Uveal Melanomas Flashcards

1
Q

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

A

> 95%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

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

A

GNAQ and GNA11

- 80%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the most common primary intraocular malignancy?

A

Uveal melanoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the second most common form of melanoma?

A

Uveal melanoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

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

A

2,000-2,500

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What percent of patients die from melanoma in 1 year?

A

50%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

60-70 yo

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the 3 main treatments for the uveal melanoma?

A
  1. Radiation Therapy
  2. Anti-VEGF
  3. Thermotherapy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

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

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Histologically, what type of tumor has a poor prognosis?

A

Epithelioid Tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Where does an epithelioid metastasize?

A

Liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What chromosomes are genetic abnormalities found in uveal melanoma?

A

Chromosome 1, 3, 6 and 8

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

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

A

50%

BAP1 is found on the RNA strand

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the 3 subgroups for a melanoma?

A
Class 1A (low risk)
Class 1B (medium risk)
Class 2 (high risk)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

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

A
  1. SF3B1
  2. EIF1AX
  3. BAP1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

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

A

Class 1B

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

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

A

Class 1A

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

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

A

Class 2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

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

A

EIF1AX

- won’t die

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

A choroidal nevus is present in what % of caucasians?

A

7.9%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

If a pt does not exhibit any risk factors for metaplasia, what % chance will their nevus become a melanoma?

A

4% chance

26
Q

If a pt has 1 risk factors for metaplasia, what % chance will their nevus become a melanoma?

A

36% chance

27
Q

If a pt has 3 risk factors for metaplasia, what % chance will their nevus become a melanoma?

A

50% chance

28
Q

If a pt has 5 risk factors for metaplasia, what % chance will their nevus become a melanoma?

A

70% chance

29
Q

A b-scan for a uveal melanoma will show what 2 things?

A
  1. Acoustical quiet zone

2. Choroidal excavation

30
Q

An a-scan for a uveal mealnoma will show what?

A
  1. low to medium reflectivity
31
Q

Class 1A tumors are what % of uveal melanomas? What’s the risk of metastasis?

A

45%

2% risk of metastasis

32
Q

Class 1B tumors are what % of uveal melanomas? What’s the risk of metastasis?

A

15%

21%

33
Q

Class 2 tumors are what % of uveal melanomas? What’s the risk of metastasis?

A

40%

72%

34
Q

What size are small melanomas?

A

<2.5mm

35
Q

What size are medium melanomas? What is the treatment?

A

2.5-10mm

Radioactive plaque

36
Q

What size are large melanomas? What’s the tx?

A

> 10mm

Enucleation

37
Q

What is the 10 year mortality rate for large melanomas? Medium? Small?

A

50%
25%
12%

38
Q

What is the median time of survival after diagnosis of metastasis?

A

6 months

39
Q

Does a uveal melanoma spread the the lymph or blood?

A

Blood

40
Q

What are the 4 most common places an uveal melanoma will metastatize to?

A

Liver, lung, bone, skin/subcutaneous

41
Q

What % of patients with uveal melanoma have another type of cancer?

A

10%

42
Q

What is the most common cancer to metastasize to the eye? 2nd most common?

A

Breast - only 85% know they have breast cancer

Lung

43
Q

Breast cancer metastasis tend to be ___ and ___.

A

bilateral and multiple

44
Q

By the time the tumor is found, what % of pts will have liver metastasis?

A

5%

45
Q

What % of uveal melanomas are found in the ISNT quadrant of the retina

A
I = 22%
S = 22%
N = 21%
T = 28%
46
Q

What % of uveal melanomas are diffuse or found in the macula?

A
Diffuse = 3%
Macula = 4-7%
47
Q

The most common site for uveal metastasis is what? What %?

A

Choroid, 90%

48
Q

How accurate is the clinical diagnosis of a melanoma based on the COMS?

A

99.5%

49
Q

What 2 classes of uveal tumors should be put on adjuvant therapy?

A

Class2 and 1B

50
Q

Why does a melanoma go to the liver when there’s alot of macrophages to kill it?

A

its immune priviledged

51
Q

What % of uveal melanoma pts have another cancer elsewhere?

A

10%

52
Q

This adjuvant therapy inhibots proteins that promote cells growth (i.e. MAP K pathway). The goal is to shrink large, proliferating tumors.

A

Selumetinib

53
Q

This adjuvant therapy is a CTLA-4 blocker which keeps the immune system turned on.

A

Yervoy (ipilimumab)

- blocks 2nd signal which allows macrophages to kill cancer cells

54
Q

These 2 adjuvant drugs are PDL-1 blockers that go after programmed cell death.

A
  1. Keytruda (pembrolizumab)

2. Opdivo (Nivolumab)

55
Q

These 2 adjuvant drugs are histone deacetylase (HDAC) inhibitors.

A
  1. Zolinza

2. Valproic Acid

56
Q

What are the 2 blood supplies to the liver?

A
  1. Hepatic Artery

2. Portal Vein

57
Q

Give 3 adjuvant therapies if a choroidal melanoma goes to the liver?

A
  1. Chemoembolization to the liver
  2. Immuneembolization to the liver
  3. Radioembolization to the liver
58
Q

How much longer will a patient live if given embolization adjuvant therapy?

A

8 more years

59
Q

What is the most deadly location of a uveal melanoma?

A

Ciliary body

60
Q

Choroidal metastasis tends to come from where in men? women?

A
Men = lungs
Women = breasts