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Malignant Tumors - PSD Block 16
Terms in this set (60)
What is the most common intraocular malignancy that we see within children?
What is a malignant tumor of incompletely differentiated retina cells (retinoblasts) seen in children?
How many infants have retinoblastoma?
1 in 18,000 infants; fortunately still rare
What is the prognosis for retinoblastoma?
Almost certain death if not diagnosed/treated early (due to direct spread into the brain)
How does retinoblastoma present? (5)
1. May be unilateral or bilateral (often multifocal)
2. sporadic or familial
3. Leukocoria (white pupil)
4. Strabismus is also common
5. Secondary glaucoma, inflammation (anterior segment or orbital), etc. are less common
What is typically the initial diagnostic sign of retinoblastoma?
Leukocoria (white pupil)
What form of retinoblastoma - sporadic or familial- are typically bilateral, multifocal lesions that predispose the individual to other non-ocular cancers as well?
What form of retinoblastoma- sporadic or familial - accounts for more than half of the cases, are unilateral, and show up often around the age of 2 and do not carry the same predisposition to other types of cancer?
Why is strabismus common with retinoblastoma?
Because they don't have fixation in that eye due to the mass present
How does retinoblastoma first appear?
It begins as a white mass within the retina, which may extend into the vitreous and beneath the retina.
What is a great screening tool for retinoblastoma?
Simple red reflex testing
What condition is diagnosed based on the following results: Suspicion usually aroused by anterior segment signs or family history
DFE (usually under anesthesia) is required!
Imaging (ultrasound and MRI) is used to differentiate, assess size, and rule out other associated systemic tumors
Genetic testing of parents
Is treatment of retinoblastoma the same for all cases? If not, what does it depend on?
No, it depends on the size/location of the tumor
What are the various forms of treatment for retinoblastoma? (5)
1. Photocoagulation (laser)
2. Cryotherapy (freezing)
3. Chemotherapy (targets highly mitotic cells)
4. Various types of radiotherapy (irradiation)
5. Enucleation (used to be the most common treatment of this condition)
If genetic testing of parents is used and uncovers the familial form, what should be done in treatment? (2)
1. Systemic investigation mandated (ongoing)
2. Genetic counseling offered
What is a malignant tumor of choroidal melanocytes and considered the malignant version of a choroidal nevus?
What is the most common primary intraocular malignancy in adults?
What ethnicity is choroidal melanoma much more common in?
What results in death of a patient that has choroidal melanoma? And how many patients who have this condition die within a decade?
Metastasis from eye to other organs often results in death of the patient; about half within a decade due to metastasis
What is the key thing with choroidal melanoma management?
To differentiate it from choroidal nevus and make sure the tumor does not metastasize.
How does choroidal melanoma usually present? (Age group? Symptoms? Appearance?)
Age group- usually found around ages 50-60
Symptoms- often asymptomatic, but may produced blurring/distortion or flashes/floaters (depends on where it's located and how much subretinal fluid is associated with it)
Appearance- typically seen as a pigmented (brown/grey) dome-shaped subretinal elevation, but may be amelanotic or relatively flat; clumps of orange pigment (lipofuscin) are frequently found on the surface (due to highly metabolically active lesion such as a melanoma)
Where in the fundus does choroidal melanoma often appear?
Close to the optic nerve and posterior pole
A helpful tool in identifying choroidal melanoma is TFSOM. What does that stand for?
T-thickness (anytime thickness is greater than 2.5-3 mm we will be worried
F-fluid (generally have sub-foveal fluid developing with choroidal melanoma to some extent)
Symptoms- nevi are almost always asymptomatic, whereas choroidal melanoma are symptomatic
Orange pigment - lipofuscin
Margins- within 3 mm of optic nerve, that is more suspicious
What condition is diagnosed by the following methods: usually evident from fundus appearance, aided by angiography (ICGA), ultrasound, MRI, and biopsy occasionally required (needle)?
What is ICGA useful for? (Hint: what layer of the eye does it make more visible?)
It displays primarily choroidal circulation.
What is a biopsy usually good for in choroidal melanoma?
It's used for prognosis more than diagnosis.
How would an optometrist differentiate choroidal melanoma from similar appearing benign lesions or other malignant lesions?
TFSOM can help; should not refer out every pigmented lesion we see, but we don't want to miss this either. Genetic testing can determine the exact defect.
When viewing an ultrasound, and a tumor has broken through Bruch's Membrane and has blossomed out on the surface of the retina into the vitreous space, what is that pathognomonic for?
True or False- Treatment is ALWAYS required for choroidal melanoma.
False - might have a slow growing tumor in an elderly, sick patient
What are the various treatments for choroidal melanoma? (4)
1. Irradiation (brachytherapy most used method- place a metal disc that will release radioactive material and suture it right on the sclera outside where the tumor is and leave it there a while before removing it)
2. Transpupillary thermotherapy (TTT) - heat it up
3. Resection (rarely done because it's most often at the posterior pole)
Systemic investigation must be done to rule out metastatic spread in choroidal melanoma, which is usually to what organ?
Once choroidal melanoma has metastasized, even with treatment, what is the life expectancy for a patient?
About 6 months
How are choroidal melanomas managed?
They are comanaged with a few doctors - ophthalmic oncologist (decides how to treat it in the eye), oncologist (checks for any places it metastasized to), and an optometrist (to recognize it and refer it out)
The most common tumor within the eye is often seen with what? Where is it most commonly found?
Metastasis ; in the choroid proper (posterior pole; 90%), which makes sense because it is a highly vascular tissue with loads of nutrient support for a tumor
What are secondary tumors which develop in the eye from cells of a primary tumor elsewhere that have travelled to the eye via bloodstream?
Metastatic tumors of the eye
What are the most common type of intraocular malignancy?
Metastatic tumors of the eye
In what order do you find metastatic tumors of the eye in each of the components of the uveal tract (from greatest to least common)?
Choroid (90%) > iris > ciliary body
What are the most common primary sites for metastatic tumors of the eye?
Lung or breast (ocular tumor is composed of these cells)
In the case of which cancer- breast or lung- does the patient know they have the condition and then it metastasizes to the eye?
In the case of which cancer- breast or lung - does the patient often not know and then it's already metastasized?
What condition presents as the following: 1. Usually affects the macula, distorting vision; 2. Typically appears as a relatively flat creamy-whitish "placoid" lesion at the posterior pole; 3. Lesions are most often solitary and unilateral but may be bilateral and/or multifocal; 4. Frequently accompanied by secondary subretinal exudation.
80% of patients will have blurring of vision and may even have a serous RD due to breakdown of RPE resulting in pigments with what condition?
What is the diagnosis of choroidal metastases based on primarily?
How many patients with choroidal metastases have history of malignancy?
Roughly two thirds
What testing can be done to diagnose choroidal metastases?
Ancillary testing such as ultrasound studies, angiography, biopsy (may be helpful when there is no known primary malignancy), and PET, as well as other scans, are useful in diagnosing choroidal metastases.
What is the prognosis for choroidal metastases patients?
Very poor (similar to choroidal melanoma); most patients die within a year of discovery
What is the treatment for choroidal metastasis?
Systemic evaluation by an oncologist is required (to locate primary tumor and for staging, as well as detecting other metastases) and ocular treatment which includes nothing if patient is asymptomatic or receiving systemic chemotherapy OR is aimed at alleviating ocular symptoms
Given choroidal melanoma, retinoblastoma, and choroidal metastasis, rate them on prognosis form worst to best.
Choroidal metastasis (worst) > choroidal melanoma > retinoblastoma (greatest)
What refers to the malignant proliferation of lymphocytes (WBCs) within the eye?
Intraocular lymphoma is a subset of what type of lymphoma?
Primary CNS lymphoma (first arises within the CNS- implied by primary)
True or False- Most patients with primary intraocular lymphoma eventually develop CNS symptoms.
Similar to having concern that choroidal melanoma will metastasize to the liver, we worry that intraocular lymphoma will metastasize to where?
How does intraocular lymphoma usually present? (Age? Accompanying signs? Appearance?)
Age- typically presents in an older person (~60s) as unilateral floaters and blurred vision (eventually bilateral in most cases)
Accompanying signs- vitritis may be severe (+mild anterior uveitis)
Appearance- multiple yellow-white subretinal infiltrates enlarge and coalesce
In cases of intraocular lymphoma, why does the vitritis not clear with corticosteroids?
Because it's not an inflammatory vitritis; it is proliferation of the WBCs within the vitreous due to the tumor, not inflammation
Any time you have an elderly patient who has vitritis that is resistant to steroid treatment and doesn't have CME, what condition do we suspect?
What condition must be considered in differential of any older patient with recalcitrant posterior uveitis?
What testing is done to diagnose intraocular lymphoma?
Vitreous biopsy may be done in the absence of known CNS lymphoma, imaging (MRI), lumbar puncture, and/or biopsy used to detect CNS disease
In terms of diagnosing intraocular lymphoma, what testing method does the recent National Eye Institute recommend be performed first?
Lumbar puncture (CSF evaluation) is recommended to be done first because it's less invasive compared to MRI and biopsy.
What is the treatment for ocular lymphoma?
Treatment of PIOL is aimed at eradicating ocular lymphoma cells and preventing spread to the CNS. It is complicated/varied and requires referral to appropriate specialists (radiotherapy often employed for ophthalmic manifestations and chemotherapy, etc. for CNS involvement)
How is the prognosis for ocular lymphoma?
Very poor - most patients die of CNS disease
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