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Flashcards in Block 16 Tumors Deck (134):
1

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

Neoplasm

2

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

Benign

3

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

Malignant

4

A benign neoplasm of RPE cells is known as _____?

CHRPE

5

What does CHRPE stand for?

Congenital hypertrophy of the RPE

6

Is CHRPE benign or malignant?

Benign

7

Which form of CHRPE is unilateral? (2 Answers)

Typical Solitary and Typical Grouped

8

What systemic condition is associated with Typical CHRPE?

None

9

What systemic condition is associated with Atypical CHRPE?

Familial Adenomatous Polyposis (FAP)

10

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

- Grouped CHRPE
- Pigmented

11

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

Atypical CHRPE

12

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

- Bear tracks (pigmented)
- Polar bear tracks (non-pigmented)

13

What do the lesions look like in Atypical CHRPE?
Where are these commonly found on the retina?

- Elongated oval lesions with a depigmented tail on one end
- Multiple scattered throughout the retina

14

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

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

15

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

Solitary CHRPE

16

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

70-80%

17

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

1. Differentiate from other pigmented fundus lesions
2. Refer to GI specialist for colonoscopy and to examine relatives

18

How should typical CHRPE be managed?

1. Differentiate from other pigmented fundus lesions
2. Document and monitor

19

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

Choroidal melanocytes (pigment-containing cells)

20

What is the most common symptom of a choroidal nevus?

Asymptomatic (incidental finding)

21

When does the growth of a choroidal nevus typically occur?

Before puberty

22

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

1. The lesion itself
2. Subretinal fluid

23

What does "amelanotic" mean when referring to a choroidal nevus?

No pigment

24

Is a choroidal nevus more commonly pigmented or amelanotic?

Pigmented

25

What type of borders are seen on choroidal nevi?

Irregular

26

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

Surface drusen

27

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

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.

28

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

To identify any malignant transformation

29

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

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

30

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

Choroidal Melanocytoma

31

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

Posterior pole

32

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

At or near optic nerve head

33

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

Choroidal nevus: Caucasians
Choroidal melanocytoma: Darkly pigmented individuals

34

What benign tumor may have an associated RAPD?

Choroidal melanocytoma

35

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?

- Near optic disc
- Combined Hamartoma of the Retina and RPE

36

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

Dragging of macula or disc

37

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

Vitrectomy with membrane peeling

38

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

Children or young adults

39

Is a choroidal hemangioma benign or malignant?

Benign

40

Is a choroidal melanocytoma being or malignant?

Benign

41

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

ICGA

42

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

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

43

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

Diffuse Choroidal Hemangioma

44

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

Circumscribed Choroidal Hemangioma

45

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

von Hippel-Lindau (VHL)

46

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

Capillary Hemangioma

47

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?

Capillary Hemangioma
Benign

48

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

Temporal

49

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

Retinal Cavernous Hemangioma
Benign

50

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

Clusters of retinal venous aneurysms

51

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

1. Brain
2. Jaw

52

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

Racemose Hemangioma

53

Why should imaging be done when discovering a Racemose Hemangioma?

To rule out non-ocular arterio-venous malformation

54

Is racemose hemangioma commonly unilateral or bilateral?

Unilateral

55

What symptoms are most often associated with Racemose Hemangioma?

Asymptomatic

56

1. What type of lesion is a retinal vasoproliferative tumor?
2. Benign or malignant?

1. Glio-vascular lesion
2. Benign

57

Are most retinal vasoproliferative tumors primary or secondary?

Primary

58

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

Bilateral

59

Are most retinal vasoproliferative tumors unilateral or bilateral?

Unilateral

60

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

- Infero-temporal periphery
- Single

61

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

Single lesion: Primary VPT (most common)
Multifocal: Secondary VPT (20%)

62

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

1. Bilateral
2. Multifocal
3. Posterior to equator

63

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

1. Retinitis pigmentosa
2. Pars planitis
3. Coat's disease
4. Retinal detachment repair

64

A retinal astrocytoma is composed of what kind of cells?

Retinal glial cells

65

When does a retinal astrocytoma typically appear?

Congenital (born with it)

66

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

Tuberous sclerosis (Bourneville Disease)

67

How is Tuberous sclerosis (Bourneville Disease) often characterized?

Skin, CNS and visceral tumors causing seizures and mental retardation

68

What is the common ocular treatment for Retinal Astrocytoma?

Not typically required

69

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

Retinal detachment

70

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

- Choroidal Osteoma
- Benign

71

How often is a Choroidal Osteoma unilateral?

75%

72

A Choroidal Osteoma is commonly seen in ____?

Otherwise healthy young women

73

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

- Initially: Orange
- Become yellow/white

74

Describe the borders seen on a Choroidal osteoma?

Well-defined scalloped margins

75

When would treatment be required in a choroidal osteoma?

If choroidal neovascularization (CNV) is present

76

What 3 things describes a malignant tumor?

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

77

The most common childhood eye malignancy is ___?

Retinoblastoma (1 in 18,000 infants)

78

Is Retinoblastoma malignant or benign?

Malignant

79

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

- Death
- Direct spread into brain

80

What is the most common presentation of Retinoblastoma?

Leukocoria (white pupil)

81

Is retinoblastoma unilateral or bilateral?

Can be either. If bilateral, it is often multifocal

82

Why does strabismus often develop in Retinoblastoma?

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

83

How does the affected retina appear in Retinoblastoma?

White dome-shaped mass

84

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

Endophytic

85

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

Exophytic

86

What type of testing is required to diagnose Retinoblastoma?

Dilated fundus exam - usually done with anesthesia

87

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

Red-reflex

88

List 5 treatment options for Retinoblastoma.

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

89

What makes up the tumor in Retinoblastoma?

Incompletely differentiated retinal cells = retinoblasts

90

When does Retinoblastoma almost always manifest?

Within first few years of life

91

What does leukocoria mean?

White pupil

92

The most common primary intraocular malignancy in adults is ____?

Choroidal melanoma

93

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

50-60's
Caucasian

94

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

Death

95

What is frequently found on surface of a choroidal melanoma?

Lipofuscin = clumps of orange pigment

96

What is the common clinical appearance of a choroidal melanoma?

Pigmented, dome-shaped sub retinal elevation.
Can also be amelanotic and relatively flat

97

60% of choroidal melanoma's are located _____?

3mm from optic disc

98

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

"Collar stud"

99

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

ICGA

100

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

Needle biopsy

101

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

False

102

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

Brachytherapy

103

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

Liver

104

List 4 treatment options for a choroidal melanoma.

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

105

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

Uveal tract:
Choroid (90%) > Iris > Ciliary body

106

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

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

107

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

Lung and breast

108

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

Macula

109

Are choroidal metastases most often solitary or multifocal?
Unilateral or bilateral?

Solitary and unilateral

110

Describe the typical clinical appearance of a choroidal metastases?

Creamy-white "placoid" lesion at posterior pole; relatively flat

111

What is often a secondary finding to choroidal metastases?

Subretinal exudation

112

How is vision often affected by choroidal metastases?

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

113

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

Primary malignant tumor

114

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

1 year or less

115

A neoplasm of immune system cells is known as ____?

Lymphoma

116

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

Intraocular lymphoma

117

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

- Usually unilateral at first, and eventually becomes bilateral
- Floaters and blurred vision

118

Is vitritis commonly mild or severe in Intraocular Lymphoma?

Severe vitritis

119

Is anterior uveitis commonly mild or severe in Intraocular Lymphoma?

Mild anterior uveitis

120

What is a common feature of vitritis in intraocular lymphoma?

The vitritis does not clear with corticosteroids

121

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

Cystoid macular edema

122

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

A choroidal melanoma breaking through Bruch's membrane

123

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

- Unremitting vitritis
- Intraocular lymphoma

124

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

Multifocal

125

Describe the appearance of intraocular lymphoma.

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

126

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

Intraocular Lymphoma

127

Most patients with primary intraocular lymphoma eventually develop ____ symptoms ?

CNS

128

_____ symptoms may precede eye involvement in an intraocular lymphoma.

CNS

129

Intraocular lymphoma is a subset of ____?

Primary CNS lymphoma

130

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

- Lumbar puncture (CSF evaluation) first

- Vitreous biopsy

131

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

CNS disease

132

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

Radiotherapy

133

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

Chemotherapy

134

List 2 goals of treatment in intraocular lymphoma.

1. Eradicating ocular lymphoma cells
2. Preventing spread to CNS