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Tumours
Ocular Disease III-Final Exam
116
Medical
Graduate
04/14/2013

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Term
Tumour made up of melanin pigmented cells; metastatic
Definition
Melanoma
Term
term for a non-pigmented melanoma
Definition
Amelanotic
Term
benign, tumour-like nodule that is made up of cells and tissue that normally occur that tissue
Definition
Hamartoma
Term
Neoplasm composed of embryonic cells of the tissue/organ
Definition
Blastoma
Term
Cancer that begins in the skin or tissues that line the body organs
Definition
Carcinoma
Term
Another name for benign choroidal melanoma
Definition
choroidal nevus
Term
What may indicate chronicity of a choroidal nevus
Definition
Overlying Drusen
Term
General shape and elevation of choroidal nevus
Definition
flat/minimally elevated, round or oval
Term
Time when choroidal nevi grow the most
Definition
Before puberty, rare to grow after this time
Term
Choroidal lesions will ___________________ with the red-free filter turned on
Definition
Disappear
Term
With what size of a choroidal nevus should one start to suspect malignancy
Definition
Greater than 2DD
Term
How often should you RTC a 2-5DD choroidal nevus
Definition
every 6 months
Term
Name 4 additional tests for evaluating a suspect choroidal nevus
Definition
Ultrasound
FA
Photodocument
P32
Term
Changes in appearance of a choroidal nevus that increase suspicion of malignancy
Definition
Elevation
Orange pigment
Term
Most common uveal tumour
Definition
Malignant choroidal melanoma
Term
Average age of incidence of malignant choroidal melanomas
Definition
50
Term
Malignant choroidal melanoma is extremely rare in which ethnicity?
Definition
African Americans
Term
Malignant choroidal melanoma-circumscribed vs. diffuse
Which is more common
Definition
Circumscribed
Term
Malignant choroidal melanoma-circumscribed vs. diffuse
Which has worse prognosis
Definition
Diffuse
Term
Color and elevation of malignant choroidal melanoma
Definition
Highly elevated
grayish-green color
Term
Symptoms associated with malignant choroidal melanoma
Definition
may be asymptomatic
reduced Vas
visual field defects
photopsia/floaters
Term
Malignant Choroidal melanoma unilateral vs. bilateral
Primary lesions are typically ___________________
Secondary lesions are typically _________________
Definition
1. unilateral
2. bilateral
Term
Males with malignant choroidal melanoma commonly have a history of these 2 types of cancer
Definition
Lung
liver
Term
Females with malignant choroidal melanoma commonly have a history of which type of cancer
Definition
Breast Cancer
Term
Which is better for visualizing lipofuscin deposits, fluid detachments and dilation of feeder vessels, contact fundus lens or BIO
Definition
contact fundus lens
Term
How to differentiate between melanoma and hamartoma
Definition
Transillumination of the sclera- pigmented will not transmit light but the nonpigmented lesions will
Term
FANG of Lipofuscin
Definition
Will block the fluorescence
Term
Tx options for malignant choroidal melanoma greater than 3 mm thick and larger than 10mm in diameter
Definition
Enucleation
Radioactive plaques
Transpupillary Thermotherapy
Local resection
Heavy charged particle irradiation
Term
3 complications from radioactive plaques
Definition
retinopathy
cataracts
vitreous hemorrhages
Term
Compare FANG of benign vs. malnignant lesion
Definition
Benign=hypofluorescence
Malignant=hyperfluorescence
Term
Definitions for TFSOM (To Find Small Ocular Melanomas)
Definition
T=Thickness >2mm
Subretinal fluid
Symptoms
Orange
Margin touching optic disk
Term
Based on the COMS study results, what is standard treatment for 'large choroidal melanomas.'
Definition
Enucleation, no difference with pre-enucleation radiation
Term
Based on the COMS study results, what is standard treatment for 'medium choroidal melanomas.'
Definition
Same mortality rate for brachytherapy as enucleation, thus most choose brachytherapy so they keep their eye.
Term
A slow-growing, benign, intrachoroidal calcification near the nerve head.
Definition
Choroidal osteoma
Term
Describe the appearance of choroidal osteomas
Definition
yellow-white to orange-red lesions with scalloped edges
Term
Appearance of choroidal osteoma on A scan
Definition
Highly reflective
Term
Appearance of choroidal osteoma on B scan
Definition
Slightly elevated mass
Term
Ciliary Body Malignant Melanomas are usually not visible until they grow very large, what are some other ways they can be diagnosed
Definition
Pressure creates irregular astigmatism
CORECTOPIA
Anterior displacement of lens
Focal opacity in lens
Focal dilation of episcleral vessels
Glaucoma
RD
Term
2 treatment options for ciliary body malignant melanomas
Definition
Enucleation if large
Sector resection if small-medium
Term
Location of a melanocytoma
Definition
Optic Nerve Head
Term
T or F
Melanocytomas usually involve the superior part of the nerve head
Definition
False
Usually the inferior part is involved
Term
T or F
Melanocytomas are almost always devastating to vision
Definition
False
75% have no visual reduction, if there is any it is minimal
Term
Main differential diagnosis for a melanocytoma
Definition
Malignant Melanoma
Term
Which test is used to differentiate a malignant melanoma from a melanocytoma
Definition
Fluorescein angiography
Malignant=hyperfluorescence
Melanocytoma blocks the dye, no vascular abnormalities
Term
Another name for a secondary tumor
Definition
Metastatic Carcinoma
Term
What is one possible reason that ocualr tissues are relatively common sites for metastasis
Definition
Vascular nature of the eye
Term
What proportion of metastatic carcinoma of the choroid are bilateral
Definition
20-25%
Term
Common site for the primary tumour in cases of metastatic carcinoma
Definition
Lung
Breast
Term
Describe appearance of metastatic carcinoma of the choroid
Definition
Generally posterior pole
oval, flat/slightly elevated
creamy white-yellow-gray
Term
Primary symptom of metastatic carcinoma of the optic nerve
Definition
Loss of VA's
Term
Prognosis for metastatic carcinoma of the optic nerve
Definition
Poor, avg survival 10 months
Term
What is the most common primary ocular tumour in children
Definition
Retinoblastoma
Term
Retinoblastoma is a common ocular tumour, second only to _____________________
Definition
Malignant melanoma
Term
Retinoblastoma
Usually solitary or multiple?
Definition
Usually multiple tumours, average of 5
Term
Mortality rate of retinoblastoma
Definition
15-20%
Term
T or F
The majority of cases of retinoblastoma are due to germinal mutations and thus can be passed on to offspring
Definition
False, 25% can be passed on, the other 75% are somatic mutations and cannot be passed on
Term
What is the risk of having a child with retinoblastoma if there are already 2 affected children in the same family
Definition
50.00000%
Term
A survivor of hereditary retinoblastoma has what chance of having a child with retinoblastoma
Definition
50.00000%
Term
2 subjective signs of retinoblastoma
Definition
Leukocoria (2/3)
Poor VA
Term
Why may Retinoblastoma be reflective on ultrasound
Definition
High Calcium Content
Term
Treatment of Retinoblastoma
Photocoagulation is an option for what size of tumours
Definition
Small tumours
Term
Treatment of Retinoblastoma
Irradiation is an option for what size of tumours
Definition
medium to large
Term
Treatment of Retinoblastoma
Cryotherapy is a treatment for what size of tumours
Definition
Small Peripheral Tumours
Term
Description of type of Retinoblastoma that has the best prognosis
Definition
Group I
Small <4DD, behind equator
Term
What is the name of the ciliary body equivalent to retinoblastoma
Definition
Embryonic Medulloepithelioma
Term
Which layer of the ciliary body is involved in embryonic medulloepithelioma
Definition
non-pigmented epithelial layer of the ciliary body
Term
Name 4 associated findings of medulloepithelioma
Definition
Glaucoma
Cataracts
Iritis
rubeosis
Term
Multiple gray to white fluffy mass of inner surface of ciliary body
Definition
Adult Medulloepithelioma
Term
Which ocular tumour is usually associated with trauma to the eye or post inflammation
Definition
Adult Medulloepithelioma
Term
What does CHRPE stand for
Definition
Congenital Hypertrophy of Retinal Pigment Epithelium
Term
Will CHRPE disappear with red-free filter?
Definition
No, RPE lesions do not disappear with red-free filter, choroidal lesions will
Term
Appearance of CHRPE
Definition
dark grey, black
distinct edges
variable size
may be single or multiple
Term
4 'causes' of RPE Hyperplasia
Definition
Insult/Trauma to retina
Chorioretinal inflammations
scars
Choroidal neo
Term
Mechanism of RPE Hyperplasia
Definition
Invasion of RPE into sensory retina
Term
Appearance of RPE Hyperplasia
Definition
Jet black irregular pigmentation
Term
Cerebral Angiomatosis aka______________________
Definition
Von Hippel Lindau Dz
Term
Describe Von Hippel-Lindau Dz
Definition
A condition that predisposes patients to benign and malignant vascular tumours, specifically of the retina, CNS, liver, kidneys, pancreas and spleen
Term
Describe the vasculature involved in cerebroretinal angiomatosis tumours
Definition
Vessels are 2-3X larger than normal and tortuous
Usually involves both afferent and efferent vasculature
Term
Cerebroretinal Angiomatosis
What proportion of ocular manifestations are bilateral
Definition
50.00000%
Term
Cerebroretinal Angiomatosis
More common location for ocular tumours
Definition
Mid-peripheral retina but can be found anywhere
Term
Cerebroretinal Angiomatosis
describe the ocular tumour
Definition
Capillaries growing in disarray with associated glial proliferation
Term
Cerebroretinal Angiomatosis
Ocular complications of the tumours
Definition
Hemorrhage/hard exudation of the tumour
Secondary glaucoma
Retinal Detachment
Term
Cerebroretinal Angiomatosis
Systemic complications
Definition
Cerebral, cerebellum and spinal cord tumours
visceral organ tumours (kidneys, pancreas etc.)
Term
Encephalotrigeminal Angiomatosis aka____________________
Definition
Sturge-Weber Syndrome
Term
3 types of angioma associated with Sturge-Weber syndrome
Definition
Intracranial angioma
Facial Angioma
Choroidal angioma
Term
Which nerves are often involved in Sturge-Weber
Definition
1st and 2nd branches of trigeminal nerve
Term
What is another term for the flat venous hemangioma associated with Sturge-Weber
Definition
Nevus Flammeus or Port Wine Stain
Term
Why might a diffuse choroidal hemangioma be missed
Definition
Because the entire fundus is deep red in color , may only be noticed if compared to the other eye
Term
Choroidal Hemangioma associated with Sturge Weber
Localized or diffuse?
Definition
Tend to be diffuse in Sturge-Weber
Term
Sturge-Weber syndrome-which additional ocular manifestation is a primary concern
Definition
Congenital glaucoma (30% develop)
Term
Involvement of what structure increases likelyhood of development of glaucoma in Sturge-Weber syndrome
Definition
Upper Lid
Term
Why are arteriovenous malformations not true phacomatoses?
Definition
True phacomatoses involve retina, CNS AND skin
This only involves the retina and CNS
Term
What is the origin/cause of arteriovenous malformations
Definition
Embryonic origin-failure of the differentiation of the vascular plexus
Term
Describe arteriovenous malformations
Definition
direct communication of arteries and veins without a capillary bed , these vessels are markedly dilated and tortuous
Term
Symptoms of Arteriovenous Malformation
Definition
typically asymptomatic but can cause hemorrhages
Term
Systemic manifestations of arteriovenous malformations
Definition
similar lesions can occur in the brain, hemorrhage can cause epilepsy
Term
Tuberous Sclerosis aka ___________________
Definition
Bourneville's Disease
Term
Cutaneous manifestations of Tuberous Sclerosis
Definition
Sebaceous Adenoma
Achromic Nevi
Shagreen Patch
Term
Ocular Manifestations of Tuberous Sclerosis
Definition
Small conjunctiva tumours
hypopigmented iris spots
Astrocytoma
Term
Describe the sebaceous adenomas found in Tuberous Sclerosis
Definition
Called Angiofibromas, they are lesions that vary in size and shape, highly vascularized, found area the nose and lips
Term
Describe Achromic nevi
Definition
hypopigmented skin patches on trunk and limbs that resembles ash leaves
Term
Describe the Shagreen patch associated with Tuberous Sclerosis
Definition
A diffuse, fibrous thickening that has an orange peel appearance, usually located on the back
Term
Describe appearance of astrocytoma associated with Tuberous Sclerosis
Definition
Gray-white raised, nodular lesions that resemble a mulberry, 0.5-1DD in size, usually near ONH
Term
Primary symptom of astrocytoma
Definition
Visual Field Defect
Term
Systemic manifestations of Tuberous Sclerosis
Definition
Astrocytic tumours can develop in the brain and block CSF drainage or cause focal defects. 80% suffer epilepsy, 60% develop some mental retardation
Tumours may also develop in kidney, thyroid and heart
Term
3 Cutaneous Manifestations of Neurofibromatosis
Definition
Café au lait spots
Plexiform neurofibromas
Fibroma molluscum
Term
Café au lait spots are associated with which diseasse
Definition
Neurofibromatosis (Von Recklinghausen's Disease)
Term
Describe the number and size of Café au Lait spots that almost guarantees the manifestation of Neurofibromatosis
Definition
6 or more spots exceeding 1.5 cm in diameter
Term
subcutaneous tumours originating in the Schwann cells of peripheral nerves
Definition
Plexiform neurofibromas (Neurofibromatosis)
Term
how do plexiform neurofibromas cause elephantiasis neuromatosis
Definition
They cause hypertrophy of the surrounding skin
Term
CNS Manifestations of Neurofibromatosis
Definition
Multiple tumours of brain, spinal cord, meninges and nerves
Term
Manifestation of Neurofibromatosis in the lids
Definition
Produces s-shaped lids with partial to complete ptosis
Term
How may neurofibromatosis result in proptosis
Definition
Orbital tumours or optic nerve gliomas can cause the proptosis
May also be due to congenital absence of portion of the sphenoid
Term
How may neurofibromatosis manifest on the iris
Definition
Formation of tumours that look like tan nodules or nevi, these increase in number with age and cause heterochromia
Term
What ocular disease may manifest due to Neurofibromatosis
Definition
Congenital unilateral glaucoma
Term
Treatment of neurofibromatosis
Definition
surgical removal of the tumours, possibly irradiation of the optic nerve gliomas
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