Iris and Ciliary Body: Benign and Malignant Tumors Flashcards
iris cyst:
- benign or malignant?
- cyst originating from the ____
benign;
iris epithelium or stroma
iris cyst etiology/associations
- idiopathic
- topical prostaglandins and miotics
- trauma or surgery
iris cyst demographics
no predilection for any patient population
iris cyst laterality
unilateral
iris cyst symptoms
- asymptomatic
- visible iris growth if stromal
- blurred vision
iris cyst signs: epithelial
- 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
iris cyst signs: stromal
- 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
iris cyst complications
secondary glaucoma: large cyst may block the TM
iris cyst management
- 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
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
enlarging;
gonioscopy, ASeg OCT, and UBM;
epithelial; stromal;
75
iris epithelial cysts may be congenital:
- known as ____
- bilateral, multiple cysts at ____
- may ____ over time
iris floccule;
the pupillary margin;
enlarge and shrink
iris ephelis (freckle):
- benign or malignant?
- ____ in the iris stroma
benign;
excess pigmentation
iris ephelis (freckle) etiology/associations
- excess melanin
- associated with chronic UV exposure
iris ephelis (freckle) demographics
more common in light-colored irises
iris ephelis (freckle) laterality
unilateral or bilateral
iris ephelis (freckle) symptoms
asymptomatic
iris ephelis (freckle) signs
- 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
iris ephelis (freckle) management
- monitor with routine exam
- consider baseline ASeg photos
iris ephelis (freckle) clinical pearls:
- no ____ potential
- occurs in ____% of people
malignant;
~60
iris nevus:
- _____ tumor of the iris stroma
- most common ____ in all age groups
benign;
solid iris tumor
iris nevus etiology
- proliferation of melanocytes
- associated with chronic UV exposure
iris nevus demographics
- often appear during puberty
- more common in light-colored irises
iris nevus laterality
unilateral or bilateral
iris nevus symptoms
asymptomatic
iris nevus signs
- 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)
iris nevus complications
- malignant potential
- most important sign is documented growth (however, may increase in size during puberty)
- use pertinent negatives as a guide!
iris nevus management
- 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)
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
4;
11
risk factors for iris nevus growth into melanoma
A= age (young) B= blood C= clock hours (inferior) D= diffuse configuration E= ectropian (of iris or uvea) F= feathery margin
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 ____
~4-6; larger and deeper; gonioscopy, ASeg OCT, and UBM; life-long; growth may occur after several years of apparent inactivity, malignant potential
uncommon variants of iris nevi
- diffuse nevus
- Lisch nodules
diffuse nevus
flat with indistinct margins; typically occurs in patients with congenital ocular melanocytosis
Lisch nodules
small bilateral iris nevi in patients with NF1
ocular melanocytosis:
- ____ iris nevus
- ____ melanocytosis most common
- ____ nevus
- risk of ____
diffuse;
scleral or choroidal;
blue;
melanoma and glaucoma
iris melanoma:
____ tumor of the iris stroma
malignant
iris melanoma etiology
- proliferation of atypical melanocytes
- associated with chronic UV exposure
- associated with changes to iris nevus
iris melanoma demographics
- typically occurs between the ages of 50-60 years
- more common in Caucasians with a light-colored iris
iris melanoma laterality
unilateral
iris melanoma symptoms
- asymptomatic
- ocular complications and visual disturbances
- systemic complications (metastatic)
iris melanoma signs
- 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
iris melanoma complications
- 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
iris melanoma metastasis:
- ____% develop metastasis within 10 years of treatment
- may also present in tandem with ____
5;
systemic cancers
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
100;
90;
50
iris melanoma management
- refer out to OMD, preferably ocular oncologist
- may need blood work and/or systemic care
iris melanoma treatment
- 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
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: ____
gonioscopy, ASeg OCT, and UBM; iris nevus A, B, C, D, E; rule out posterior findings; relatively slowly; uveal (~5%); choroid (85%)
ciliary body melanoma:
-____ ciliary body tumor
malignant
ciliary body melanoma etiology/associations
proliferation of atypical melanocytes
ciliary body melanoma demographics
- typically occurs over the age of 50 years
- more common in Caucasians with a light-colored iris
ciliary body melanoma laterality
unilateral
ciliary body melanoma symptoms
asymptomatic
ciliary body melanoma signs
- 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
ciliary body melanoma complications
- 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
ciliary body melanoma management
- refer out to OMD, preferably ocular oncologist
- may need blood work and/or systemic care
ciliary body melanoma treatment
- 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)
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
~10;
worst;
attain a relatively large size before detected clinically;
gonioscopy through a well-dilated pupil and UBM