Block 16 Tumors Flashcards

1
Q

An abnormal growth of tissue that exceeds and is uncoordinated with the tissues around it is called a ______?

A

Neoplasm

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2
Q

A tumor that does not grow uncontrollably, invade neighboring tissues or spread throughout body is ______?

A

Benign

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3
Q

A tumor that grows uncontrollably, invades nearby tissues and may spread throughout the body is _____?

A

Malignant

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4
Q

A benign neoplasm of RPE cells is known as _____?

A

CHRPE

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5
Q

What does CHRPE stand for?

A

Congenital hypertrophy of the RPE

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6
Q

Is CHRPE benign or malignant?

A

Benign

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7
Q

Which form of CHRPE is unilateral? (2 Answers)

A

Typical Solitary and Typical Grouped

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8
Q

What systemic condition is associated with Typical CHRPE?

A

None

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9
Q

What systemic condition is associated with Atypical CHRPE?

A

Familial Adenomatous Polyposis (FAP)

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10
Q

What condition has fundus lesions described as “bear tracks”? Are the lesions pigmented or non-pigmented?

A
  • Grouped CHRPE

- Pigmented

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11
Q

Which benign tumor is associated with precancerous colorectal polyps in teenagers?

A

Atypical CHRPE

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12
Q

What are two terms that describe the different appearances of multifocal lesions in the more common form of benign neoplasms of RPE cells?

A
  • Bear tracks (pigmented)

- Polar bear tracks (non-pigmented)

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13
Q

What do the lesions look like in Atypical CHRPE?

Where are these commonly found on the retina?

A
  • Elongated oval lesions with a depigmented tail on one end

- Multiple scattered throughout the retina

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14
Q

What are the common sizes of lesions found in both Solitary CHRPE and Grouped CHRPE?

A

Solitary: 1-6 mm (Average is 4-6)
Grouped: 0.1-2 mm

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15
Q

What condition is described as having hypopigmented halos and lacunae scattered within the lesion as it matures?

A

Solitary CHRPE

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16
Q

What percent of patients with Familial Adenomatous Polyposis have characteristic fundus lesions present at birth?

A

70-80%

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17
Q

When discovering multiple comma-shaped lesions with depigmentation on one end scattered throughout the retina, what is the role of an optometrist?

A
  1. Differentiate from other pigmented fundus lesions

2. Refer to GI specialist for colonoscopy and to examine relatives

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18
Q

How should typical CHRPE be managed?

A
  1. Differentiate from other pigmented fundus lesions

2. Document and monitor

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19
Q

What type of cells exhibit abnormal growth in an choroidal nevus?

A

Choroidal melanocytes (pigment-containing cells)

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20
Q

What is the most common symptom of a choroidal nevus?

A

Asymptomatic (incidental finding)

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21
Q

When does the growth of a choroidal nevus typically occur?

A

Before puberty

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22
Q

What two factors could affect the macula cause vision impairment with a choroidal nevus?

A
  1. The lesion itself

2. Subretinal fluid

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23
Q

What does “amelanotic” mean when referring to a choroidal nevus?

A

No pigment

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24
Q

Is a choroidal nevus more commonly pigmented or amelanotic?

A

Pigmented

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25
Q

What type of borders are seen on choroidal nevi?

A

Irregular

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26
Q

What additional sign is seen when a choroidal nevus is chronic?

A

Surface drusen

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27
Q

What examination technique can be used to differentiate a choroidal nevus from a CHRPE?

A

Red-free filter (green light) to confirm choroidal location for nevus (CHRPE is RPE cells) - Red free filter will diminish appearance of choroidal nevus. A red filter will increase visibility of choroidal nevus.

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28
Q

Why is it important to document and monitor a choroidal nevus?

A

To identify any malignant transformation

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29
Q

List 4 suspicious findings on a choroidal nevus that point to possible malignant transformation?

A
  1. Increase in size or thickness beyond puberty
  2. Subretinal fluid
  3. Lipofuscin (orange pigment)
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30
Q

What benign tumor could be a variant of a choroidal nevus?

A

Choroidal Melanocytoma

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31
Q

What area of the retina does a choroidal nevus typically favor?

A

Posterior pole

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32
Q

What area of the retina is a choroidal melanocytoma commonly found?

A

At or near optic nerve head

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33
Q

Who is more commonly affected by a choroidal nevus vs a choroidal melanocytoma?

A

Choroidal nevus: Caucasians

Choroidal melanocytoma: Darkly pigmented individuals

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34
Q

What benign tumor may have an associated RAPD?

A

Choroidal melanocytoma

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35
Q

The retinal lesion from a benign congenital tumor composed of various cell types form both the retina and RPE is often located ____?
What condition is this called?

A
  • Near optic disc

- Combined Hamartoma of the Retina and RPE

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36
Q

What can cause visual distortion in Combined Hamartoma of Retina and RPE cells?

A

Dragging of macula or disc

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37
Q

If Combined Hamartoma of Retina and RPE cells becomes symptomatic, what is the best treatment?

A

Vitrectomy with membrane peeling

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38
Q

A benign choroidal tumor consisting of varying-sized vascular channels is often diagnosed in what age group?

A

Children or young adults

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39
Q

Is a choroidal hemangioma benign or malignant?

A

Benign

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40
Q

Is a choroidal melanocytoma being or malignant?

A

Benign

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41
Q

What diagnostic test can best show tumor vessels in circumscribed choroidal hemangioma?

A

ICGA

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42
Q

In a case where a choroidal hemangioma is not asymptomatic, what may be the cause of the impaired vision?

A

Macular distortion due to the tumor itself or a exudative retinal detachment

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43
Q

A benign choroidal vascular tumor found in a patient with Sturge-Weber Syndrome is most likely ____?

A

Diffuse Choroidal Hemangioma

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44
Q

A benign choroidal vascular tumor found in a patient with no systemic association is most likely ____?

A

Circumscribed Choroidal Hemangioma

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45
Q

A benign vascular tumor of retinal capillary bed is most commonly associated with what systemic condition?

A

von Hippel-Lindau (VHL)

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46
Q

60% of patients with VHL display what type of tumor that has sight and light threatening complications?

A

Capillary Hemangioma

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47
Q

Dilation and tortuosity of the supplying artery and draining vein extending from the optic disc is associated with which ocular tumor? Is it benign or malignant?

A

Capillary Hemangioma

Benign

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48
Q

Are lesions typically nasal or temporal to the optic disc in a capillary hemangioma?

A

Temporal

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49
Q

A “bunch of grapes” on the surface of the retina describes which ocular tumor? Is it benign or malignant?

A

Retinal Cavernous Hemangioma

Benign

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50
Q

What gives a retinal cavernous hemangioma its “bunch of grapes” appearance?

A

Clusters of retinal venous aneurysms

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51
Q

A congenital arterio-venous malformation of retinal vasculature is sometimes accompanied by similar lesions in what 2 locations?

A
  1. Brain

2. Jaw

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52
Q

Direct communication between enlarged and tortuous retinal veins and arteries is caused by ___?

A

Racemose Hemangioma

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53
Q

Why should imaging be done when discovering a Racemose Hemangioma?

A

To rule out non-ocular arterio-venous malformation

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54
Q

Is racemose hemangioma commonly unilateral or bilateral?

A

Unilateral

55
Q

What symptoms are most often associated with Racemose Hemangioma?

A

Asymptomatic

56
Q
  1. What type of lesion is a retinal vasoproliferative tumor?

2. Benign or malignant?

A
  1. Glio-vascular lesion

2. Benign

57
Q

Are most retinal vasoproliferative tumors primary or secondary?

A

Primary

58
Q

Are retinal vasoproliferative tumors that are secondary to another condition unilateral or bilateral?

A

Bilateral

59
Q

Are most retinal vasoproliferative tumors unilateral or bilateral?

A

Unilateral

60
Q

Where are lesions from retinal vasoproliferative tumors most often found? Are they most often single or multifocal?

A
  • Infero-temporal periphery

- Single

61
Q

When is there a single lesion vs multifocal lesions due to a retinal vasoproliferative tumor?

A

Single lesion: Primary VPT (most common)

Multifocal: Secondary VPT (20%)

62
Q

What are 3 ways a mass is different when the retinal vasoproliferative tumor is secondary to another condition (rather than primary)?

A
  1. Bilateral
  2. Multifocal
  3. Posterior to equator
63
Q

What are 4 examples of conditions that a retinal vasoproliferative tumor could be secondary to?

A
  1. Retinitis pigmentosa
  2. Pars planitis
  3. Coat’s disease
  4. Retinal detachment repair
64
Q

A retinal astrocytoma is composed of what kind of cells?

A

Retinal glial cells

65
Q

When does a retinal astrocytoma typically appear?

A

Congenital (born with it)

66
Q

What systemic association is commonly associated with a benign congenital tumor or retinal glial cells?

A

Tuberous sclerosis (Bourneville Disease)

67
Q

How is Tuberous sclerosis (Bourneville Disease) often characterized?

A

Skin, CNS and visceral tumors causing seizures and mental retardation

68
Q

What is the common ocular treatment for Retinal Astrocytoma?

A

Not typically required

69
Q

What may be a reason ocular treatment is needed in Retinal Astrocytoma?

A

Retinal detachment

70
Q

A tumor consisting of mature bone within the choroid and atrophied RPE above it describes ______?
Benign or malignant?

A
  • Choroidal Osteoma

- Benign

71
Q

How often is a Choroidal Osteoma unilateral?

A

75%

72
Q

A Choroidal Osteoma is commonly seen in ____?

A

Otherwise healthy young women

73
Q

What color do lesions initially appear on a choroidal osteoma? What color do they become?

A
  • Initially: Orange

- Become yellow/white

74
Q

Describe the borders seen on a Choroidal osteoma?

A

Well-defined scalloped margins

75
Q

When would treatment be required in a choroidal osteoma?

A

If choroidal neovascularization (CNV) is present

76
Q

What 3 things describes a malignant tumor?

A
  1. Grows uncontrollably
  2. Invades nearby tissues
  3. May spread throughout body
77
Q

The most common childhood eye malignancy is ___?

A

Retinoblastoma (1 in 18,000 infants)

78
Q

Is Retinoblastoma malignant or benign?

A

Malignant

79
Q

What is the most likely outcome of Retinoblastoma if left untreated? Why?

A
  • Death

- Direct spread into brain

80
Q

What is the most common presentation of Retinoblastoma?

A

Leukocoria (white pupil)

81
Q

Is retinoblastoma unilateral or bilateral?

A

Can be either. If bilateral, it is often multifocal

82
Q

Why does strabismus often develop in Retinoblastoma?

A

No sensory information is being received by affected eye, so the eye turns from lack of use

83
Q

How does the affected retina appear in Retinoblastoma?

A

White dome-shaped mass

84
Q

In Retinoblastoma, what is it called if the white mass extends into the vitreous?

A

Endophytic

85
Q

In Retinoblastoma, what is it called if the white mass extends beneath the retina?

A

Exophytic

86
Q

What type of testing is required to diagnose Retinoblastoma?

A

Dilated fundus exam - usually done with anesthesia

87
Q

What type of testing is used as a screening for Retinoblastoma?

A

Red-reflex

88
Q

List 5 treatment options for Retinoblastoma.

A
  1. Photocoagulation (laser)
  2. Cryotherapy (freezing)
  3. Chemotherapy (targets highly mitotic cells)
  4. Various types of radiotherapy (irradiation)
  5. Enucleation
89
Q

What makes up the tumor in Retinoblastoma?

A

Incompletely differentiated retinal cells = retinoblasts

90
Q

When does Retinoblastoma almost always manifest?

A

Within first few years of life

91
Q

What does leukocoria mean?

A

White pupil

92
Q

The most common primary intraocular malignancy in adults is ____?

A

Choroidal melanoma

93
Q

Choroidal melanoma is more common in what age group? What race?

A

50-60’s

Caucasian

94
Q

What is often the result if a choroidal melanoma metastasizes from the eye to other organs?

A

Death

95
Q

What is frequently found on surface of a choroidal melanoma?

A

Lipofuscin = clumps of orange pigment

96
Q

What is the common clinical appearance of a choroidal melanoma?

A

Pigmented, dome-shaped sub retinal elevation.

Can also be amelanotic and relatively flat

97
Q

60% of choroidal melanoma’s are located _____?

A

3mm from optic disc

98
Q

If a choroidal melanoma breaks through Bruch’s membrane, how is the appearance described?

A

“Collar stud”

99
Q

What type of angiography is useful in diagnosing a choroidal melanoma?

A

ICGA

100
Q

What test can be used to aid in determining the prognosis for a choroidal melanoma?

A

Needle biopsy

101
Q

True or false: Treatment of a choroidal melanoma is always required

A

False

102
Q

Which method of irradiation is most commonly used to treat a choroidal melanoma?

A

Brachytherapy

103
Q

Which organ does a choroidal melanoma commonly spread to (if it spreads at all)?

A

Liver

104
Q

List 4 treatment options for a choroidal melanoma.

A
  1. Irradiation (brachytherapy most often)
  2. Transpupillary thermotherapy (TTT)
  3. Resection (rare)
  4. Enucleation
105
Q

The most common type of intraocular malignancy almost always occurs where within the eye?

A

Uveal tract:

Choroid (90%) > Iris > Ciliary body

106
Q

The most common type of intraocular malignancy is a primary or secondary tumor?

A

Secondary (primary tumor is elsewhere and travelled to eye via bloodstream)

107
Q

What are the most common primary sites of metastatic tumors that spread to choroid?

A

Lung and breast

108
Q

What portion of the macula is usually affected by choroidal metastases?

A

Macula

109
Q

Are choroidal metastases most often solitary or multifocal?

Unilateral or bilateral?

A

Solitary and unilateral

110
Q

Describe the typical clinical appearance of a choroidal metastases?

A

Creamy-white “placoid” lesion at posterior pole; relatively flat

111
Q

What is often a secondary finding to choroidal metastases?

A

Subretinal exudation

112
Q

How is vision often affected by choroidal metastases?

A

Distorted - due to location of lesion at macula/posterior pole

113
Q

2/3 of patients with choroidal metastases have a history of ____?

A

Primary malignant tumor

114
Q

How long do most patients have to live when choroidal metastases from a primary tumor is discovered?

A

1 year or less

115
Q

A neoplasm of immune system cells is known as ____?

A

Lymphoma

116
Q

A malignant proliferation of white blood cells within the eye is called _____?

A

Intraocular lymphoma

117
Q

Do most cases of intraocular lymphoma initially have unilateral or bilateral symptoms? What are the symptoms?

A
  • Usually unilateral at first, and eventually becomes bilateral
  • Floaters and blurred vision
118
Q

Is vitritis commonly mild or severe in Intraocular Lymphoma?

A

Severe vitritis

119
Q

Is anterior uveitis commonly mild or severe in Intraocular Lymphoma?

A

Mild anterior uveitis

120
Q

What is a common feature of vitritis in intraocular lymphoma?

A

The vitritis does not clear with corticosteroids

121
Q

What is often a result of severe, unremitting vitritis - but does NOT occur in intraocular lymphoma?

A

Cystoid macular edema

122
Q

“Collar stud” appearance on the retina occurs due to _______ in the most common primary intraocular malignancy in adults?

A

A choroidal melanoma breaking through Bruch’s membrane

123
Q

Cystoid macular edema does NOT occur despite unremitting _______ in which condition?

A
  • Unremitting vitritis

- Intraocular lymphoma

124
Q

Is the appearance of a Intraocular Lymphoma on the retina multifocal or single?

A

Multifocal

125
Q

Describe the appearance of intraocular lymphoma.

A

Multiple yellow-white sub retinal infiltrates that enlarge and coalesce.
(plus severe vitritis, mild anterior uveitis, and no CME)

126
Q

What diagnosis should be considered in any elderly patient with recalcitrant posterior uveitis?

A

Intraocular Lymphoma

127
Q

Most patients with primary intraocular lymphoma eventually develop ____ symptoms ?

A

CNS

128
Q

_____ symptoms may precede eye involvement in an intraocular lymphoma.

A

CNS

129
Q

Intraocular lymphoma is a subset of ____?

A

Primary CNS lymphoma

130
Q

When attempting to diagnose intraocular lymphoma, National Eye Institute recommends to perform ________ first, before performing _______.

A
  • Lumbar puncture (CSF evaluation) first

- Vitreous biopsy

131
Q

What is the reason most patients die with primary intraocular lymphoma?

A

CNS disease

132
Q

What type of treatment is often used for ophthalmic manifestations of ocular lymphoma?

A

Radiotherapy

133
Q

What type of treatment is often used for CNS involvement of ocular lymphoma?

A

Chemotherapy

134
Q

List 2 goals of treatment in intraocular lymphoma.

A
  1. Eradicating ocular lymphoma cells

2. Preventing spread to CNS