7. PATH Eye

Question 1

What is the MCC of uni or bilateral exopthalmos due to enlargement of the extraocular muscles with nongranulomatous inflammation, increased glycosaminoglycans and endomysial fibrosis?

Answer 1

Thyroid Dz: Graves orbitopathy

Question 2

In Graves dz extraocular muscles are enlarged in size but the tendons of the muscles and adipose are NOT inflammed/ enlarged as compared to a pseudotumor (idopathic orbital inflammation) which the opposite occurs. Complications of proptosis/exopthalmos includes corneal complications due to excessive exposure as well as what?

Answer 2

Vision loss due to compression of the optic N

Question 3

What inflammation morphology of the eye is characterized by chronic inflammation and fibrosis, where the orbital fat is replaced by fibrosis, with the inflammatory infiltrate consisting of lymphocytes, plasma cells, and occasionally eosinophils?

Answer 3

Idiopathic orbital inflammation

also necrosis+degenerating collagen w vasculitis = wegener granulomatosis

Question 4

What is a systemic dz with granulomatous uveitis and sympathetic ophthalmia, may produce bilateral granulomatous inflammation secondary to penetrating injury, ‘Mutton fat’ is keratic ppt in the anterior segment and candle wax drippings see on opthalmic exam which is perivascular inflammation of the retina?

Answer 4

Sarcoidosis

Question 5

What is the MC malignant tumor of periocular skin (eyelid), 15-40X greater than squamous cell carcinoma, seen in younger patients with lots of sun exposure, more common in the lower eyelid> inner canthus > upper lid > outer canthus, nests of basaloid cells that show peripheral palisading of nuclei*?

Answer 5

Basal Cell Carcinoma BCC

Question 6

Basal Cell Carcinoma BCC is typically nodulo-ulcerative containing pearly nodules, telangiectatic vessels, central ulcer (rodent ulcer) and rolled edges, with morpheaform which is cords/tendrils of tumor cells embedded in densely fibrotic stroma, widely and deeply infiltrative..aka as Blue and Below- which means what?

Answer 6

large masses (blue colored on histo) below the epidermis

Note: SCC has keratin pearls and is more pink colored

Question 7

What carcinoma is as common as SCC of the eyelid, seen in >40yo asian women, 2/3 are in the UPPER eyelid and meibomian gland and has a masquerade syndrome known as unilateral keratoconjunctivitis unresponsive to therapy, intraepithelial spread of tumor cells replaces conjunctival epithelium in a pagetoid or bowenoid fasion?

Answer 7

Sebaceous Carcinoma

(form a focal mass that mimics chalazion- result from a granulomtous response where lipid extravasates into surrounding tissue/ lipogranuloma)

Question 8

Sebaceous Carcinoma has larger nuclei with increased pleomorphism, and more hyperchromatic and more atypical than BCC, comedocarcinoma = necrotic centrally, can met to LN, lung, liver brain and skull, tumor mortality is 15%, +EMA (epithelial membrane antigen), focally + BRST1, P16 highlights pagetoid cells, what is a stain that tests positively for fat on frozen section?

Answer 8

Oil Red O Fat Stain +

*NOTE: well differentiated with vacuolization of the cytoplasm (foamy)

Question 9

What submucosal elevation of the conjuctiva which is benign, results from sun exposure (actinic damage) and appear at the interpalpebral fissure, formed by growths of fibrovascular connective tissue that migrates onto the cornea, dissecting into the plane normall occupied by the bowmen layer resulting in a corneal scar- does not pose a threat to vision?

Answer 9

Pterygium ***in cornea may cover eye

Question 10

What is a small yellowish submucosal elevation of the conjuctiva which is benign, has thin epithelium (hyperplastic or dysplastic) with fragmented stromal collagen and basophilic degeneration (elastic degen), due to sun exposure and typically originate NEXT to cornea- not involving it?

Answer 10

Pinguecula **next to

Question 11

A freckle is increased melanin within basal keratinocytes, melanocytes normal in # but may be slightly enlarged. What is a linear non-nested melanocytic hyperplasia restricted to the basal cell layer?

Answer 11

Lentigo

Question 12

Nevus are melanocytes that become round and grow in aggregates of nests. Junctional nevi are epidermal nests ALONG THE dermoepidermal junction, compound nevi is when junctional grows INTO underlying dermis as nests/cords, what nevi is when the epidermal nests are completely lost?

Answer 12

Intradermal Nevi

Nevi are uniform, with round nucleui, inconspicuous nucleoli w no mitotic activity

Question 13

What kind of nevi are of the eye and are common, rarely invade the cornea or appear in the fornix or over the palpebral conjunctiva, the pigmented zones most likely represent melanoma or melanoma precursors?

Answer 13

Conjuctival Nevi

Question 14

What is the MC intraocular tumor in adults via metastasis to the eye, uvea is the MC PRIMARY intraocular tumor in adults accounting for 5% of US cases, GNAQ and GNA11 are the MC uveal type, morphologically see spindle/fusiform, if epithelioid type- is spherical, greater atypia and a WORSE prognosis?

Answer 14

Melanoma/ Uveal melanoma

uvea consists of iris, choroid and ciliary body

Question 15

Uveal Melanoma usually has large nuclei and prominent nucleoli with infiltrating plasma cells and lymphs, it first spreads to the liver, 80% 5 yr / 40% at 10yr, there is no difference in survival between enucleation vs. eye sparing XRT, what melanoma cells are associated with an adverse outcome?

Answer 15

Epithelioid melanoma cells

Question 16

The corneal tissue is stained by PAS to highlight basement membranes, has 3 layers including epithelium, bowmans layer and the stroma (inner most), a thin PAS+ basement membrane separates the epithelium from the bowman layer - which is acellular, on the inside of the cornea, stroma continues and transitions to what PAS + membrane, which is then next to the cornea endothelium?

Answer 16

PAS+ Descemet membrane

layers outside to in: epithelium, bowmans, stroma, descemet membrane, endothelium

Question 17

Corneal stroma lacks blood vessels and lymphatics which contributes to its opacity and lack* of rejection, during transplant the risk of graft rejection increases with stromal vascularization and invasion, non-immunologic rejection (ie: loss endothelial cells/subsequent corneal edema) is more common than immunological, what may accompany chronic corneal edema, inflammation and scarring?

Answer 17

Corneal Vascularization - tx/prevent with VEGF antagonists

Question 18

Myopia is when the eye is too long for its refractive power- image falls in front of the retina = short sightedness- can see near not far.. What occurs when the eye is too short for its refractive power and the image falls behind the retina causing far sightedness in which a person can see far but not near?

Answer 18

Hyperopia

Question 19

MC infections of the eye are staphylococcus aureus, strep pneumo, pseudomonas, and enterobacteriaceae, PMNs and necrotic debris, gram stain but culture is MOST accurate, What keratitis is the MC with dendrite- linear arborizing pattern of opacification and swelling of epithelial cells, giemsa reveals intranuclear viral inclusions, infected cells may coalesce to form multinucleated giant cells, chronic stromal keratitis leads to ulceration and scarring,and it can cause a granulomatous reaction at descemets membrane (hallmark) when full thickness involved?

Answer 19

Herpes Simplex Virus Keratitis

histo: cornea is thin and scarred w increased # of fibroblast nuclei

Question 20

What are lenticular opacities that may be congenital or acquired, with risk factors including DM*, wilsons, atopic dermatitis, drugs, radiation and trauma, can be age related typically resulting from opacification of the lens nucleus = nuclear sclerosis and can render the nucleus brown to distort the patients perception of blue?

Answer 20

Cataract (anterior segment)

Question 21

Migration of the lens epithelium posterior to the lens equator may result in posterior subscapular cataract secondary to enlargement of abnormall positioned lens epithelium, if the lens cortex liquifues nearly entirely, it is a condition known as?

Answer 21

Morgagnin (hypermature) cataract

Question 22

What type of glaucoma is a secondary open angle glaucoma where high molecular weight proteins can leak through the lens capsule (known as phacolysis), these proteins can clob the trabecular meshwork and contribute to elevation in IOP and optic nerve damage?

Answer 22

Phacolytic Glaucoma

Question 23

Glaucoma is a collection of diseases with distinctive changes in the visual field and in the cup of the optic nerve, most are assoc with elevated intraocular pressure, the aqueous humor from posterior to anterior chamber drains thru trabecular network into canal of schlemm, what kind of glaucoma is when the peripheral zone of the iris adheres to the trabecular meshwork and physically impedes the egress of humor from the eye?

Answer 23

Angle-closure glaucoma (closed-angle)

Question 24

Open angle glaucoma is where aqueous humor has complete open access to the trabecular meshwork but there is resistance to outflow causing IOP. Primary open angle is MC form, MYOC (myocilin) gene mutations for juveniles and adults, some adults with OPTN (optineurin) mutation instead, secondary open angle MC type is what, which is due to deposition of fibrillary material throughout anterior segment- *LOX1 gene?

Answer 24

Pseudoexfoliation

LOX= lysyl oxidase like 1 gene, also deposits seen in vessels, CT, liver kidney and GB

Question 25

Risk factors for glaucoma include race (more common in AA and hispanics), family hx, trauma, prolonged corticosteroid use, eye abnormalities (shallow anterior chamber as seen in hyperopia), and what two most important?

Answer 25

Age and Diabetes Mellitus

Question 26

Diabetic retinopathy causes 1 in 4 of the new blindness causes in the US due to primary changes in retinal microcirculation, there is a laser photocoagulation tx that causes variable destruction of outer retina, destruction of RPE (retinal pigment epithelium) and occlusion of choriocapillaris, they heal by proliferation of adjacent RPE and?

Answer 26

Glial scarring

Question 27

Uveal melanomas are assoc with GNAQ and GNA11 oncogene mutations, the tumor also has loss of BAP1 on chr3 which is a tumor suppressor gene unique to melanomas, they contain two types of cells, spindle which are fusiform shape and epithelioid which are spherical and have greater cytologic atypicality, uniquely have lopping slit like spaces lined by laminin surround tumor cells that arent vessels, but act as vessels in a process called?

Answer 27

Vasculogenic Mimicry - a way for the melanoma to get nutrients

Question 28

Uveal melanomas are found by chance or when vision problems present, prognosis is related to lateral size of the tumor if there are epithelioid cells present and the proliferative index like if they have loss of chr 3, MM of the ciliary body and choroid are more aggressive, mets appear out of the blue many years after treatment which is due to or known as?

Answer 28

Tumor Dormancy

Question 29

What comes from damage to the macula which is required for central vision, either wet (neoangiogenesis) or dry (no neoangiogenesis), advanced age is a risk factor, dry is characterized by deposits in the Bruch membrane and geographic atropy of RPE - no tx, and wet is characterized by choroidal neovascularization of vessels that originate from choriocapillaries and penetrate through** the Brunch membrane beneath RPE and can even go beneath retina?

Answer 29

Age-Related Macular Degeneration - Leading cause of blindness in US

Question 30

NOTE: functional unit = retinal pigment epi RPE, bruch membrane (contains BM of RPE), and innermost layer of choroidal vasculature = choriocapillari— disturbing any of these three leads to damage to photoreceptors and visual loss as seen in Macular degeneration

Answer 30

MEOW

Question 31

What is the MC primary intraocular malignancy in kids, associated with inherited mutation in the RB gene and are bilateral, familial is usually germline mutation in on RB allele in 40% of cases, a separate -second somatic mutation in RB gene causes LOF, sporadic occurs by two separate sporadic muations on the same RB allele, causing LOF and what disease?

Answer 31

Retinoblastoma

Question 32

Retinoblastoma has a neuroblastic origin from nucleated layers of retina- chr 13 long arm - 13Q14 = RB, normal gene suppresses developmment of RB and other tumors, 40% are familial germline mutation and MC bilateral, MC sx include strabismus, ocular inflammation and what which is known as white pupillary reflex**? (less common sx include heterochromia, spont hyphema and cellulitis)

Answer 32

Leukocoria

Question 33

With Retinoblastoma, cells shed into anterior chamber may aggregate and form nodules on iris or settle inferiorly forming pseudohypopyon*, proptosis and enlarged preauricular and cervical nodes is seen when it extends anteriorly, mets to skull bones brain, SC, LN, abd viscera, MC route of escape is via optic nerve w direct infiltration can lead to extension into brain which is assoc with?

Answer 33

poor prognosis (optic nerve invasion)

Question 34

Retinoblastoma can have both differentiated and undifferentiated elemnts with differentiated appearing as round, small cells with hyperchromatic nuclei and SCANT cytoplasm, high mitotic rate, necrosis with calcification and perivascular cuffs of viable tumor cells. What rosettes are characteristic and seen as a single row of eosinophilic columnar cells with peripherally oriented nuclei surrounding central lumen lined by a refractile structure?

Answer 34

Flexner-Wintersteiner

Homer wright rosettes less common w lumen filled w tangle of eosinophilic cytoplasmic processes
Fleurette rosettes = clusters of cells composed of rod/cone inner segments attached to abortive outer segments

Question 35

Flexner Wintersteiner rosettes are characterized by focal zones of?

Answer 35

dystrophic calcification***

Question 36

A retinocytoma or retinoma is a premalignant lesion, appearance of retinoblastoma in one eye and reinocytoma in the other eye is characteristic of hertiable?

Answer 36

retinoblastoma