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PSD Block 16- Benign Tumors
Terms in this set (74)
Retinal detachments due to diabetic retinopathy are most often initially of what type?
During choroidal detachment, what two anatomical layers become separated?
Choroid and the sclera
Where does the extracellular fluid usually come from during a serous detachment?
Underneath the RPE; retinal or choroidal blood vessels
What is the most common triggering event for rhegmatogenous retinal detachments?
Posterior vitreous detachment (PVD)
What is an abnormal growth of tissue which exceeds and is uncoordinated with that of the normal tissues around it?
What is a neoplasm that has formed a lump (some neoplasms do not cause a lump)?
What is an abnormal growth of tissue that may be benign or malignant?
This type of tumor does not grow uncontrollably, does not invade neighboring tissues, and does not spread through the body.
Benign; an example of this is Papilloma
This type of tumor grows uncontrollably, invades nearby tissues, and may spread to other parts of the body.
Malignant; an example of this is basal cell carcinoma
What is known as the movement or spreading of malignant tumor cells from one organ or tissue to another via the blood or lymphatic system?
What is the examination of a sample from a tumor to determine if the tumor is benign or malignant?
This represents a benign neoplasm of the RPE cells, and occurs in typical and atypical forms.
Congenital Hypertrophy of the RPE (CHRPE)
Which form of CHRPE - typical or atypical - is common and not associated with any systemic condition?
Which form of CHRPE- typical or atypical- is rare and associated with familial adenomatous polyposis (FAP)?
What is the difference between hyperplasia and hypertrophy?
Hyperplasia means that cells are increasing in number, whereas hypertrophy means cells are increasing in size.
Which form of CHRPE - typical or atypical - is asymptomatic (discovered incidentally), usually unilateral, may be solitary or multiple, and is essentially non-progressive?
Which form of CHRPE presents as multiple lesions in both eyes, which signal a systemic disorder known as FAP?
In typical CHRPE, there may be some hypopigmented areas of the lesion, where you can see down to the sclera. What are these whitish areas of the lesion known as?
How does the solitary presentation of typical CHRPE usually appear? (3)
1. Darkly pigmented, relatively flat, round or oval lesion with well-defined borders (dark grey/black with variable amounts of hypopigmented "lacunae" within the lesion)
2. Typically range in size from 1-6 mm across
3. Usually found near the equator
What is an indication of typical CHRPE being present for a while?
In typical CHRPE, how does the grouped presentation appear? (2)
1. Multiple smaller (0.1-2 mm) lesions, usually grouped together in one sector/quadrant of the fundus
2. May be darkly pigmented ("bear tracks") or non-pigmented ("polar bear tracks")
What is the atypical aspect of atypical CHRPE?
Laterality - it is bilateral
What form of CHRPE - typical or atypical - appears as bilateral, multiple lesions which are often scattered throughout the fundus (not clustered together), and usually assume an elongated oval shape with a depigmented "tail" at one end?
What is the importance of atypical CHRPE?
The fact that they are associated with a systemic condition known as familial adenomatous polyposis (FAP)
What would CHRPE look like with a red-free filter (green light)? What would that help you distinguish it from?
Because it's a shorter wavelength, it gets bent more easily, so the light would not get into the choroid and you would still see the CHRPE, since it involves the RPE cells. It would help distinguish it from a choroidal nevus. You would be able to see a choroidal nevus/lesion with white light.
What percentage of patients will have atypical CHRPE and FAP at birth?
What is a genetic disease that is characterized by devlopment of precancerous colorectal polyps in teenagers?
Familial Adenomatous Polyposis (FAP)
If untreated, what will happen to patients with atypical CHRPE and FAP?
If untreated, it will progress to colorectal carcinoma in almost all patients by age 50.
What is the management for typical CHRPE?
Differentiate CHRPE from other pigmented fundus lesions, document the cases, and monitor periodically.
What is the management for atypical CHRPE?
Refer atypical cases to GI specialist for colonoscopy (also examine relatives)
What is a very common benign tumor of choroidal melanocytes (pigment containing cells)?
True or false- about 1% of choroidal nevi can transform to malignant choroidal melanoma.
True ; in other words, about 1 in 100 will transform to choroidal melanoma.
What is the commonly affected group and age of choroidal nevi?
They are much more common in Caucasians (5-10%) than darker skinned individuals and growth may occur prior to puberty, but is unusual in adulthood.
What are the symptoms of a choroidal nevus?
They are almost always asymptomatic (often an incidental finding) and very rarely, vision may be impaired when: the lesion itself or subretinal fluid affects the macula
What condition has the following clinical signs: oval or round lesion with indistinct borders, usually slate grey (occasionally amelanotic), propensity for posterior pole, flat or minimally elevated, surface drusen signify chronicity, and secondary CNV is uncommon, but possible?
With a choroidal nevus, are blood vessels deterred or undeterred? Can a choroidal nevus be seen with a red-free filter? What will that differentiate from a choroidal nevus?
They are undeterred because it is in the choroid. No, it can't be seen because red-free filter doesn't penetrate that deep. It will help differentiate a choroidal nevus from CHRPE.
How is diagnosis made for a choroidal nevus? (3)
1. Based on fundus appearance
2. Red-free filter can be used to confirm choroidal location (differentiate from CHRPE)
3. Ultrasound may be used to demonstrate thickness (thickness is important at because this is another distinction from more malignant melanomas)
How is a choroidal nevus managed? (2)
1. Documentation (baseline fundus photography and ultrasound or OCT to establish baseline elevation) *
Note: OCT should be used to monitor a choroidal nevus.
2. Periodic evaluation (in order to identify malignant transformation, increase in size and/or thickness beyond puberty suspicious, and orange pigment (lipofuscin) also suspicious)
What is a relatively rare benign tumor of choroidal melanocytes found at or near the optic nerve head that may be a variant of choroidal nevus?
Which condition is seen more often in darkly pigmented individuals than typical choroidal nevi?
Which tumor presents as the following: minimally progressive and usually asymptomatic (rarely visual loss via growth or vascular obstruction - CRVO due to it compressing veins), RAPD may accompany (even with good VA), and has potential for malignant transformation (even less than choroidal nevi)?
What is management for choroidal melanocytoma?
Recognize that this is a benign lesion, counsel the patient, and realize the patient will have had no complaints until they come in for something else, and this does not need to be sent off for biopsy.
What is a rare benign congenital tumor composed of various cell types from both the retina and RPE (usually one cell type predominantly)?
Combined Hamartoma of the Retina and RPE
What condition appears as the following: usually located near the optic disc (similar to choroidal melanocytoma) and has visual distortion that may occur due to "dragging" of the macula or disc?
Combined Hamartoma of the Retina and RPE
What is the management for combined hamartoma of the Retina and RPE?
Observation alone unless symptomatic, then vitrectomy with membrane peeling is attempted. Refer to retinal specialist for symptomatic cases.
What consists of a membranous fibrous sheet on the surface of the retina that obscures retinal blood vessels and cause problems such as vascularity leakage and exudation and blurred vision from exudation at the fovea?
Combined Hamartoma of the Retina and RPE
What is a benign choroidal vascular tumor that may be circumscribed (distinct lesion with fairly well defined borders) or diffuse (really spread out) and is usually diagnosed in children or young adults?
What are the symptoms for a choroidal hemangioma?
Asymptomatic unless the macula is distorted by the tumor itself or serous retinal detachment.
What form - circumscribed or diffuse - of choroidal hemangioma is seen in patients with Sturge-Weber syndrome (neurological consult indicated)?
What is the management for a choroidal hemangioma?
OD management is to monitor for macular involvement or secondary glaucoma
Similar to combined hamartoma of retina and RPE, what do you do with a circumscribed choroidal hemangioma if there are symptoms?
Refer to a retinal specialist
What form of choroidal hemangioma- diffuse or circumscribed - won't usually cause a huge problem for vision with the patient, but if it becomes leaky it will?
What form of choroidal hemangioma- diffuse or circumscribed - do we see hemangioma within the choroid and cutaneous vessels, which can affect the angle and episcleral vessels (resulting in poor drainage in the angle)?
What is a benign vascular tumor of the retinal capillary bed, with sight and life-threatening associations? (note: NOT choroidal)
Which condition presents as the following: asymptomatic unless complications occur such as macular edema/exudates, vitreous hemorrhage, serous RD (all due to leaky vessels)?
Half of the patients with this condition are cases that are associated with von Hippel-Lindau disease (a genetic cancer syndrome)?
What should we do with patients who have capillary hemangioma?
ALL patients and their relatives must be evaluated for VHL disease (life-long monitoring if +) ; Most VHL patients will display capillary hemangioma and will die before the age of 50, so treat the systemic condition
What is a benign vascular tumor characterized by clusters of retinal venous aneurysms and appears as a "bunch of grapes" on the surface of the retina?
Retinal cavernous hemangioma
How does a retinal cavernous hemangioma case present?
Asymptomatic unless complications such as vitreous hemorrhage occur and are occasionally associated with similar lesions of the skin and CNS
On rare occasions, what could a retinal cavernous hemangioma lead to? And what can be done for that?
Vitreous hemorrhage ; vitrectomy
What is a rare congenital arteriole-venous malformation of the retinal vasculature, in which there is no intervening capillary bed? (IN other words, there is a direct anastomoses between arteriole and venous systems.)
How does racemose hemangioma usually present? (3)
It is typically asymptomatic (found incidentally), is seen as a direct communication between enlarged and tortuous retinal veins/arteries, and is sometimes accompanied by similar lesions of the brain or jaw (which may hemorrhage)
What is the treatment for racemose hemangioma?
Ocular treatment is not required, but imaging should be done to rule out non-ocular AVM.
What is a rare gliovascular lesion often discovered following visual decrease from ERM or macular edema that appears as a yellow-red peripheral mass (2/3 inferior temporal quadrant)?
Retinal Vasoproliferative Tumor (VPT)
How does a retinal vasolproliferative tumor appear? (3)
It is typically associated with extensive subretinal and intraretinal exudation, is usually solitary and primary, but 20% are secondary, and are yellow-red peripheral masses (2/3 inferno-temporal)
In 20% of cases, what condition is secondary to RP, pars planitis, Coat's disease, RD repair, etc? How does it appear in these cases?
Retinal Vasoproliferative Tumor and it is more likely bilateral, multifocal, and posterior to the equator
What is the management of asymptomatic retinal VPT? Symptomatic VPT?
Asymptomatic non-leaking tumors may be observed; otherwise, cryotherapy, laser photocoagulation, PDT or plaque radiotherapy (If we have fibrosis on the surface, that would be an indication for treatment, refer out to retinal specialist)
What is a benign **
** congenital tumor of retinal glial cells (also known as astrocytic hamartoma)?
Retinal astrocytoma is usually found in association with what condition? How does that present?
Tuberous sclerosis (Bourneville disease); usually characterized by skin, CNS, and visceral tumors (causing seizures, mental retardation, etc.)
What is the management for retinal astrocytoma?
Management entails evaluation for TS; ocular treatment is not required (unless rare RD)
What is a rare benign tumor made up of mature bone within the choroid (RPE above is atrophied) that is usually seen in otherwise healthy young women?
What condition appears as the following: may be unilateral (75%) or bilateral (25%), is typically found at or near the optic disc, atrophy of overlying RPE and sometimes CNV, and vision is distorted if the macula is affected, but otherwise asymptomatic?
How is a diagnosis made for Choroidal osteoma?
Diagnosis is made based on appearance and confirmed by ultrasound/CT scan.
What is treatment for choroidal osteoma?
Treatment is only required for CNV (associated with RD)
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