Retinal Tumors Flashcards by Nishan Pressley

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

CHRPE - congenital hypertrophy of the retinal pigment epithelium

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What are the characteristics of a CHRPE?

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

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

Bear tracks

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

Familial Adenomatous Polyposis (FAP)

- autosomal dominant

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

> 80%

- football shaped or tear drop

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When does FAP begin to develop?

adolescence (15-40 yo)

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If untreated, what will FAP patients develop?

Colorectal cancer

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

Choroidal Metastasis

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What is the tx plan for choroidal metastasis?

Observation
Radiotherapy
TTT
Systemic Therapy
Enucleation

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

85%

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What % of breast cancer metastasis to the brain?

40%

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

70%

- painful

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

Melanocytoma

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What are the top 2 groups that melanocytoma presents in?

African Americans

2. Females

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

Adjacent to or within the ON
feathery margins
black

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Can an APD be present with a melanocytoma?

Yes

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

- no treatment needed

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

Combined Hamartoma of the Retina and RPE

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

Strabismus
Blurred vision
Metamorphopsia
Loss of vision

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Where are the lesions of combined hamartoma ?

Juxtapapillary
Peripapillary
in PP

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

Neurofibromatosis

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If FAP becomes cancerous, what does the patient have?

Gardner’s Syndrome

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

Turcot’s syndrome

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

dome-shaped elevation of retina and RPE w/ adjacent subretinal fluid

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

diffuse choroidal thickening

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

early hypofluorescence and diffuse, late staining

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

- 25% at 5 years | - in 12% it goes to the eye

For a melanocytoma, what will the OCT look like?

- disruption of ILM - ruined involved retina - visualization of subretinal surface

For a melanocytoma, what will the FA look like?

- hypofluorescence of deep vessels due to blockage | - disc margin may stain

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

Retinoblastoma

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

Retinoblastoma

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

- 70% of cases | - can lead to pinealblastoma

What % of retinoblastoma cases are non-heritable?

30%

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

18 months | 300 new cases

What are the initial features and the % of retinoblastoma?

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

What % of retinoblastoma cases will present with iris neovascularization?

15%

What type of pupil will coat's disease cause?

Xanthocoria (yellow pupil)

In what % of retinoblastoma cases will be enucleated?

75%

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

25%

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

- Small < 3mm | - Plaque or TTT

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

1. Larger > 3mm | 2. Chemoreduction (systemically or IV), External Beam Radiation

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

Group C = contained seeds | Group D = Diffuse seeds

What are the findings in Group E of retinoblastoma?

- Takes up >50% of globe - NVI - Opaque media

What are the findings in Neurofribromatosis Type 1?

- Cafe au lait spots - Neurofibromas - Lisch nodules - Short stature - Scoliosis

What are the findings in Neurofibromatosis type 2?

- Acoustic neuroma - Hearing loss - balance issues - cataracts

For combined hamartoma, what will the OCT look like?

- retinal disorganization and elevation with overlying ERM

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

``` FA = Dx not important B-scan = pick up depth ```

What glial cells are proliferating in combined hamartoma?

- microglial cells - astrocytes - mueller cells

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

1. Circumscribed | 2. Diffuse

What are the characteristics of a circumscribed choroidal hemangioma?

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

What are the characteristics of a diffuse choroidal hemangioma?

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

What is the disease associated with diffuse choroidal hemangioma?

Sturge-weber syndrome

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

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

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

glaucoma

What is a hamartoma?

tumor made up of tissue in the same area

What is a choristoma?

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

What are the 3 body locations sturge weber syndrome affects?

1. Eye 2. Skin 3. CNS - called Phacometosis

What does the OCT look like for a choroidal hemangioma?

- acute leakage = intact PR layer & normal retinal thickness - Chronic leakage = loss of PRs, retinoschisis, retinal edema

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

- Acoustically solid lesion | - High internal reflectivity

What does the FA look like for a choroidal hemangioma?

- Spotty hyperfluorescence in early phase | - diffuse hyperfluorescence in later stage

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

Choroidal Osteoma

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

Choroidal neovascularization net/bleed

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

56-58%

What are the typical characteristics of choroidal osteoma?

- orange/yellow - unilateral - defined - juxtapapillary

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

1. Subretinal fluid 2. Neovascularization 3. PR loss

What will the OCT show in a choroidal osteoma px?

Inner retina is preserved

What will the Bscan show in a choroidal osteoma px?

- highly reflective anterior surface | - orbital shadowing

What will the FA show in a choroidal osteoma px?

irregular, diffuse hyperfluorescence

What is the tx for choroidal osteoma?

None | - do not need to work up, no malignant potential

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

Retinal Astrocytic Hamartoma

What disease is retinal astrocytic hamartoma associated with?

Tuberous sclerosis

Define endophytic

Growing into the vitreous

Define Exophytic

Growing into the choroid/subretinal space

What % of patients have astrocytomas?

50%

Where do tumors grow in tuberous sclerosis?

benign tumors grow on skin, brain, kidneys and heart

What does the OCT show for retinal astrocytic hamartoma?

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

What does the FA show for astrocytic hamartoma?

Hyperfluorescens w/o leakage

What are the markers for breast cancer?

1. Estrogen 2. Perceptin 3. Progesterone

Is there a systemic association in choroidal osteoma?

What's the most common treatment for retinoblastoma?

Intra-arterial chemotherapy