Iris and Ciliary Body: Benign and Malignant Tumors Flashcards

1
Q

iris cyst:

  • benign or malignant?
  • cyst originating from the ____
A

benign;

iris epithelium or stroma

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2
Q

iris cyst etiology/associations

A
  • idiopathic
  • topical prostaglandins and miotics
  • trauma or surgery
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3
Q

iris cyst demographics

A

no predilection for any patient population

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4
Q

iris cyst laterality

A

unilateral

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5
Q

iris cyst symptoms

A
  • asymptomatic
  • visible iris growth if stromal
  • blurred vision
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6
Q

iris cyst signs: epithelial

A
  • solitary, smooth, dome-shaped elevation of the iris
  • unable to view cyst unless in the pupil
  • may extend into the pupil: appears as a darkly pigmented lesion that transilluminates, may cause pupillary distortion and occlusion of the visual axis
  • may detach and float freely in the anterior or vitreous chamber
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7
Q

iris cyst signs: stromal

A
  • solitary, smooth, translucent lesion on the surface of the iris
  • transilluminates
  • may extend into the pupil: may cause pupillary distortion and occlusion of the visual axis
  • may detach and float freely in the anterior chamber
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8
Q

iris cyst complications

A

secondary glaucoma: large cyst may block the TM

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9
Q

iris cyst management

A
  • monitor if asymptomatic and no complications: yearly or RTC prn if changes in vision; SL exam, ASeg photos, gonio; ASeg OCT or UBM if available
  • refer out if affecting vision or blocking the TM: general OMD or glaucoma specialist
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10
Q

iris cyst clinical pearls:

  • may remain stable for years before ____
  • ____ can aid in differentiating between iris cyst, nevus, and melanoma (malignant)
  • ____ cysts are more common than ____ cysts
  • ____% of iris epithelial cysts are in the peripheral iris
A

enlarging;
gonioscopy, ASeg OCT, and UBM;
epithelial; stromal;
75

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11
Q

iris epithelial cysts may be congenital:

  • known as ____
  • bilateral, multiple cysts at ____
  • may ____ over time
A

iris floccule;
the pupillary margin;
enlarge and shrink

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12
Q

iris ephelis (freckle):

  • benign or malignant?
  • ____ in the iris stroma
A

benign;

excess pigmentation

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13
Q

iris ephelis (freckle) etiology/associations

A
  • excess melanin

- associated with chronic UV exposure

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14
Q

iris ephelis (freckle) demographics

A

more common in light-colored irises

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15
Q

iris ephelis (freckle) laterality

A

unilateral or bilateral

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16
Q

iris ephelis (freckle) symptoms

A

asymptomatic

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17
Q

iris ephelis (freckle) signs

A
  • visible discoloration of the iris
  • tan to brown, flat circumscribed pigmentation on the anterior iris surface
  • may occur in one or multiple areas
  • typically inferior and small size
  • normal iris architecture
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18
Q

iris ephelis (freckle) management

A
  • monitor with routine exam

- consider baseline ASeg photos

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19
Q

iris ephelis (freckle) clinical pearls:

  • no ____ potential
  • occurs in ____% of people
A

malignant;

~60

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20
Q

iris nevus:

  • _____ tumor of the iris stroma
  • most common ____ in all age groups
A

benign;

solid iris tumor

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21
Q

iris nevus etiology

A
  • proliferation of melanocytes

- associated with chronic UV exposure

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22
Q

iris nevus demographics

A
  • often appear during puberty

- more common in light-colored irises

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23
Q

iris nevus laterality

A

unilateral or bilateral

24
Q

iris nevus symptoms

A

asymptomatic

25
Q

iris nevus signs

A
  • visible discoloration of the iris
  • tan to brown, flat or slightly elevated, circumscribed lesion in the iris stroma; may be amelanotic
  • one or multiple lesions may be present
  • typically inferior, < 3 mm in diameter, and < 1 mm thick
  • occasionally causes pupillary peaking and ectropian uveae; congenital vs. change over time (progressive)
26
Q

iris nevus complications

A
  • malignant potential
  • most important sign is documented growth (however, may increase in size during puberty)
  • use pertinent negatives as a guide!
27
Q

iris nevus management

A
  • monitor yearly for malignant tendencies –> melanoma: SL exam, ASeg photos, gonio; ASeg OCT or UBM if available
  • if changes in nevus size or shape, iris, or pupil deformity: refer out (general OMD or oculoplastics)
28
Q

transformation of iris nevus to melanoma:

  • occurred in ___% of patients within 10 years of nevus development
  • occurred in ___% of patients within 20 years of nevus development
A

4;

11

29
Q

risk factors for iris nevus growth into melanoma

A
A= age (young)
B= blood
C= clock hours (inferior)
D= diffuse configuration
E= ectropian (of iris or uvea)
F= feathery margin
30
Q

iris nevus clinical pearls:

  • occurs in ____% of people
  • iris nevi are ____ than iris freckles
  • ____ can aid in differentiating between iris cyst, nevus, and melanoma (malignant)
  • follow up is _____ because ____
A
~4-6;
larger and deeper;
gonioscopy, ASeg OCT, and UBM;
life-long; 
growth may occur after several years of apparent inactivity, malignant potential
31
Q

uncommon variants of iris nevi

A
  • diffuse nevus

- Lisch nodules

32
Q

diffuse nevus

A

flat with indistinct margins; typically occurs in patients with congenital ocular melanocytosis

33
Q

Lisch nodules

A

small bilateral iris nevi in patients with NF1

34
Q

ocular melanocytosis:

  • ____ iris nevus
  • ____ melanocytosis most common
  • ____ nevus
  • risk of ____
A

diffuse;
scleral or choroidal;
blue;
melanoma and glaucoma

35
Q

iris melanoma:

____ tumor of the iris stroma

A

malignant

36
Q

iris melanoma etiology

A
  • proliferation of atypical melanocytes
  • associated with chronic UV exposure
  • associated with changes to iris nevus
37
Q

iris melanoma demographics

A
  • typically occurs between the ages of 50-60 years

- more common in Caucasians with a light-colored iris

38
Q

iris melanoma laterality

A

unilateral

39
Q

iris melanoma symptoms

A
  • asymptomatic
  • ocular complications and visual disturbances
  • systemic complications (metastatic)
40
Q

iris melanoma signs

A
  • visible spot or discoloration of the iris or enlargement of a preexisting iris lesion
  • tan to brown, elevated lesion in the iris stroma and possibly extending to the CB
  • may be amelanotic
  • may be localized or diffuse
  • typically inferior, > 3 mm in diameter, and > 1 mm thick
  • often associated with intralesional blood vessels and a sectoral cataract
  • occasionally causes pupillary peaking and acquired ectropian uveae
41
Q

iris melanoma complications

A
  • intraocular tissue and orbital extension, iris deformity
  • metastasis
  • hyphema: intralesional vessels may leak blood into the AC
  • secondary glaucoma: tumor cells or liberated pigment may block the TM
42
Q

iris melanoma metastasis:

  • ____% develop metastasis within 10 years of treatment
  • may also present in tandem with ____
A

5;

systemic cancers

43
Q

if there is no metastasis with iris melanoma:

  • ____% survival rate if melanoma is confined to the iris only
  • ____% survival rate as melanoma involves CB and choroid
  • ____% if involves extrascleral structures
A

100;
90;
50

44
Q

iris melanoma management

A
  • refer out to OMD, preferably ocular oncologist

- may need blood work and/or systemic care

45
Q

iris melanoma treatment

A
  • iridectomy/excision (for small tumors): 3-4 clock hours in size, without “seeding”
  • radiotherapy with a readioactive or external beam (for large tumors): >3-4 clock hours, with “seeding”
  • enucleation (diffusely growing tumors, or if radiotherapy contraindicated): uncontrollable secondary glaucoma
46
Q

iris melanoma clinical pearls:

  • ____ can aid in differentiating between iris cyst, nevus, and melanoma
  • can use the _____ rule to help with clinical eval
  • DFE is a must to _____
  • grows _____
  • least common site of melanoma: ____
  • most common site of melanoma: ____
A
gonioscopy, ASeg OCT, and UBM;
iris nevus A, B, C, D, E;
rule out posterior findings;
relatively slowly;
uveal (~5%);
choroid (85%)
47
Q

ciliary body melanoma:

-____ ciliary body tumor

A

malignant

48
Q

ciliary body melanoma etiology/associations

A

proliferation of atypical melanocytes

49
Q

ciliary body melanoma demographics

A
  • typically occurs over the age of 50 years

- more common in Caucasians with a light-colored iris

50
Q

ciliary body melanoma laterality

A

unilateral

51
Q

ciliary body melanoma symptoms

A

asymptomatic

52
Q

ciliary body melanoma signs

A
  • smooth, dome-shaped elevation of the iris; may be visualized as an amelanotic or dark brown mass on fundoscopy or gonioscopy following dilation
  • sentinel vessel (dilated episcleral vessel) in the same quadrant as the tumor
  • may extend through the sclera and become visible as a dark epibulbar nodule
  • higher IOP than the fellow eye
  • pressure on the lens may give rise to astigmatism, subluxation, or cataract
53
Q

ciliary body melanoma complications

A
  • intraocular tissue and orbital extension
  • metastasis: 10 year mortality rate is 30-50% due to metastasis
  • secondary glaucoma: tumor cells or liberated pigment may block the TM or the tumor may push the iris root anterior and narrow the angle
54
Q

ciliary body melanoma management

A
  • refer out to OMD, preferably ocular oncologist

- may need blood work and/or systemic care

55
Q

ciliary body melanoma treatment

A
  • iridocyclectomy (small, local tumors)
  • radiotherapy with a radioactive plaque or external irradiation with a proton beam (medium tumors, preferably local)
  • enucleation (large tumor)
  • exenteration (if extrascleral extension into the orbit)
56
Q

ciliary body melanoma clinical pearls:

  • ciliary body melanomas comprise ____% of all uveal melanomas
  • _____ prognosis of the uveal melanomas
  • since CB melanomas are hidden behind the iris, they usually _____
  • ___ can aid in viewing a ciliary body melanoma
A

~10;
worst;
attain a relatively large size before detected clinically;
gonioscopy through a well-dilated pupil and UBM