior) retina
ANTERIOR
Management for RPE Hyperplasia
Observation
Melanocytomas are usually _______ (unilateral/bilateral)
Unilateral
Location of Melanocytoma
Eccentric to Optic Nerve
Racial p revalence of Melanocytoma
No racial preference
Melanocytes is composed of what type of cells?
Darkly-pigmented, plump, polyhedral cells
Melanocytoma: benign or malignant?
Benign
*Malignant transformation is rare (1-2% of cases)
Describe the appearance of a Melanocytoma (4)
- Dark brown
- Elevated
- Usually does not exceed 1 DD
- Indistinct (sometimes “feathered”) margins
What appearance in a Melanocytoma is indicative of extension into the NFL?
Feathered margins
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
T/F: Melanocytoma can produce an APD
TRUE; affects ON
What would you expect to see on a Visual Field of a patient that has a Melanocytoma?
Enlarged blind spot and extensive NFL defects
What choroidal association would you find with a Melanocytoma?
Peripapillary choroidal nexus
T/F: growth is common in melanocytomas
TRUE
What is an indication of Melanocytoma malignancy?
Progressive growth with visual loss
Management of Malignant-transformed Melanocytoma
May have to consider enucleation
What is the most common intraocular tumor?
Choroidal nevus
Prevalence of Choroidal Nevi
~10% of population
Choroidal nevi are precursors for ____
Choroidal melanoma
Annual rate of choroidal nevus malignant transformation ______ (increases/decreases) with age
INCREASES
Rate at which choroidal nevi tranforms into a malignancy
1 in 8,845
Signs of Choroidal Nevus Chronicity (6)
- Overlying drusen
- Overlying PED
- Chorioretinal Atrophy
- RPE Hypertrophy
- Fibrous metaplasia
- RPE Trough
What is the best imaging to visualize and differentiate choroidal nevus from choroidal melanoma?
Enhanced Depth Imaging (EDI)
OCT tends to _______ (underestimate/overestimate) thickness of lesion by ___%
Underestimate by 50%
On OCT, any nevus larger than _____ mm is suspicious of melanoma
Larger than 1 mm
“Shaggy Receptors” are commonly seen in what type of lesion?
Melanomas
What risk factor is pathognomonic for choroidal melanoma?
NONE
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
Which risk factors have the 3 highest Hazard Ratios for Choroidal Melanoma?
- Thickness > 2 mm
- Fluid
- Orange Pigment
How many choroidal nevi are halo nevi?
5%
Describe a Halo Nevus
Typical nevus, surrounded by irregular depigmented chorioretinal halo
Halo nevi are associated with previous diagnoses of…
Cutaneous melanoma
Halo nevi may represent…
An autoimmune reaction
T/F: Halo around nevi indicates growth
FALSE; it indicates stability
How to Manage Choroidal Nevus
- Photo documentation (color photos, FAF, and OCT at every visit)
- Use TFSOM criteria:
- No risk factors —> monitor
- 1 RF —> q6m-1yr
- Growth or 2+ RF —> consider treatment
Racial Melanosis is commonly found where?
Conjunctiva, near limbus
Racial melanosis ____ (increase/decrease) by age
INCREASE
T/F: Racial Melanosis increases risk of Melanoma
FALSE
Racial Melanosis is typically _____ (unilateral/bilateral)
Bilateral
Primary Acquired Melanosis:
- onset?
- unilateral vs bilateral?
- likelihood to transform to conj melanoma?
- Adult onset
- Unilateral
- 20%
Treatment Options for Choroidal Melanoma
- Enucleation
- Brachytherapy
- Proton Laser Therapy
____% of Choroidal melanomas will demonstrate extra-scleral extension
3%
Ciliary Body Melanomas usually spread via
Scleral channels
“Sentinel vessels”
Posterior Melanomas spread via…
vortex veins and posterior ciliary arteries
Top Ten Pseudomelanomas (DDX)
- Nevus
- PERCH
- CHRPE
- Hemorrhagic Retinal or RPE detachment
- Choroidal hemangioma
- AMD
- RPE Hyperplasia
- Optic Disc Melanocytoma
- Choroidal Metastasis
- Hemorrhagic Choroidal Detachment
Most common location of choroidal melanoma metastasis
LIVER
also Lungs and Brain
RF for Metastasis of Choroidal Melanoma
Tumor touching ON margin or histopathologic, cytogenic, and genetic factors
About 25% of pts w/ uveal melanoma have ______ 5 yrs after treatment
Metastasis
What is the metastatic work up necessary for all melanoma patients?
- Liver function test
- Liver imaging
- Chest x-ray
annually
CHRPE w/ lacunae
Bear Tracks (CHRPE)
Halo Choroidal Nevus
RPE Hyperplasia
CHRPE w/ lacunae
Retinal Hamartoma (as related to FAP)
RPE Hyperplasia
Nevus
Note: No SRF
Nevus
Note: Drusen/PED
Melanoma
Note: Shaggy PRs and SRF
Melanoma
Note: Shaggy PR and SRF
Melanoma
Note: Lipofuscin, SRF, and shaggy PRs
Nevus
Note: Although PRs are irregular, fully attached to RPE (no PED)
Nevus :)
Melanoma /:
Note: Shaggy PR + SRF + minimal shadowing
Nevus :D
Note: Drusen + DENSE shadowing
Melanocytoma of ON
Note: the lesion extends intraocularly and outward, into the optic disc
RPE Hyperplasia
Halo Choroidal Nevus
Melanocytoma of ON
RPE Hyperplasia
Melanocytoma w/ Peripapillary Choroidal Nevus
Retinal Hamartoma, as assc with FAP
What is the color of a Choroidal Nevus?
Gray-Brown
Can Choroidal Nevi be amelanotic?
YES
Margins of a nevus?
INDISTINCT
Elevation of Nevus?
Flat (or just minimally elevated)
Which clinical factor is pathognomonic for whether a melanocytic lesion is benign or malignant?
NOTHING!
Virtually all lesions < _____ mm thick are benign nevi
1 mm
Virtually all melanocytic lesions > ___ mm thick are melanomas
> 3 mm
A flat melanocytic lesion less than ____ mm in diameter is almost always benign
10 mm
What color pigment is indicative of a melanoma (instead of a nevus)?
ORANGE
Melanoma
Note: orange pigment
Melanoma
Note: orange pigment
Melanoma: orange pigment substance
Lipofuscin
Melanoma: why is orange pigment worrisome?
Indicates the the lesion is actively growing ‼️
Nevus or Melanoma: presence of drusen
Likely nevus (indicates chronicity)
Nevus or Melanoma: absence of drusen
Choroidal melanoma
Choroidal nevus with drusen
Choroidal nevus with drusen
Nevus or melanoma: absence of SRF
Likely nevus
Nevus or melanoma: presence of SRF
Likely melanoma
Nevus or melanoma: location near ONH
Likely melanoma
Choroidal melanoma
Drs. Shields Mnemonic and what does it represent
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
What symptoms are worrisome when suspecting a choroidal melanoma?
- Metamorphopsia
- Photopsia
- VF Loss
What is the incidence rate of a choroidal/ciliary body melanoma?
1 in 6-7 million
Which is more common: cutaneous or intraocular melanoma? By how much?
CUTANEOUS (by 20x)
Peak age for choroidal/ciliary body melanoma
55-65
For choroidal/ciliary body melanoma, aside from the peak age, there is a smaller secondary peak age of:
20-40
For choroidal/ciliary body melanoma, what is the sex preference for the main peak age?
M = F
For choroidal/ciliary body melanoma, what is the sex preference for the smaller/secondary peak age?
F > M
Does intraocular melanoma occur in children?
Yes, but rarely
For intraocular melanoma, which age group has a better prognosis?
Kids have a better prognosis :)
Risk Factors for Choroidal/Ciliary Body Melanoma
- Oculodermal melanocytosis (aka Nevus of Ota)
- Light irides
- Cigarette smoking
- “Northern European” ethnicity (wypipo)
How does dermal melanocytosis present clinically?
Brown, gray, or blue pigmentation
How does ocular melanocytosis present clinically?
Slate-gray patches of episcleral pigment
In addition to choroidal melanoma, ocular melanocytosis has an increased risk of…
Glaucoma
10% of pts with Nevus of Ota will develop GLC
An example of oculodermal (ocular + dermal) melanocytosis
What is racial predilection of oculodermal melanocytosis?
Hispanic, African, and/or Asian
pigmented people
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)
Is sun exposure a risk factor for choroidal melanoma?
Maybe (lacking data to confirm)
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
In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for small tumors?
Observational
In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for medium tumors?
Randomized Controlled Study
In the major clinical trial that addressed management of intraocular melanoma, what experiment design was used for large tumors?
Randomized Controlled Study
In the major clinical trial that addressed management of intraocular melanoma, how many small tumors were enrolled?
~200 (least)
In the major clinical trial that addressed management of intraocular melanoma, how many medium tumors were enrolled?
~1300 (most)
In the major clinical trial that addressed management of intraocular melanoma, how many large tumors were enrolled?
~1000
In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for small tumors?
Treatment vs. Observation
In the major clinical trial that addressed management of intraocular melanoma, what were the study arms for medium tumors?
Enucleation vs. Plaque Therapy
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
In the major clinical trial that addressed management of intraocular melanoma, did pre-op XBRT improve survival?
No /:
In the major clinical trial that addressed management of intraocular melanoma, did treatment modality affect survival for medium tumors?
Nope
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
In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within 1 year.
10%
In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within 5 year.
20%
In the major clinical trial that addressed management of intraocular melanoma, _____% of small tumors grew to medium or large tumor within 10 years.
30%
Clinical Testing for Melanoma
- Indirect Ophthalmoscopy
- Gonioscopy
- Transillumination
- Fluorescein angiography
What is the classic FA pattern for a choroidal melanoma?
Late hyperfluorescence
double circulation
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
The double circulation pattern is more evident under: FA or ICGA?
ICGA!
What is the #1 Ancillary study for Melanoma?
Ultrasound (both A & B scans)
T/F: CT/MRI scans are suggested for all pts that have a choroidal melanoma
Not really widely used
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
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
What percent of ocular melanomas have demonstrable metastasis at time of diagnosis?
2%
What percent of ocular melanomas have occult (hidden/imperceivable) metastasis at time of diagnosis?
Unknown, but higher than 2%
In terms of melanoma, COMS found that ____% of patients harbored a second malignancy
10%
Choroidal melanoma: what are some general pre-treatment tests?
- Complete physical exam
- Chest X-Ray
- Liver Function Test
- CT/MRI (not as much tbh)
In what cases should the appropriate treatment for the melanoma be observation?
- Tumor < 1 mm thick
- Pt unable to tolerate treatment
In what cases should the appropriate treatment for the melanoma be *enucleation *?
For *many medium tumors, but all large tumors
What are 3 radiation modalities employed for melanomas?
- XBRT (External Beam Radiotherapy)
- Plaque Therapy
- Charged-particle
What is the main benefit of XBRT?
Reduced rate of orbital recurrence
Reminder: XBRT has limited effect on survival rate /:
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)
What would you expect to see on an A-scan of a melanoma?
First spike (retina)
Hollow/dip (melanoma)
Second spike (sclera)
What shapes are most commonly found on B scan of a melanoma?
Dome or mushroom/“collar button”
How would a melanoma look differently than an osteoma or hemangioma on a B-scan?
Melanomas appear hollow
Osteomas and hemangiomas would not
What adverse effects do Plaque Radiotherapy cause?
Radiotherapy retinopathy and optic neuropathy
affects posterior
What adverse effects do Charged-Particle (Proton) Radiotherapy cause?
Cataracts or NVG
affects anterior
True/False: both Plaque RT and Proton RT are said to have “good tumor control rate” as melanoma treatment options
TRUE
Which location for uveal melanoma has a better prognosis: anterior or posterior?
Posterior — better prognosis
Anterior — worse prognosis
What is the mechanism for metastasis of melanomas?
Hematogenous (via blood)
What is a ring melanoma and what does it indicate?
Poor prognosis
What are 8 factors that indicate poor prognosis in melanomas?
LARGEST
- Larger size
- Anterior location
- Recurrence
- Growth (documented)
- Extraocular extension
- Sclera contact
- Type of cell: Epithelioid
What are the two most important factors indicating poor prognosis in melanoma?
Type of cell (Epithelioid) and Scleral contact
What is the median duration from treatment of melanoma to diagnosis of metastasis?
7 yrs
WHat is the median duration from diagnosis of melanoma metastasis to death?
6 months
Most common site of melanoma METS?
LIVER (by 95%)
What percent of fatalities, secondary to melanoma metastasis, only have liver Mets?
33%
