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Post. Seg 2 > Retinal Tumors > Flashcards

Retinal Tumors Flashcards

1
Q

This is a focal area in which RPE cells are taller and more densely packed with melanosomes; common benign lesion, not true tumors.

A

CHRPE - congenital hypertrophy of the retinal pigment epithelium

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2
Q

What are the characteristics of a CHRPE?

A
  • Distinct margins
  • near the equator
  • No growth
  • No subretinal fluid
  • Ultrasound will show flat lesion
  • No intrinsic vessels
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3
Q

This is when multiple CHRPE lesions are confined to one sector of the fundus.

A

Bear tracks

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4
Q

What is the systemic association with CHRPEs? What’s the inheritance pattern?

A

Familial Adenomatous Polyposis (FAP)

- autosomal dominant

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5
Q

What % of patients with FAP have atypical CHRPE lesions? What are the shape of the atypical CHRPEs?

A

> 80%

- football shaped or tear drop

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6
Q

When does FAP begin to develop?

A

adolescence (15-40 yo)

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7
Q

If untreated, what will FAP patients develop?

A

Colorectal cancer

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8
Q

This tumor is a creamy-white, placoid choroidal lesion, fast growing, found in PP; exudative RD is common.

A

Choroidal Metastasis

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9
Q

What is the tx plan for choroidal metastasis?

A
  1. Observation
  2. Radiotherapy
  3. TTT
  4. Systemic Therapy
  5. Enucleation
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10
Q

What % of patients with breast cancer metastases will have a known history of breast cancer?

A

85%

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11
Q

What % of breast cancer metastasis to the brain?

A

40%

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12
Q

What % of lung cancer metastasis patients don’t know they have cancer? Is it painful?

A

70%

- painful

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13
Q

This is a benign uveal tumor composed of large, plump, magnocellular nevus cells that are heavily pigmented.

A

Melanocytoma

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14
Q

What are the top 2 groups that melanocytoma presents in?

A
  1. African Americans

2. Females

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15
Q

Where is a melanocytoma found in the retina? What do the margins look like? Color?

A
  • Adjacent to or within the ON
  • feathery margins
  • black
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16
Q

Can an APD be present with a melanocytoma?

A

Yes

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17
Q

What % of melanocytoma cases develop into a melanoma? What’s the treatment?

A
  • 2%

- no treatment needed

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18
Q

This is a rare, benign, congenital tumor. Involves the retina, RPE and overlying vitreous. It has grey pigmentation with superficial gliosis.

A

Combined Hamartoma of the Retina and RPE

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19
Q

What are the 4 possible ocular manifestatins of combined hamartoma of the retina and rpe?

A
  1. Strabismus
  2. Blurred vision
  3. Metamorphopsia
  4. Loss of vision
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20
Q

Where are the lesions of combined hamartoma ?

A
  1. Juxtapapillary
  2. Peripapillary
  3. in PP
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21
Q

What is the systemic association of Combined Harmatoma of Retina & RPE?

A

Neurofibromatosis

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22
Q

If FAP becomes cancerous, what does the patient have?

A

Gardner’s Syndrome

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23
Q

If FAP cancer metastasizes to the brain, what is this calle?

A

Turcot’s syndrome

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24
Q

For a choroidal metastasis, what will the OCT look like?

A
  • dome-shaped elevation of retina and RPE w/ adjacent subretinal fluid
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25
Q

For a choroidal metastasis, what will the Bscan look like?

A

diffuse choroidal thickening

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26
Q

For a choroidal metastasis, what will the FA look like?

A

early hypofluorescence and diffuse, late staining

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27
Q

Metastasis from breast cancer occurs in what % of women at 5 years? And what % of those women have metastasis to the eye?

A
  • 25% at 5 years

- in 12% it goes to the eye

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28
Q

For a melanocytoma, what will the OCT look like?

A
  • disruption of ILM
  • ruined involved retina
  • visualization of subretinal surface
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29
Q

For a melanocytoma, what will the FA look like?

A
  • hypofluorescence of deep vessels due to blockage

- disc margin may stain

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30
Q

What’s the most common primary, intraocular malignancy in childhood?

A

Retinoblastoma

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31
Q

This results from malignant transformation of primitive retinal cells before final differentiation; seldom seen after age 3.

A

Retinoblastoma

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32
Q

What % of retinoblastoma cases are heritable? What other condition can it lead to?

A
  • 70% of cases

- can lead to pinealblastoma

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33
Q

What % of retinoblastoma cases are non-heritable?

A

30%

34
Q

When is the average diagnosis of retinoblastoma? How many new U.S. cases each year?

A

18 months

300 new cases

35
Q

What are the initial features and the % of retinoblastoma?

A
  1. Leukocoria 70%
  2. Strabismus 25%
  3. Other (VA) 5%
36
Q

What % of retinoblastoma cases will present with iris neovascularization?

A

15%

37
Q

What type of pupil will coat’s disease cause?

A

Xanthocoria (yellow pupil)

38
Q

In what % of retinoblastoma cases will be enucleated?

A

75%

39
Q

In what % of retinoblastoma cases will conservative treatment be administered?

A

25%

40
Q

What is the size of the tumors and tx for Group A retinoblastoma?

A
  • Small < 3mm

- Plaque or TTT

41
Q

What is the size of the tumors and tx for Group B,C,D retinoblastoma?

A
  1. Larger > 3mm

2. Chemoreduction (systemically or IV), External Beam Radiation

42
Q

Group C has what type of seeds compared to Group D?

A

Group C = contained seeds

Group D = Diffuse seeds

43
Q

What are the findings in Group E of retinoblastoma?

A
  • Takes up >50% of globe
  • NVI
  • Opaque media
44
Q

What are the findings in Neurofribromatosis Type 1?

A
  • Cafe au lait spots
  • Neurofibromas
  • Lisch nodules
  • Short stature
  • Scoliosis
45
Q

What are the findings in Neurofibromatosis type 2?

A
  • Acoustic neuroma
  • Hearing loss
  • balance issues
  • cataracts
46
Q

For combined hamartoma, what will the OCT look like?

A
  • retinal disorganization and elevation with overlying ERM
47
Q

For combined hamartoma, what will the B-scan/FA look like?

A 
FA = Dx not important
B-scan = pick up depth
48
Q

What glial cells are proliferating in combined hamartoma?

A
  • microglial cells
  • astrocytes
  • mueller cells
49
Q

Choroidal hemangioma is a mass of vascular channels within choroid. What are the 2 types?

A
  1. Circumscribed

2. Diffuse

50
Q

What are the characteristics of a circumscribed choroidal hemangioma?

A
  1. Orange/red mass
  2. indistinct margins
  3. Acoustically solid lesion
  4. Subretinal fluid or macular edema
51
Q

What are the characteristics of a diffuse choroidal hemangioma?

A
  1. Affects over 1/2 of choroid
  2. Enlarges slowly
  3. Deep-red colored fundus
52
Q

What is the disease associated with diffuse choroidal hemangioma?

A

Sturge-weber syndrome

53
Q

What are the systemic findings of SW syndrome and the %?

A
  1. Port Wine Stain 96%
  2. Motor Seizures 85%
  3. Mental Retardation 60%
54
Q

What ocular finding are you worried about if the patient has a PW stain?

A

glaucoma

55
Q

What is a hamartoma?

A

tumor made up of tissue in the same area

56
Q

What is a choristoma?

A

tumor made up of tissue elsewhere (i.e. metastasis)

57
Q

What are the 3 body locations sturge weber syndrome affects?

A
  1. Eye
  2. Skin
  3. CNS
    - called Phacometosis
58
Q

What does the OCT look like for a choroidal hemangioma?

A
  • acute leakage = intact PR layer & normal retinal thickness
  • Chronic leakage = loss of PRs, retinoschisis, retinal edema
59
Q

What does the B scan look like for a choroidal hemangioma?

A
  • Acoustically solid lesion

- High internal reflectivity

60
Q

What does the FA look like for a choroidal hemangioma?

A
  • Spotty hyperfluorescence in early phase

- diffuse hyperfluorescence in later stage

61
Q

This is a benign ossifying tumor of the choroid; composed of mature bone; it may grow

A

Choroidal Osteoma

62
Q

Choroidal osteoma affects bruch’s membrane and can cause what?

A

Choroidal neovascularization net/bleed

63
Q

The VA of choroidal osteoma pts is < 20/200 in what % of patients after 10 years.

A

56-58%

64
Q

What are the typical characteristics of choroidal osteoma?

A
  • orange/yellow
  • unilateral
  • defined
  • juxtapapillary
65
Q

What are the 3 reasons vision loss occurs in choroidal osteoma patients?

A
  1. Subretinal fluid
  2. Neovascularization
  3. PR loss
66
Q

What will the OCT show in a choroidal osteoma px?

A

Inner retina is preserved

67
Q

What will the Bscan show in a choroidal osteoma px?

A
  • highly reflective anterior surface

- orbital shadowing

68
Q

What will the FA show in a choroidal osteoma px?

A

irregular, diffuse hyperfluorescence

69
Q

What is the tx for choroidal osteoma?

A

None

- do not need to work up, no malignant potential

70
Q

This is a vascular and glial tumor of the retina, no threat to vision.

A

Retinal Astrocytic Hamartoma

71
Q

What disease is retinal astrocytic hamartoma associated with?

A

Tuberous sclerosis

72
Q

Define endophytic

A

Growing into the vitreous

73
Q

Define Exophytic

A

Growing into the choroid/subretinal space

74
Q

What % of patients have astrocytomas?

A

50%

75
Q

Where do tumors grow in tuberous sclerosis?

A

benign tumors grow on skin, brain, kidneys and heart

76
Q

What does the OCT show for retinal astrocytic hamartoma?

A
  • Inner retinal thickening
    -Retinal traction (27%)
  • Intratumoral cysts (67%)
    Adjacent edema (47%)
77
Q

What does the FA show for astrocytic hamartoma?

A

Hyperfluorescens w/o leakage

78
Q

What are the markers for breast cancer?

A
  1. Estrogen
  2. Perceptin
  3. Progesterone
79
Q

Is there a systemic association in choroidal osteoma?

A

No

80
Q

What’s the most common treatment for retinoblastoma?

A

Intra-arterial chemotherapy

Post. Seg 2 (4 decks)

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