Tumors

1. melanomas/nevus

2. uveal lymphoma

3. metastatic carcinoma

4. diffuse choroidal hemangioma

5. choroidal osteoma

what are the choroidal tumors?

1. retinoblastoma

2. retinal astrocytoma

3. retinal capillary hemangioma

4. retinal cavernous hemangioma

what are the tumors of the retina?

1. melanocytoma of the optic disc

2. combined hamartoma of the RPE & retina

what are the tumors of the RPE?

malignant melanoma

• choroid > ciliary body > iris

• most common primary intraocular tumors in adults

• risk factors:

  • caucasian/fair-skinned individuals

  • dysplastic nevus syndrome

  • melanocytosis

  • nevus

• commonly presents 5th-6th decade

melanomas

• 1% of cases secondary to inherited BAP1 mutation (nuclear protein)

• sx:

  • usually begins asymptomatic

  • blurry vision, VF loss, flashes/floaters

• circumscribed (nodular) or diffuse (rarer form)

• appearance:

  • pigmentation: gray-green or brown, amelanotic

  • elevated >2mm

  • oval, dome-shape or mushroom/collar button if break in bruch’s

  • overlying lipofuscin (orange pigment)

  • presence of overlying RPE changes (atrophy, drusen, PED w/ subretinal fluid)

  • choroidal folds, hemorrhage, exudate, CNVM, secondary glaucoma, cataract, uveitis

• poor prognosis w/ increasing age

  • referral to retinal subspecialist ASAP

  • PCP/oncologist referral

• tx:

  • observe small tumors

  • radioactive plaques for small-medium tumors

  • transpupillary thermotherapy

  • photodynamic therapy

  • enucleation

• testing:

  • ultrasound

  • IVFA

  • liver enzymes

  • systemic testing to r/o metastasis

1. observe small tumors

2. radioactive plaques

3. transpupillary thermotherapy

4. photodynamic therapy

5. enucleation

what are the options for management of malignant melanomas?

1. no difference in mortality rates after 12yr for medium sized tumors treated with brachytherapy or enucleation

2. in cases w/ distant metastases, tx of the intraocular melanoma become palliative & systemic chemo is primary tx

what did COMS find about melanoma management?

no difference

COMS showed there was _________ in gender for the prognosis of a malignant melanoma

16

___% 5y mortality rate for small malignant melanomas (<2-3mm)

32

____% 5y mortality rate for medium malignant melanomas (3-8mm)

53

____% 5y mortality rate for large malignant melanomas (>8mm)

15-GEP testing

• done w/ fine needle aspiration

• able to differentiate high risk from low risk of metastasis

• may aid in how intense pts are monitored/treated

mottled fluorescence in arteriovenous phase, progressive staining, prolonged dye retention, double circulation

what does a choroidal melanoma show on IVFA?

low-medium reflectivity, excavation of underlying uveal tissue, shadowing of subjacent soft tissues, internal vascularity, acoustic hollowing

what do intraocular melanomas show on B-scan?

acoustic hollowing

characteristic finding on B-scan of an intraocular melanoma; acoustic quiet zone at the base of the tumor

liver (lung, bone, skin, CNS)

what is the most common metastatic site for a choroidal melanoma?

30-50

______% of pts w/ malignant melanoma will die w/in 10y of diagnosis

larger, older

____ sized malignant melanomas in ____ patients have the greatest risk of mortality

1. VA of affected eye

2. VA of other eye

3. size of tumor

4. age

5. general health of pt

6. ocular structures involved

7. presence of metastasis

what are the factors to consider when treating malignant melanoma?

30

ocular melanocytosis is ___x more common in pts w/ uveal melanoma

increased

there is an _____ incidence of uveal melanoma in white patients w/ ocular or oculodermal melanocytosis

choroidal nevus

• found in 10% of pop, present at birth

• maximum growth occurs pre-puberty

• usually asymptomatic

• appearance:

  • flat or minimally elevated

  • oval, circular, slate gray-pigmented or nonpigmented

  • drusen develop over time

  • usually less than 2mm thick

  • may develop RPE atrophy, hyperplasia

• CNVM risk

1. thickness

2. fluid

3. symptoms

4. orange pigment

5. margin of tumor less than 2DD from disc

6. ultrasonographic hollowness

7. halo absence

8. drusen absence

what are the risks for malignant transformation of a nevus?

3%

if the melanoma suspect has 0 risk factors, there is a ____ chance for growth at 5y (most likely choroidal nevi)

38

if the melanoma suspect has 1 risk factor, there is a ____ chance for growth at 5y

50+

if the melanoma suspect has 2+ risk factors, there is a ____ chance for growth at 5y

1. observation

2. RTC x3-4mo to determine stability

3. if stable, RTC 2x/yr to document any change

what is the management for a low risk melanoma suspect?

monitor more closely every 3-6mo

what is the management for a high risk melanoma suspect?

uveal lymphoma

• typically affect immune compromised individuals

• more commonly non-Hodgkin’s B-cell type

  • may be T-cell or Hodgkin’s

• typically a metastatic form of a systemic lymphoma

• unilateral > bilateral

• highly variable presentation:

  • circumscribed or diffuse

  • flat or elevated

  • amelanotic

  • yellow-white infiltration

  • can be multifocal (resembling birdshot or MFC)

  • may have associated conjunctival & orbital involvement

• testing:

  • ultrasound

  • OCT

  • biopsy

uveal thickening, characteristic ovoid echolucent mass

what does a uveal lymphoma show on ultrasound?

characteristic rippled seasick pattern

what does a uveal lymphoma show on OCT?

1. systemic evaluation

2. systemic tx

3. local radiation

4. rarely, if no systemic involvement: observation

what is the management for uveal lymphoma?

choroidal hemangioma

• unilateral, benign vascular tumor

• no sexual or racial predilection

• not a cancer

• does not metastasize

• appearance:

  • can be well circumscribed or diffuse

  • unilateral VA loss in adulthood (or incidental finding)

  • tumors compromised of blood vessels

  • smoothly elevated, dome-shaped (NOT mushroom shaped)

  • oval/circular shape

  • most common location is posterior pole

  • most 3-9mm in diameter

  • can cause CME, exudative RD, & secondary glaucoma

• testing:

  • photograph to document location & size

  • B-scan: sharp borders, acoustic solidity

  • FA: not pathognomonic

• tx:

  • usually none unless subretinal fluid threatens macular region

  • laser photocoagulation if VA threatened due to serous RD

choroidal osteoma

• slow growing intrachoroidal bone-like tumor

• rare, benign

• unilateral > bilateral

• found in healthy, young females

• typically near optic nerve or posterior poles

• sx depend on location but often asymptomatic

• ocular manifestations:

  • yellow-white to red-orange in apperance

  • oval or round

  • edges are scalloped-pseudopod-like projections

  • can cause macula detachments or neo

• tx/prognosis:

  • depends on lesion location

  • growth suggests malignant, must r/o

metastatic carcinoma of choroid

• more common than primary malignancies

• seed of cancer that started in a cancer elsewhere & spread to eye via blood flow

• features:

  • cream, yellow, light brown

  • flat or slightly elevated mottled pigment clumping on surface

  • extensive exudative RD

  • maybe multifocal or bilateral

• testing:

  • B scan: diffuse choroidal thickening

• tx:

  • no enucleation unless painful

  • chemo

  • external beam radiation

breast

what is the most frequent primary site for metastatic carcinoma of the choroid for females?

lungs

what is the most frequent primary site for metastatic carcinoma of the choroid for males?

choroid

what is the most common site for metastasis?

retinoblastoma

• cancer affecting retinal photoreceptors

• most common primary intraocular malignant tumor of childhood

• predisposed to secondary non-ocular malignancies

• unilateral > bilateral (except in inherited cases)

• average age of dx: 18mo (90% under 5y)

• 5-10% of pts have +FHx (AD)

• ocular manifestations:

  • leukocoria

  • strabismus

  • dome-shaped lesion

  • whitish pink nodular mass

  • can cause RRD

  • secondary glaucoma & pseudouveitis

  • iris neo

• testing:

  • B-scan

  • CT

  • MRI

  • genetic testing & counseling

• tx/prognosis:

  • early detection is critical

  • enucleation is last resort

    • remove worse eye if bilateral

    • remove long piece of optic nerve too

  • radiation

  • photocoagulation or cryotherapy

  • systemic chemo (**tx of choice)

1. external beam radiation

2. photocoagulation or cryotherapy (for small tumors behind retina & confined to sensory retina, contraindicated if vitreous seeding)

3. systemic chemotherapy

4. enucleation is last resort

what are the tx strategies for retinoblastoma?

systemic chemo

what is the tx of choice for retinoblastoma?

15%

what is the overall mortality rate for retinoblastoma?

1. scleral or optic nerve involvement

2. tumor size & location

3. cellular differentiation

4. age of pt

5. laterality (bilateral is worse)

what characteristics of a retinoblastoma influence mortality rate?

high reflectivity

what does a retinoblastoma show on B-scan?

endophytic

• retinoblastoma type

• white, pearly-pink, creamy nodular mass that breaks through the ILM into vitreous

• vitreous seeding

• fine blood vessels on surface

• secondary calcification leads to sharp demarcations (cottage cheese)

• multiple tumors possible

exophytic

• retinoblastoma type

• yellowish subretinal mass lesion often underlying a serous RD

• total RD risk

• tumor is difficult to view

1. endophytic

2. exophytic

3. diffusely spreading lesion simulating posterior uveitis

what are the types of retinoblastoma?

retinal capillary hemangiomas

• 75% not associated w/ systemic disease

• may be multiple & bilateral in 50% of pts

• tx:

  • may initially just observe

  • laser or cryo to preserve vision

retinal cavernous hemangioma

• rare, congenital unilateral asymptomatic vascular lesion

• clumps of thin-walled saccular aneurysms filled w/ dark blood

• cluster of grapes

• tx:

  • usually not needed

melanocytoma of optic disc

• rare, benign, unilateral melanotic lesion

• usually affects darkly skinned individuals

• usually incidental finding

• appearance:

  • jet black, feathery edges, inferior location

  • stable or slow growing

• tx:

  • none

FAF will show hypofluorescence for melanoma

how do you differentiate a melanoma or nevus of optic disc?

combined hamartoma of RPE & retina

• rare, unilateral hamartoma

• involves RPE, neurosensory retina, retinal vessels, & adjacent vitreous

• males > females

• appearance:

  • juxtapapillary more common than peripheral

  • strabismus

  • slightly elevated

  • variable amount of intraretinal gliosis

  • ERM

  • dilated capillaries & retinal blood vessels

solitary idiopathic choroiditis

• unknown etiology

• occurs at any age

• no predilection for age or sex

• appearance:

  • yellow-ish white lesion

  • usually good VA except if lesion in juxtapapillary or foveal area

  • normal IOP & AC

• pt usually lacks any medical, systemic, or ocular hx

  • dx of exclusion

• active & inactive phase

choroidal melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanoma

melanocytosis/uveal melanoma

melanocytosis/uveal melanoma

low

what is the concern for this being melanoma?

low

what is the concern for this being melanoma?

low

what is the concern for this being melanoma?

increased

what is the concern for this being melanoma?

high

what is the concern for this being melanoma?

low

what is the concern for this being melanoma?

increased

what is the concern for this being melanoma?

uveal lymphoma

solitary choroidal hemangioma

choroidal hemangioma

choroidal hemangioma

choroidal hemangioma

choroidal hemangioma

choroidal hemangioma

choroidal osteoma

choroidal osteoma

metastatic carcinoma

metastatic carcinoma

metastatic carcinoma

metastatic carcinoma

metastatic carcinoma

metastatic carcinoma

metastatic carcinoma

metastatic carcinoma

metastatic carcinoma

retinoblastoma

retinoblastoma

retinoblastoma