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Uveal Melanomas Flashcards

1
Q

What % of ocular melanomas are found in the uveal tract?

A

> 95%

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2
Q

What 2 oncogenes are exclusive for a patient developing a nevus? What % definitely receive a nevus?

A

GNAQ and GNA11

- 80%

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3
Q

What is the most common primary intraocular malignancy?

A

Uveal melanoma

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4
Q

What is the second most common form of melanoma?

A

Uveal melanoma

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5
Q

How many new cases of uveal melanomas are found each year in the US?

A

2,000-2,500

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6
Q

What percent of patients die from melanoma in 1 year?

A

50%

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7
Q

What is the mean age of patients who get uveal melanomas?

A

60-70 yo

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8
Q

What are the 3 main treatments for the uveal melanoma?

A
  1. Radiation Therapy
  2. Anti-VEGF
  3. Thermotherapy
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9
Q

What % of uveal melanomas metastisize to the liver? lungs? fellow-eye?

A

Liver = 93%
Lungs = 24%
Fellow Eye = 0%

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10
Q

Histologically, what type of tumor has a poor prognosis?

A

Epithelioid Tumor

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11
Q

Where does an epithelioid metastasize?

A

Liver

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12
Q

What chromosomes are genetic abnormalities found in uveal melanoma?

A

Chromosome 1, 3, 6 and 8

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13
Q

Monosomy 3 is found in what % of uveal melanoma? Why?

A

50%

BAP1 is found on the RNA strand

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14
Q

What are the 3 subgroups for a melanoma?

A 
Class 1A (low risk)
Class 1B (medium risk)
Class 2 (high risk)
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15
Q

What tumor suppressor genes will cause a nevus to become a uveal melanoma?

A
  1. SF3B1
  2. EIF1AX
  3. BAP1
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16
Q

If a patient has SF3B1, what subgroup of uveal melanoma will they be?

A

Class 1B

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17
Q

If a patient has EIF1AX, what subgroup of uveal melanoma will they be in?

A

Class 1A

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18
Q

If a patient has BAP1, what subgroup will they be in?

A

Class 2

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19
Q

PRAME (+) prognosis is usually found with what tumor suppressor gene? When will this patient die?

A

SF3B1

- die later in life, 10 yrs

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20
Q

PRAME (-) prognosis is usually found with what tumor suppressor gene? When will this patient die?

A

EIF1AX

- won’t die

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21
Q

A choroidal nevus is present in what % of caucasians?

A

7.9%

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22
Q

What are the characteristics of a choroidal nevus?

A
  1. Less than 2mm thickness
  2. No relationship to sun exposure
  3. Indistinct borders
  4. Can undergo malignant change into melanoma
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23
Q

As we age, what happens to a choroidal nevus?

A
  1. Nevi increases in number and thickness
  2. Pigment changes (stable)
  3. Metaplasia
  4. Drusen (stable)/Lipofuscin (tumor)
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24
Q

What fraction of persons with a nevus of oto found on the skin or conj, will have a uveal melanoma?

A

1/400 patients

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25
If a pt does not exhibit any risk factors for metaplasia, what % chance will their nevus become a melanoma?
4% chance
26
If a pt has 1 risk factors for metaplasia, what % chance will their nevus become a melanoma?
36% chance
27
If a pt has 3 risk factors for metaplasia, what % chance will their nevus become a melanoma?
50% chance
28
If a pt has 5 risk factors for metaplasia, what % chance will their nevus become a melanoma?
70% chance
29
A b-scan for a uveal melanoma will show what 2 things?
1. Acoustical quiet zone | 2. Choroidal excavation
30
An a-scan for a uveal mealnoma will show what?
1. low to medium reflectivity
31
Class 1A tumors are what % of uveal melanomas? What's the risk of metastasis?
45% | 2% risk of metastasis
32
Class 1B tumors are what % of uveal melanomas? What's the risk of metastasis?
15% | 21%
33
Class 2 tumors are what % of uveal melanomas? What's the risk of metastasis?
40% | 72%
34
What size are small melanomas?
<2.5mm
35
What size are medium melanomas? What is the treatment?
2.5-10mm | Radioactive plaque
36
What size are large melanomas? What's the tx?
> 10mm | Enucleation
37
What is the 10 year mortality rate for large melanomas? Medium? Small?
50% 25% 12%
38
What is the median time of survival after diagnosis of metastasis?
6 months
39
Does a uveal melanoma spread the the lymph or blood?
Blood
40
What are the 4 most common places an uveal melanoma will metastatize to?
Liver, lung, bone, skin/subcutaneous
41
What % of patients with uveal melanoma have another type of cancer?
10%
42
What is the most common cancer to metastasize to the eye? 2nd most common?
Breast - only 85% know they have breast cancer | Lung
43
Breast cancer metastasis tend to be ___ and ___.
bilateral and multiple
44
By the time the tumor is found, what % of pts will have liver metastasis?
5%
45
What % of uveal melanomas are found in the ISNT quadrant of the retina
``` I = 22% S = 22% N = 21% T = 28% ```
46
What % of uveal melanomas are diffuse or found in the macula?
``` Diffuse = 3% Macula = 4-7% ```
47
The most common site for uveal metastasis is what? What %?
Choroid, 90%
48
How accurate is the clinical diagnosis of a melanoma based on the COMS?
99.5%
49
What 2 classes of uveal tumors should be put on adjuvant therapy?
Class2 and 1B
50
Why does a melanoma go to the liver when there's alot of macrophages to kill it?
its immune priviledged
51
What % of uveal melanoma pts have another cancer elsewhere?
10%
52
This adjuvant therapy inhibots proteins that promote cells growth (i.e. MAP K pathway). The goal is to shrink large, proliferating tumors.
Selumetinib
53
This adjuvant therapy is a CTLA-4 blocker which keeps the immune system turned on.
Yervoy (ipilimumab) | - blocks 2nd signal which allows macrophages to kill cancer cells
54
These 2 adjuvant drugs are PDL-1 blockers that go after programmed cell death.
1. Keytruda (pembrolizumab) | 2. Opdivo (Nivolumab)
55
These 2 adjuvant drugs are histone deacetylase (HDAC) inhibitors.
1. Zolinza | 2. Valproic Acid
56
What are the 2 blood supplies to the liver?
1. Hepatic Artery | 2. Portal Vein
57
Give 3 adjuvant therapies if a choroidal melanoma goes to the liver?
1. Chemoembolization to the liver 2. Immuneembolization to the liver 2. Radioembolization to the liver
58
How much longer will a patient live if given embolization adjuvant therapy?
8 more years
59
What is the most deadly location of a uveal melanoma?
Ciliary body
60
Choroidal metastasis tends to come from where in men? women?
``` Men = lungs Women = breasts ```

Posterior Segment (8 decks)

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